Cortical Activity During the Writing Task in Dystonia ‐ A Study with Functional Near Infrared Spectroscopy (fNIRS)

Objective: This study aimed to investigate the cortical activity in focal right upper limb dystonia patients using functional near infrared spectroscopy (fNIRS) during writing. Background: Techniques such as fMRI and PET impose important physical constraints. Latter advances in functional near infrared spectroscopy offer a new possibility for investigating cortical areas and the neural correlates of complex motor behaviors non‐invasively under naturalistic experimentation. Methods: Twenty‐one patients with right upper limb idiopathic dystonia (6 with task‐specific dystonia) and twenty‐one healthy volunteers matched for age and years of education were submitted to a simple right‐hand writing task paradigm that consisted of 4 epochs of alternating writing/resting blocks. To test a priori hypotheses that brain activation to simple handwriting in cortical sensorimotor and supplementary motor regions would be less specific in patients than in controls, we defined right and left primary motor (M1) and somatosensory (S1) and supplementary motor area (SMA) ROIs. Differences between groups on changes in both deoxy and oxy‐Hemoglobin (HbO2) and for each ROI were then compared using Mann Whitney tests. Results: Channels exhibiting increased HbO2 for the writing task compared to the resting condition in the patient and control groups are described in Fig. 1. In controls, a lateralization predominantly to the left side was observed in most of the central channels covering the primary motor and somatosensory cortices. A more bilateral pattern of activation was observed in patients. However, in a direct channel‐wise comparison between groups, no significant differences were observed in any channel. In the ROI‐based group analysis, between‐group differences were observed in two of the five ROIs: left M1 (p = 0.022) and S1 (p = 0.022) exhibited increased HbO2 in patients in relation to controls. Conclusions: Overactivity findings on M1 as S1 are in agreement with previous studies of the writing task in non‐ecological conditions1,2,3, reinforcing the role of these areas in dystonia. As symptoms in dystonia are very specific to the task, we believe the pattern of brain activation is as much as specific, therefore the importance of an experimental set that mimics real life conditions. To our knowledge, this is the first study to measure cortical activity during ecological writing (subjects sited in anatomical position, in less noisy environment, using traditional paper and pen) in focal upper limb dystonia patients, outlining the potential of fNIRS for the study of the movement disorders in unconstrained environments.

ABSTRACTS Ataxia 13 Cerebellar Ataxias: Clinical and Molecular Description – A Case Series in a Center of Buenos Aires Natalia Gonzalez Rojas (La Plata, Argentina), Martin Cesarini (La Plata, Argentina), Gustavo Andres Da Prat de Magalhaes (Buenos Aires, Argentina), Jose Luis Etcheverry (Caba, Argentina), Emilia Gatto (Buenos Aires, Argentina) Objective: Molecularly characterize a series of hereditary ataxias evaluated in a center of Buenos Aires. Background: Hereditary ataxias comprise a diverse group of neurodegenerative disorders. Clinical phenotypes vary from predominantly cerebellar syndromes to sensorimotor neuropathy, ophthalmological disturbances, involuntary movements, seizures, cognitive dysfunction, skeletal abnormalities and cutaneous disorders, among others.The new genetic diagnostic techniques allowed a huge knowledge expansion of hereditary ataxias, with a growing number of new variants as causatives. Classically, a regional distribution of some of them is described. There is a lack of a national registry in Argentina, with only case descriptions published in the literature. Methods: Data was obtained from the medical records of 50 patients with a diagnosis of ataxia. The positive molecular diagnosis was prioritized in order to typify the demographic and clinical characteristics and identify the most prevalent variants in our cohort. Results: The sample included 25 men and 25 women. The average age of onset was 52.5 years. The average time of disease evolution was 3.18 years. 38% (n = 19) had a positive family history. 22 patients agreed to the molecular study corresponding to the following diagnoses: SCA3 (n = 9, corresponding to 4 families), SCA1 (n = 1), SCA2 (n = 4), SCA10 (n = 1), Friedreich’s ataxia (n = 4), Episodic Ataxia type 1 (n = 1); Stub 1 (n = 1), FMR-1 (n = 1). The predominant symptom at onset was gait instability and falls. A proportion of cases had another neurological signs (5.5%) in which pyramidalism and lower limb polyneuropathy (MMII) were the most frequent ones. It is important to highlight the presence of Anti-GAD antibodies in one of the patients with SCA2 (+), with a positive response to the administration of intravenous immunoglobulins. By last, in one SCA3 families the presence of triplet expansion for Kennedy disease was identiﬁed in one of its members. Conclusions: This case series demonstrates that SCA3 is the most prevalent variant in our center. On the other hand, although exceptional, we mention the coexistence of genetic and immunomediated causes, in addition to the coexistence of two entities related to triplet expansions in the same family. References: Brent L Fogel, Susan Perlman. Clinical features and molecular genetics of autosomal recessive cerebellar ataxias (Lancet Neurol 2007; 6: 245–57) Alexandra Durr. Autosomal dominant cerebellar ataxias: polyglutamineexpansions and beyond (Lancet Neurol 2010; 9: 885–94). 14 Overwhelming Genetic Heterogeneity and Exhausting Molecular Diagnostic Process in Chronic and Progressive Ataxias: Facing Up with an Algorithm, a Gene, a Panel at the Same Time Sergio Rodriguez Quiroga (Buenos Aires- Capital Federal, Argentina), Joseﬁna Perez Maturo (CABA, Argentina), Lucia Zavala (Buenos Aires, Argentina), Patricia Vega (CABA, Argentina), Nancy Medina (CABA, Argentina), Dolores González Morón (CABA, Argentina), Valeria Salinas (Buenos Aires, Argentina), Julieta Rosales (Buenos Aires, Argentina), Marta Cordoba (Buenos Aires, Argentina), Tomoko Arakaki (CABA, Argentina), Nélida Garretto (Buenos Aires, Argentina), Marcelo Kauffman (CABA, Argentina) Objective: Our objective was to characterize a group of adult and pediatric patients with ataxia and to evaluate the yield of our own clinical-molecular algorithm, by the use of classical and new generation sequencing techniques. Background: Ataxia is a frequent chief complaint in Neurogenetics. There are more than 50 dominant ataxias and a similar number of recessive ataxias. All of them are characterized by a wide genetic heterogeneity, conditioning a complex diagnostic process. Methods: An exploratory, prospective, observational and descriptive study was carried out in 268 patients with progressive ataxia evaluated between May 2008 and May 2019. Patients were stratiﬁed in autosomal dominant, recessive and sporadic inheritance ataxias and we used the clinical-molecular algorithm proposed in each subject. Molecular studies included individual gene sequencing, trinucleotide expansion characterization, new generation multigene sequencing and whole exome and genome sequencing. Results: Through the use of our clinical-molecular algorithm, we identiﬁed the causative gene in 96 subjects, obtaining a diagnosis yield of 31%, the diagnosis yield increases if we consider only subjects with positive family history (57%), particularly in the subgroup of recessive ataxias (71%). Spinocerebellar ataxia type-2 (35%) and Friedreich ataxia (65%) were the most frequent dominant and recessive ataxias respectively. The use of massively parallel sequencing methods were of diagnostic utility in 53% of cases where this techniques were used. Conclusions: We developed and implemented locally a clinicalmolecular algorithm that allowed us obtaining a genetic diagnosis in a signiﬁcant number of patients. Our study describes the most important series of hereditary ataxia patients to date and provides relevant epidemiological information for a better and precise knowledge of the most prevalent subtypes of genetic ataxias in our country. References: Ruano, L., et al., The global epidemiology of hereditary ataxia and spastic paraplegia: a systematic review of prevalence studies. Neuroepidemiology, 2014. 42(3): p. 174-83.Sequeiros, J., S. Martins, and Silveira, I., Epidemiology and population genetics of degenerative ataxias. Handb Clin Neurol, 2012. 103: p. 227-51.Moseley, M.L., et al., Incidence of dominant spinocerebellar and Friedreich triplet repeats among 361 ataxia families. Neurology, 1998. 51(6): p. 1666-71. Rodriguez-Quiroga, S.A., et al., Neurogenetics in Argentina: diagnostic yield in a personalized research based clinic. Genet Res (Camb), 2015. 97: p. e10. 15 Outcomes After Weighted Lumbosacral Orthosis (LSO) and Exercises in Patients with Progressive Cerebellar Ataxia Sabrina Mele (Philadelphia, PA, United States) Objective: Readers will recognize the weighted lumbosacral orthosis (LSO) as a tool to enhance balance exercises to improve function in persons with progressive cerebellar ataxia. Background: Somatosensory input via an LSO creates an enriched environment in outpatient PT to enhance the efﬁcacy of static and dynamic gaze stabilization and postural control exercises in persons with progressive ataxia. A 59 year old female baseline scores performed 3-7 years prior ranged from 30% at worst to 43% at best on the Sensory Organization Test (SOT) while a 69 year old male scores ranged from 29% to 32% at worst and 36 % at best. Methods: Functional outcomes were compared pre and post weighted LSO with exercises over 10 to 12 visits. Results: Pre intervention, the female’s gait speed was 0.85 m/s with poor quality. Five time sit to stand (FTSTS) was 20 seconds, SOT 43, 6 minute walk Test (6 MWT) 360 feet x 2. Post 8 visits, her FTSTS was 20 seconds, SOT 46, 6 MWT 565 feet, gait speed was 0.6 m/s with improved quality. Post 12 visits, FTSTS 20 seconds, SOT 45, 6 MWT 780 feet and gait speed 0.66 m/s with improved quality. Pre intervention, the male’s gait speed was 0.82 m/s with poor quality, FTSTS 15 seconds. Post intervention, gait speed was 1.0 m/ with improved quality, FTSTS 12 seconds, SOT 36 % and 1080 feet in a 6MWT. Post visit 10, while SOT was never repeated and FTSTS 16 seconds, 6 MWT 1146 feet, gait speed 1.0 m/s, both with improved quality. Conclusions: While more research is needed, immediately post (same session) donning the weighted LSO, certain functional outcome scores were preserved compared to their functional scores from 3-7 years prior in the same. Other functional outcome measures improved over the course of 10 to 12 visits of training with the weighted LSO. References: 1. Hadjivassiliou M., et al. Causes of progressive cerebellar ataxia: prospective evaluation of 1500 patients. J Neurol Neurosurg Psychiatry 2017;88:301–309. This abstract was presented at the 2019 International Congress of Parkinson’s Disease and Movement Disorders in September 2019. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 © 2020 International Parkinson and Movement Disorder Society S13 ABSTRACTS 16 Adult Ataxia-Telangiectasia: A Case Report and Description of Genetic and Functional Findings Joseﬁna Perez Maturo (CABA, Argentina), Lucia Zavala (Buenos Aires, Argentina), Patricia Vega (CABA, Argentina), Dolores González Morón (CABA, Argentina), Nancy Medina (CABA, Argentina), Marcela Gonzalez Cid (Buenos Aires, Argentina), Sergio Rodriguez Quiroga (Buenos Aires- Capital Federal, Argentina), Marcelo Kauffman (CABA, Argentina) Objective: To describe the clinical and the genetic study performed in an Argentinian patient with an atypical presentation of Ataxia Telangiectasia (AT). Background: Pathogenic variants in ATM gene are responsible for AT, an autosomal recessive neurodegenerative disease. As a result of the large size of the gene and the diverse spectrum of ATM variants, diagnosis of AT could be a challenge. Methods: A 22-year-old female patient, without relevant family history for any neurological or clinical problem. She started at the ﬁrst year of life with slowly progressive gait disorders and motor clumsiness in upper limbs. On examination, she presented impaired horizontal smooth pursuit and slow and hypometric saccades. Axial and appendicular cerebellar ataxia was evident (SARA scale for 7 points). Distal chorea in upper limbs was present.MRI demonstrated cerebellar atrophy, the alpha-fetoprotein(AFP) levels, were high (156.2 ng/mL, reference value <10 ng/mL) and levels of immunoglobulins were low (IgA <2 mg/dL, reference range: 71-360 mg/dL; IgE 1 UI/mL, reference value < 100UI/mL). Genetic testing for friedreich ataxia was negative. Results: Following our diagnostic algorithm of sporadic ataxic patients, we proceeded to perform an ataxias NGS multigene panel. Two variants were identiﬁed in compound heterozygous in the ATM gene (NM_000051) c.1373G> A (p. Cys458Tyr); c.8785_8786delAGGT (p. Arg2929Metfs * 2). These variants have not been previously reported in the literature. Subsequently, a functional study allowed us to detect, a signiﬁcantly increased frequency of chromosomal aberrations, mainly chromatid breaks, in metaphases of peripheral blood lymphocytes from our patient treated with etoposide compared to a healthy control. This result conﬁrms that our patient has a deﬁciency in the DNA damage repair mechanism, a process that involves the ATM protein kinase supporting the diagnosis of AT. Conclusions: Classic forms of AT with childhood-onset and a life expectancy reduced to the mid-20s, are the result of the presence of two truncating variants in ATM, which cause total loss of the ATM protein. Milder phenotypes are characterized by a later onset and a slower progression, usually associated with missense and splicing variants where some expression of normal ATM protein remains.This case highlights the clinical characteristics of an adulthood AT patient, with a benign course of cerebellar dysfunction and the importance of performing a genetic testing to give adequate genetic counseling to affected patients and parents as obligate heterozygotes. References: Taylor AM, Byrd PJ. Molecular pathology of ataxia telangiectasia. J Clin Pathol. 2005;58(10):1009–1015. doi:10.1136/ jcp.2005.026062. Méneret A., Ahmar-Beaugendre Y., Rieunier G., Mahlaoui N., Gaymard B., Apartis E, …Anheim M.Neurology. The pleiotropic movement disorders phenotype of adult ataxia-telangiectasia. 2014 Sep 16;83(12):1087-95. doi: 10.1212/WNL.0000000000000794. Epub 2014 Aug 13. 17 Neuronal Ceroid Lipofuscinosis Type 2 as a Form of Early Onset Ataxia: Another Potentially Treatable Cause in the Spectrum of Recessive Ataxias Lucia Zavala (Buenos Aires, Argentina), Joseﬁna Perez Maturo (CABA, Argentina), Patricia Vega (CABA, Argentina), Dolores González Morón (CABA, Argentina), Sergio Rodriguez Quiroga (Buenos Aires- Capital Federal, Argentina), Marcelo Kauffman (CABA, Argentina) Objective: The objective of this work is to present a subject with an atypical phenotype of Neuronal ceroid-lipofuscinoses type 2 (CLN2), characterized by a predominant ataxic presentation beginning in the ﬁrst decade of life. S14 Background: CLN2 comprises a group of lysosomal storage disorders, caused by pathogenic variants in TPP1 gene. Classic onset is between 2-5 years, with a survival of 6-10 years, and severe quality of life. However, atypical benign phenotypes are described. Methods: We present a 20-year-old patient, with no relevant family history or consanguinity, who starts at the age of 7 with a progressive ataxic syndrome associated with regression of developmental milestones. The neurological examination highlighted the presence of axial and appendicular ataxia (28 points SARA scale), dysarthria, and severe cognitive impairment. Epilepsy with complex-partial seizures appears when he was 19. Brain MRI showed marked cerebellar atrophy. No abnormal expansions in the frataxin gene were detected. Results: Following our diagnostic algorithm of sporadic ataxic patients, we proceeded to perform an ataxias NGS multigene panel.Two variants in compound heterozygosis previously reported were identiﬁed in the TTP1 gene (NM_000391.3): c827A> T (p.Asp276Val) and TTP1 (NM_000391.3) c.887-10A> G. Subsequently, was determined the absence of enzymatic activity of Tripeptidyl Peptidase 1 in a peripheral blood sample, supporting the diagnosis of CLN2. Conclusions: Autosomal Recessive ataxias represents a diagnosis challenge because of the wide clinical and genetic heterogeneity. NGS techniques revolutionized molecular diagnosis, allowing the identiﬁcation of new genes.The phenotype associated with TPP1 mutations recently had been expanded to an ataxic cerebellar presentation unifying a previously described disease termed as spinocerebellar ataxia autosomal recessive-7 (SCAR-7). Recent clinical studies demonstrated the utility of an enzyme replacement therapy, slowing the progression of CLN2.Our case highlights the importance of the use of NGS techniques and illustrates that atypical forms of CLN2 should be considered, since an early diagnosis is crucial for optimal management of these patients. References: 1. Bessa C, Teixeira CA, Dias A, Alves M, Rocha S, Lacerda L, et al. CLN2 / TPP1 deﬁciency?: The novel mutation IVS7-10A > G causes intron retention and is associated with a mild disease phenotype q. 2008;93:66–73. 2. Gardner E, Bailey M, Schulz A, Aristorena M, Miller N, Kingdom U, et al. Mutation update: Review of. :0–3. 3. Sims KB, Friedman J. Clinical / Scientiﬁc Notes. 2015;1–4. 18 Novel Recessive NDUFS3 Mutation Causing Leigh’s Syndrome with Dystonia, Tremor and Ataxia Brandon Barton (Chicago, IL, United States), Camilo Toro (Bethesda, MD, United States) Objective: Report discovery of a mitochondrial mutation with well documented progression over 20 years. Background: A 26-year-old man was followed chronically for chronic tremor and gait imbalance. After normal birth and development, at 5 years old he subacutely developed falls while running. Ataxia workup found deviation of the left eye, left arm intention tremor, and unsteady gait with poor tandem. Extensive laboratory work including common mitochondrial mutations was negative except for lactic acidosis. MRI brain showed two foci of high signal in lateral medulla and mild vermis atrophy. Over several weeks he recovered gradually and by age 12 was reported to be normal, though he had learning disability and short stature. At age 18 he developed head and hand tremor, and repeat evaluation found both truncal and appendicular mild ataxia, dystonic neck tremor, and action/intention tremor in the hands. There was diffuse mild slowness, slow vertical saccades, and strabismus. MRI showed marked diffuse cerebellar atrophy and bilateral gliosis and some cavitation of the posterior caudate. Retesting was negative, though mitochondrial disease was the chief default diagnosis. His older sister developed similar gait symptoms and dysarthria at age 5, stabilizing with chronic tremor. Two other siblings were normal. He was evaluated by the NIH Undiagnosed Diseases Program at age 21. Methods: Case report and review of literature. Results: After extensive analysis the NIH program discovered that the syndrome was most likely due to a mild complex-I deﬁciency due to mutations in the gene NDUFS3 (NADH-ubiquinone oxidoreductase Fe-S Protein 3), reported previously in few patients. This is an autosomal recessive mutation affecting the ﬁrst enzyme complex in the electron transport chain of mitochondria, coding for subunits essential for MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS catalyzing electron transfer from NADH to ubiquinone and for the generation of the proton motive force. These genes highly conserved across species. The mutations for this patient are previously unreported: one of his mutations was intronic affecting splicing, the other was a coding mutation that created a new cryptic splice site that resulted in a muchshortened transcript. The abnormal splice products in his ﬁbroblasts compared to controls. Conclusions: The case is consistent with a “mild” Leigh’s phenotype given the later and longer clinical course and adds to the growing description of phenotypes of rare complex-1 mutations. While mitochondrial disease is often suspected, obtaining a speciﬁc genetic diagnosis is elusive without access to specialty genetic laboratories. 19 Prevalence and Distribution of Autosomal Dominant Spinocerebellar Ataxia at the University of Miami Jason Margolesky (Miami, FL, United States), Elizabeth Jordan (Miami, FL, United States), Sarah Marmol (Miami, FL, United States), Matthew Feldman (Miami, FL, United States), Danielle Shpiner (Miami, FL, United States), Corneliu Luca (Miami, FL, United States), Henry Moore (Boca Raton, FL, United States), Carlos Singer (Fort Lauderdale, FL, United States) Objective: To assess the prevalence of autosomal dominant Spinocerebellar Ataxia (SCA) genotypes seen in the University of Miami Movement Disorders Clinic, a referral center for Florida, Latin America, and the Caribbean. Background: SCAs include almost 50 genetically deﬁned conditions (SCA1-48; DRPLA) with varying phenotypes, but all with ataxia due degeneration of the cerebellum and cerebellar pathways and an autosomal dominant inheritance pattern. Methods: We utilized the “Slicer Dicer” function of Epic (our electronic medical record system) to search for diagnostic terms “spinocerebellar ataxia,” “hereditary ataxia,” and/or “cerebellar ataxia” used by any of 5 Movement Disorder specialists between 2014 to 2019. Patient charts were reviewed for genetic testing results, and only patients with genetically deﬁned SCAs were included. Results: Of 9964 total patients seen during this time period, 241 patients carried the ICD 9 or ICD 10 diagnostic codes for, “spinocerebellar ataxia (n=26),” “cerebellar ataxia” (n=233) and/or “hereditary ataxia” (n=163). Of these 241 patients, 24 had a diagnosis of SCA conﬁrmed through genetic testing. SCA2 (9/24; 37.5%) was the most prevalent; 3 additional Cuban patients from Holguin were seen and suspected to have SCA2, but this was not conﬁrmed with genetic testing. In decreasing order of prevalence, the next most common diagnoses were SCA8 (5; 20.8%), SCA3 (2; 8.3%), SCA5 (2; 8.3%) and SCA7 (2; 8.3%). There was one case each of SCAs 10, 28, 29, and 42. Conclusions: The prevalence of the different SCAs varies by geographic region and patient ethnicity. For example, SCA2 is the most prevalent SCA in patients of Cuban ancestry, particularly from the province of Holguin1. Despite limitations of this retrospective review, and considering that only 10% of the cases extracted had genetic conﬁrmation, SCA2 seems to be the most prevalent SCA in our institution. This may be explained by the high prevalence of Cuban ancestry in the population of South Florida. References: Teive HAG, Meira AT, Camargo CHF, Munhoz RP. The Geographic Diversity of Spinocerebellar Ataxias (SCAs) in the Americas: A Systematic Review. Disorders ? Clinical Practice 2019; 6: 531-40. 20 Atypical Presentation of a Patient with SCA-2 Gabriela Ziegler (Ciudad Autonoma de Buenos Aires, Argentina), Pavel Hernandez (Buenos Aires, Argentina), Sergio Rodriguez Quiroga (Buenos Aires- Capital Federal, Argentina), Tomoko Arakaki (CABA, Argentina), Lucia Zavala (Buenos Aires, Argentina), Joseﬁna Perez Maturo (CABA, Argentina), Nancy Medina (CABA, Argentina), Marcelo Kauffman (CABA, Argentina), Nélida Garretto (Buenos Aires, Argentina) Objective: To describe a late onset ataxic syndrome in a Spinocerebellar Ataxia Type 2 (SCA-2) patient. Background: SCA-2 is an autosomal dominant cerebellar ataxia caused by an abnormal CAG repeat expansion in the ataxin-2 gene (=33 repeats). Age of onset is typically in the fourth decade. Low numbers of CAG repeats are seen in late onset cases. Methods: We describe a 57-year-old man with a 7-year-history of a cerebellar syndrome with molecular diagnosis of SCA2. Results: Patient was examined regularly at clinics for three years for slowly progressive gait and stance problems. He described mild gait disorders in his mother. He had minor and occasional swallowing difﬁculties with solids, but reported no falls or speech disturbances. His initial SARA scale was 6, currently rating 8. He had slightly decreased horizontal saccadic eye movement’s velocity. Routine laboratory tests including vitamin E and antiGAD antibodies were normal. EMG revealed a sensitive axonal polyneuropathy in the lower limbs. Brain MRI revealed slight cerebellar atrophy. Molecular tests for SCA-1, 2 and 3 was performed. Molecular analysis showed an abnormally expanded 40 CAG repeats in the ATXN2 gene consistent with a SCA-2. Conclusions: We describe a SCA2 patient with a 50 y.o. onset, predominantly cerebellar syndrome and 40 CAG repeats in the expanded allele. Large variation in age at onset for particular CAG repeat numbers has been demonstrated in SCA2 and other polyglutamine disorders. Nonetheless, CAG repeat length has proven to be the main determinant for age at onset in SCA2. As has been previously described in the literature an earlier age of onset would be expected in relation with repeat length in this case. Secondary unidenti?ed genetic and environmental factors may account for later age at onset. This case highlights that SCA2 is a diagnosis to be considered in late onset hereditary ataxia. References: 1. A Antenora, F Sacca, C Rinaldi, A Roca, C Pane, M Lieto, S Peluso, G De Michele, A Filla. Annals of Clinical and Translational Neurology 2017; 4(9): 687–695. 2. L Almaguer-Mederos, N Falcon, Y Zaldivar, Y Almira, E Gongora, D Almarales, M Herrera, K Batallan, R Armiñan, M Manresa, J Laf?ta-Mesa, G Cruz, V Chang, T Cyuz, S Gispert, G Auburger, L Perez. Estimation of the age at onset in spinocerebellar ataxia type 2 Cuban patients by survival analysis. Clin Genet 2010: 78: 169–174. Behavioral disorders 49 The Impact of Deep Brain Stimulation in Parkinson’s Disease for Depression, Quality of Life, Sctivities of Daily Living, and Subjective Memory Feruzjon Rakhimov (Tashkent, Uzbekistan) Objective: In the ﬁeld of Parkinson disease (PD) research, many studies have shown that deep brain stimulation (DBS) can soften side effects, which arise during long-term medical therapy. This study focuses on the changes in depressive symptoms, quality of life (with the subdivisions physical and mental health), activities of daily living, and subjective memory functioning in PD patients testing the baseline and the outcome 1 year after DBS. Methods: For the ﬁrst time, the reliable change index (RCI) methodology was applied to compare PD-DBS patients (n = 22) with best medically treated PD patients (PD-BMT; n = 28), subjects with mild cognitive impairment (MCI, n = 43) and healthy controls (n = 25) in the above-mentioned domains. The used questionnaires included the revised Beck Depression Inventory (BDI-II), the Short Form (36) Health Survey (SF-36), the Bayer Activities of Daily Living Scale (B-ADL), and the Forgetfulness Assessment Inventory (FAI). Results: The reliable change indices show high constant or improved results of the PD-DBS patients in the domains subjective memory (85.7%-100.0%), activities of daily living (60.0%-90.0%), physical health summary (77.8%), depressive symptoms (61.9%), and mental health summary (50.0%) in comparison with the PD-BMT, MCI, and control group. Conclusions: DBS is an established alternative to best medical treatment of PD. The comparisons between the PD-DBS and PD-BMT groups do suggest that the domains mental health, depressive symptoms, and physical health beneﬁt most, while the domains activities of daily MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S15 ABSTRACTS living and subjective memory functioning are rather constant. Nevertheless, further research is needed to identify mechanisms and predictors that lead to improvement in individual cases. Choreas (Non-Huntington’s Disease) 25 Huntington Disease (HD)-Like Presentation of Spinocerebellar Ataxia 17 (SCA17) in a Patient with 45 CAG Repeats Chandler Gill (Chicago, IL, United States), Jori Fleisher (Chicago, IL, United States), Mitra Afshari (Chicago, IL, United States) Objective: To describe a case of SCA17 with intermediate-range CAG repeat expansion presenting with chorea, ataxia, psychosis, and dementia. Background: SCA17 is a rare autosomal dominant cerebellar ataxia due to CAG repeat expansion in TATA-box binding protein (TBP) gene causing transcription deregulation. Expansions in the intermediate range of 41-49 repeats are expressed in 50-80% of cases. Methods: Case report of a 59 year-old woman with a history of right temporal astrocytoma s/p resection/radiation complicated by localizationrelated epilepsy and bipolar disorder was referred for 3 years of involuntary movements. Family reported chronic cognitive impairment due to remote astrocytoma treatment, recently progressive, and new complex hallucinations and delusions without mania or depression. Further history revealed 1 month of prior neuroleptic exposure, remote cocaine abuse, 60 packyear smoking history, and unintentional weight loss. Her parents were nonconsanguineous of English and French Canadian descent. There was no family history of similar symptoms (Figure 1), although paternal history was largely unknown due to estrangement. [ﬁgure1] Results: Her examination was notable for disinhibition, deﬁcits in attention, language, abstraction, and delayed recall (MOCA 16/30), dysarthria, delayed initiation and slowing of saccades, gaze impersistence and jaw thrust. She had diffuse chorea, most prominent in the neck, trunk, and arms. Other ﬁndings were, dysmetria, dysdiadochokinesis, hyperreﬂexia, and ataxic gait. Peripheral smear, TSH, ANA, HIV, antiphospholipid, anti-GAD and antigliadin antibodies, body CT/PET and serum/CSF paraneoplastic panel were negative. MRI showed stable right temporal encephalomalacia and gliosis, and cerebellar atrophy. Genetic testing showed normal HTT and C9ORF72 genes, but heterozygous CAG repeat expansion in TBP gene (37 and 45 repeats). Conclusions: SCA17 represents about 1% of HD-like syndromes; symptoms include ataxia, dementia, parkinsonism, chorea, psychosis, or seizures. To date approximately 100 families have been identiﬁed although it is likely underdiagnosed. We report a case which presented a S16 particular diagnostic challenge due to a complicated neurologic and psychiatric history and baseline abnormal exam; although she had baseline cognitive impairment and bipolar disorder, her symptoms of new progressive cognitive decline, hallucinations and delusions were new. Longer follow-up studies are needed to deﬁne the factors that inﬂuence clinical heterogeneity in SCA17; the combination of chorea, ataxia and hallucinations should prompt consideration of this diagnosis. References: 1. Nethisinghe S, Lim WN, Ging H, Zeitlberger A, Abeti R, Pemble S, et al. Complexity of the genetics and clinical presentation of Spinocerebellar Ataxia 17. Front Cell Neurosci 2018; 12:429.2. Stevanin G, Brice A. Spinocerebellar ataxia 17 (SCA17) and Huntington’s disease-like 4 (HDL4). Cerebellum 2008;7(2):170-8. 3. Shin JH, Park H, Ehm GH, Lee WW, Yun JY, Kim YE, et al. The pathogenic role of low range repeats in SCA17. PLoSONE 2015; 10(8): e0135275. doi:10.1371/journal.pone.01352754. Nakamura K, Jeong S. SCA17, a novel autosomal dominant cerebellar ataxia caused by an expanded polyglutamine in TATA-binding protein. Hum. Mol. Genet 2001; 10:1441–1448. 5. Bauer P, Laccone F, Rolfs A, Wullner U, Bosch S, Peters H, et al. Trinucleotide repeat expansion in SCA17/TBP in white patients with Huntington’s disease-like phenotype. J Med Genet 2004; 41:230–232. 6. Toyoshima Y, Yamada M, Onodera O, Shimohata M, Inenaga C, Fujita N, et al. SCA17 homozygote showing Huntington’s disease-like phenotype. Ann Neurol 2004; 55:281–286. 26 Case Report: Pseudoatetosis as Manifestation of the Vitmain B12 Deﬁcit Juan Vargas Jaramillo (Bogota, Colombia) Objective: Subacute combined degeneration (DSAC) of the spinal cord is a classic manifestation in the nervous system of vitamin B12 deﬁciency, clinically characterized by sensory ataxia due to the demyelinating commitment of thick ﬁbers in the posterior cords, which can lead to disorders of movement secondary to proprioceptive loss such as pseudoatetosis occurs in this case.1 Background: A 72-year-old patient with a 2-year history that begins with paraesthesia of the 4 extremities, progresses to hypoaesthesia in the hands, later in the feet associated with burning pain. Parallel to this he presented an unintended loss of 7kg the 6 months prior to his admission to the emergency room, as well as clumsiness in his hands and deterioration of the march that manifested as a slowing in the ambulation of which the patient refers to as a sensation of “walking between the clouds” that ended up restricting the march completely. 2 months before I was in an outpatient study due to ﬁndings of macrocytic anemia. Methods: Blood count showed the presence of leukopenia with 3,890 whites, macrocytic anemia with hemoglobin of 8.7g/dl, high MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS MCV of 110 ﬂ. and mild thrombocytopenia 115,700mm3. Reduced vitamin B12 of 83 pg / ml and normal folic acid. EVDA biopsies with atrophic gastritis.Neuroconduction studies showed axonal sensory neuropathy of the 4 extremities, absence of the H reﬂex in the lower limbs with complete preservation of motor responses and F wave, suggestive of Ganglionopathy. MRI of the cervical-dorsal cord was carried out, showing marked hyperintensity of the dorsal horns throughout its entire length, with the presence of the inverted “V” sign, compatible with Subacute Combined Degeneration.The patient was treated with cyanocobalamin at a dose of 1000μg intramuscularly per day for 1 week, to continue with monthly doses for 6 months, at 4 months the patient has resolved involuntary movements and gait recovery. Results: Vitamin B12 deﬁciency is a cofactor of the enzymes: Lmethyl-malonylCoA mutase and methionine synthase, the dysfunction of these generates an abnormal insertion of even chain fatty acids, forming defective and unstable myelin sheaths.2 The selective demyelination of the posterior cords causes defects in proprioception and results in “pseudoatotic” movements that refer to the inability to keep the ﬁngers in a ﬁxed position when displaced by continuous involuntary movements, which differ from athetosis, due to its origin in sensitive deafferentation. 3 Conclusions: Vitamin B12 deﬁciency generates severe hematological and neurological complications, being an uncommon cause of myelopathy. Pseudoatetosis is a reversible sign of long-standing proprioceptive deﬁcit. The DSAC due to vitamin B12 deﬁciency causes severe disability and is easily treatable, so it should always be considered in differential myelopathy diagnoses. References: Cao J, Su ZY, Xu SB, Liu CC. Subacute Combined Degeneration: A Retrospective Study of 68 Cases with Short-Term Follow-Up. Eur Neurol. 2018;79(5-6):247-55.Stabler SP. Clinical practice. Vitamin B12 deﬁciency. N Engl J Med. 2013;368(2):149-60. Healton EB, Savage DG, Brust JC, Garrett TJ, Lindenbaum J. Neurologic aspects of cobalamin deﬁciency. Medicine (Baltimore). 1991;70(4):229-45. pharmacokinetic model. Area under the concentration time curves over the dosing interval (AUC0-12) were calculated dividing dose by CL/F. Results: 166/170 study participants randomized to isradipine treatment arm had plasma isradipine concentrations measured. A twocompartment model, coupled to a transit absorption model, best described the isradipine plasma-concentration data. Increasing age was associated with decreasing CL/F. Population estimates [95% conﬁdence interval] of CL/F was 292 [265 – 319] liters/hour (L/h) and for central volume of distribution (Vc) was 895 [509 – 1281] liters. Interindividual variability in CL/F and Vc were 46% and 120%, respectively. The median (range) AUC0-12 was 16.05 (3.4 – 41.4) ng/mL*h. Conclusions: Isradipine plasma exposures were associated with substantial interindividual variability in the STEADY-PD3 trial cohort. Average estimates of isradipine CL/F from prior pharmacokinetic studies have ranged from 122 to 497 L/h in non-PD populations, with typical estimates approximating 300 L/h.3-4 Our model demonstrated that age affected isradipine pharmacokinetics, with increasing age leading to higher plasma isradipine exposures (decrease in CL/F). With model-based individual estimates of isradipine exposures available, exposure-response analyses are underway and will be presented. References: 1.Ilijic E, Guzman JN, Surmeier DJ. The L-type channel antagonist isradipine is neuroprotective in a mouse model of Parkinson’s disease. Neurobiol Dis. 2011 Aug;43(2):364-71. PubMed PMID: 21515375.2. Guzman JN, Ilijic E, Yang B, Sanchez-Padilla J, Wokosin D, Galtieri D, Kondapalli J, Schumacker PT, Surmeier DJ. Systemic isradipine treatment diminishes calcium-dependent mitochondrial oxidant stress. J Clin Invest. 2018 Jun 1;128(6):2266-2280. PubMed PMID: 29708514.3. Johnson BA, Javors MA, Lam YW, Wells LT, Tiouririne M, Roache JD, Ait-Daoud N, Lawson K. Kinetic and cardiovascular comparison of immediate-release isradipine and sustainedrelease isradipine among non-treatment-seeking, cocaine-dependent individuals. Prog Neuropsychopharmacol Biol Psychiatry. 2005 Jan;29 (1):15-20. PubMed PMID: 15610940.4. Wang Y, Jin Z, Wang Z, Jiang X, Wang L. Pharmacokinetic properties of isradipine after singledose and multiple-dose oral administration in Chinese volunteers: a randomized, open-label, parallel-group phase I study. Biomed Chromatogr. 2013 Dec;27(12):1664-70. PubMed PMID: 23813620. Clinical Trials and Pharmacology 30 29 Pharmacokinetics of Isradipine in Participants with Parkinson’s Disease from the Phase 3 STEADY-PD Clinical Trial Charles Venuto (Rochester, NY, United States), D. James Surmeier (Chicago, IL, United States), Arthur Watts (Rochester, NY, United States), Kevin Biglan (Indianapolis, IN, United States), Robert Hauser (Tampa, FL, United States), Sue Henderson (Rochester, NY, United States), Karen Hodgeman (Rochester, NY, United States), Robert Holloway (Rochester, NY, United States), Elise Kayson (Rochester, NY, United States), Daniel Kinel (Rochester, NY, United States), Anthony Lang (Toronto, ON, Canada), Codrin Lungu (Rockville, MD, United States), Jillian Lowell (Rochester, NY, United States), David Oakes (Rochester, NY, United States), Saloni Sharma (Rochester, NY, United States), Ira Shoulson (Longboat Key, FL, United States), Christopher Tarolli (Rochester, NY, United States), Tatyana Simuni (Chicago, IL, United States) Objective: To describe the pharmacokinetics of isradipine in STEADY-PD3 study participants and to evaluate associations between isradipine exposures with study outcomes. Background: Isradipine is a dihydropyridine calcium channel inhibitor demonstrating concentration-dependent protective effects in animal models of PD.1-2 A phase 3, randomized, double-blinded, placebocontrolled 36 months trial recently concluded that Isradipine IR 5 mg twice daily did not slow the clinical progression in early Parkinson’s disease (PD) participants (STEADY-PD3). As an exploratory objective, plasma samples were collected to characterize isradipine pharmacokinetics. Methods: Plasma isradipine concentrations were collected at entry, and 3 and 6 months post-baseline visit from all STEADY-PD3 participants. Isradipine plasma concentration-time data were analyzed using nonlinear mixed effects modeling, and one- and two-compartment pharmacokinetic models were evaluated. Individual parameter estimates of isradipine apparent oral clearance (CL/F) were estimated from the ﬁnal A Phase 2 Study of the Efﬁcacy, Durability, and Safety of Ampreloxetine (TD-9855), a Norepinephrine Reuptake Inhibitor, Given Once-Daily to Treat Symptomatic Neurogenic Orthostatic Hypotension Horacio Kaufmann (New York, NY, United States), Italo Biaggioni (Nashville, TN, United States), Ashok Panneerselvam (Nashville, TN, United States), Brett Haumann (Nashville, TN, United States), Ross Vickery (South San Francisco, CA, United States) Objective: To assess the efﬁcacy, durability, and safety of once-daily oral ampreloxetine for the treatment of symptomatic neurogenic orthostatic hypotension (nOH) in an open-label, phase 2 multicenter study. Background: Autonomic failure results in nOH with inadequate increase in synaptic norepinephrine and blood pressure in the upright position. Ampreloxetine is a novel norepinephrine reuptake inhibitor for the treatment of symptomatic nOH. Methods: Responders to single-dose, oral ampreloxetine were eligible to receive open-label ampreloxetine (3-20 mg) once daily for up to 20 weeks, with 4-week follow-up after stopping ampreloxetine and restarting alternative pressor agents (eg, midodrine and/or droxidopa). Primary efﬁcacy was assessed using Item 1 of the Orthostatic Hypotension Symptom Assessment (OHSA#1; dizziness, lightheadedness, feeling faint; symptomatic: score >4/10). Other endpoints to assess symptom improvement included OHSA and Orthostatic Hypotension Daily Activities Scale (OHDAS) composite scores and patient-reported impression of symptom severity and symptom change (PGI-S, PGI-C). Efﬁcacy was assessed at the end of Week 4; durability, Week 20; and follow-up, Week 24. Results: Of 21 subjects enrolled, 17 were symptomatic at baseline (mean age, 65 years). Symptom improvement on OHSA#1 was observed as early as Week 1, with a mean (SD) reduction in score of -3.8 (3.1) at Week 4 and -3.1 (3.0) at Week 20. OHSA #1 scores reverted to baseline MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S17 ABSTRACTS on stopping ampreloxetine and restarting alternative pressor agents. Similar trends were seen in OHSA and OHDAS composite scores, PGI-S, and PGI-C, with durable symptom improvement on ampreloxetine treatment and symptom deterioration on discontinuation. Most common adverse events (AEs) were urinary tract infection (24%), hypertension (19%), and headache (14%). Two subjects (10%) discontinued treatment due to AEs; 5 (24%) had serious AEs, none considered related to study drug. Conclusions: In subjects with nOH, ampreloxetine demonstrated clinically meaningful improvement in symptoms of nOH at Week 4, which was sustained over 20 weeks, and reverted to baseline after discontinuation of ampreloxetine and reinitiation of alternative pressor agents. Ampreloxetine was well tolerated with a favorable safety proﬁle. United States), Esther Cubo Delgado (Burgos, Spain), Marieta AncaHerschkovitsch (Shoham, Israel), Robert Iansek (Cheltenham, VIC, Australia), Mustafa Siddiqui (Winston-Salem, NC, United States), Mihaela Simu (Timisoara, Romania), Lars Bergmann (Ludwigshafen, Germany), Pavnit Kukreja (North Chicago, IL, United States), Weining Robieson (Hawthorn Woods, IL, United States), K. Ray Chaudhuri (London, United Kingdom) Objective: Evaluate the effect of levodopa-carbidopa intestinal gel (LCIG) on dyskinesia symptoms as well as quality of life (QoL) and caregiver burden in a multicountry observational study in advanced Parkinson’s disease (aPD) patients treated with LCIG in routine clinical practice. 31 Effects of Once-Daily Ampreloxetine (TD-9855), a Norepinephrine Reuptake Inhibitor, on Blood Pressure in Subjects with Symptomatic Neurogenic Orthostatic Hypotension Horacio Kaufmann (New York, NY, United States), Italo Biaggioni (Nashville, TN, United States), Ashok Panneerselvam (Nashville, TN, United States), Brett Haumann (Nashville, TN, United States), Ross Vickery (South San Francisco, CA, United States) Objective: To evaluate the relationship between symptom improvement and blood pressure regulation in subjects with symptomatic neurogenic orthostatic hypotension (nOH) treated with open-label ampreloxetine. Background: In nOH subjects, blood pressure (BP) falls due to inadequate release of norepinephrine (NE) when upright. Ampreloxetine, a novel NE reuptake inhibitor, has shown efﬁcacy and durability in symptom improvement in subjects with nOH. Methods: In a phase 2, multicenter study, subjects received ampreloxetine once-daily (3–20 mg) for up to 20 weeks, with a 4-week follow-up after stopping ampreloxetine and restarting other pressor agents (midodrine and/or droxidopa). Symptom assessment was Orthostatic Hypotension Symptom Assessment Item 1 score (OHSA#1; dizziness, lightheadedness, feeling faint; symptomatic: score >4/10). BP regulation assessments were standing, sitting, and supine systolic BP (SBP), standing duration, and plasma NE. Efﬁcacy was assessed at end of Week 4; durability, Week 20; follow-up, Week 24. Results: 17 subjects were symptomatic at baseline (mean age 65 yrs [range, 51–83 yrs]). Standing and sitting SBP, standing duration, plasma NE, and symptoms of nOH improved from Weeks 1 to 20. Mean increase in 3-minute standing SBP from baseline was 7.6 mmHg at Week 4. Through Week 20, 67–78% of subjects had standing SBP >80 mmHg. Changes in sitting SBP were less with little change in supine SBP. At Week 4, 67% of subjects could stand for >5 mins, an improvement of 31% from baseline. NE plasma levels rose from pre-dose to Week 4 (2275.7–3395.9 pmol/l). Standing, sitting, and supine SBP increased after stopping ampreloxetine and restarting other pressor agents; conversely, symptoms deteriorated to baseline. Ampreloxetine was generally well tolerated. Conclusions: This phase 2 open-label trial of ampreloxetine demonstrated efﬁcacy and durability in subjective and objective assessments of nOH. Symptom improvement on ampreloxetine treatment was accompanied by increase in standing and sitting SBP, standing duration, and NE plasma levels. Symptoms deteriorated to baseline after stopping ampreloxetine, despite sitting and standing SBP being maintained on restarting other pressor agents. Ampreloxetine had little effect on supine SBP throughout the study. 32 Improvements in Dyskinesia with Levodopa-Carbidopa Intestinal Gel in Advanced Parkinson’s Disease Patients in a ‘Real-World’ Study: Interim Results of the Multinational DUOGLOBE Study With up to 24 Months Follow-Up David Standaert (Birmingham, AL, United States), Norbert Kovács (Pécs, Hungary), Francesco Pontieri (Roma, Italy), Jason Aldred (Spokane, WA, United States), Paul Bourgeois (Kortrijk, Belgium), Thomas Davis (Nashville, TN, S18 Background: As PD progresses, chronic oral levodopa therapy can be associated with disabling motor complications including wearing off and MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS dyskinesia. LCIG has established beneﬁt in reducing “Off” time, but prospective long-term data on the effect of LCIG on dyskinesia symptoms and associated effects on QoL and caregiver burden in a real-world setting are limited. Methods: DUOGLOBE is a prospective multinational observational study (including US sites) of LCIG naïve patients treated as part of routine clinical practice with 3-years follow-up planned (NCT02611713). This is the ﬁrst multinational LCIG study using the Uniﬁed Dyskinesia Rating Scale (UDysRS), a novel validated scale for dyskinesia. Other assessments included the UPDRS Part IV, “Off” time, Non-Motor Symptoms Scale (NMSS), QoL (8-item PD questionnaire [PDQ-8]), Modiﬁed Caregiver Strain Index, and Serious Adverse Events (SAEs). Interim outcomes from baseline up to month (M) 24 are presented. Results: In this interim analysis, 196 patients were included (62% male, 78% =65 years old; 51% =10 years’ PD duration. Median daily duration of LCIG infusion was 16.0 h/d LCIG through M24, with up to 9% of patients on 24h LCIG infusion. Signiﬁcant improvements (mean change from baseline to M24) were observed in “Off” time (-3.6 h/d) and NMSS total scores (-27.0). LCIG treatment signiﬁcantly improved dyskinesia symptoms and signs assessed by the UDysRS and UPDRS Part IV items 33 and 34 through M18 [Figure 1]. QoL and caregiver burden [Figure 2] were also improved through M18. Overall, 41% of patients experienced SAEs [Table 1]; 31% (n=60) discontinued participation in DUOGLOBE with 13 patients continuing LCIG outside the study. Conclusions: This interim analysis shows sustained improvements of dyskinesia symptoms and signs, measured using the UDysRS, with LCIG in routine clinical practice and supports the real-world effectiveness of LCIG on “Off” time, NMS, QoL, and caregiver burden in aPD patients. Safety was consistent with the established LCIG proﬁle. 33 Utilization of Monotherapy and Combination Therapies in Advanced Parkinson Disease Patients During LevodopaCarbidopa Intestinal Gel Treatment from the COSMOS Study Alfonso Fasano (Toronto, ON, Canada), Juan Parra Riaza (Madrid, Spain), Tanya Gurevich (Tel-Aviv, Israel), Robert Jech (Prague 7, Czech Republic), Norbert Kovács (Pécs, Hungary), Per Svenningsson (Stockholm, Sweden), Jozsef Szasz (Targu Mures, Romania), Lars Bergmann (Ludwigshafen, Germany), Anita Johnson (North Chicago, IL, United States), Olga Sanchez-Soliño (Madrid, Spain), Zhongwen Tang (North Chicago, IL, United States), Lydia Vela (Pozuelo De Alarcon, Madrid, Spain) Objective: Assess the percentages of advanced Parkinson’s disease (APD) patients treated with levodopa-carbidopa intestinal gel (LCIG) as a monotherapy or with add-on PD medications and the reasons for changes in add-on PD medications throughout LCIG treatment. Background: This is the ﬁrst study fully dedicated to collecting realworld data assessing add-on PD medication use with LCIG during longterm therapy in a large patient cohort. Methods: COSMOS is a multi-country, retrospective, cross-sectional, post-marketing observational study (NCT03362879). Main inclusion criteria were APD patients treated for at least 12 months by the same physician since LCIG initiation. Patients were grouped into LCIG monotherapy (LCIG was the only treatment for patient´s PD), LCIG monotherapy during infusion hours (allowing add-on PD medications [eg, oral or transdermal] only after LCIG infusion was stopped), and those on LCIG + add-on therapy. Study assessments were obtained at the patient visit and collected retrospectively including if patients were on monotherapy and descriptions of add-on PD medications and LCIG infusion settings. Results: This real-world study included 409 LCIG treated APD patients from 49 clinics in 14 countries. Patient demographics and baseline disease characteristics are presented in Table 1. Mean LCIG treatment duration at the study visit was 35.7 (range: 12.0-139.3) months with 9.7% of patients (n=39) on 24h/day LCIG infusion. Preliminary data indicate that overall, the percentage of patients on LCIG monotherapy and LCIG monotherapy during infusion hours increased from LCIG initiation to month 12 while the percentage of patients on LCIG + add-on therapy decreased at month 12 (Table 2). In all groups, a majority of patients discontinued the use of any add-on PD medication at the initiation of LCIG treatment. Safety data are currently being analyzed and will be included in the presentation. Conclusions: Analyses are ongoing; however, these preliminary data indicate that during routine clinical practice LCIG treatment can be initiated and used long term in APD patients with many patients well controlled using LCIG monotherapy. Furthermore, for patients not on MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S19 ABSTRACTS monotherapy, LCIG provides important reductions in add-on PD medications. This abstract was presented at the 2019 International Congress of Parkinson’s Disease and Movement Disorders in September 2019. 34 Percutaneous Gastrojejunostomy Tubing Utilization and Safety with Levodopa-Carbidopa Intestinal Gel in Advanced Parkinson’s Disease Patients: Interim Results of the DUOGLOBE Observational Study Peter Draganov (Gainesville, FL, United States), C Mel Wilcox (Birmingham, AL, United States), Michelle Lee (North Chicago, IL, United States), Weining Robieson (Hawthorn Woods, IL, United States), Pavnit Kukreja (North Chicago, IL, United States), Lars Bergmann (Ludwigshafen, Germany), Shajan S20 Peter (Birmingham, AL, United States), Kenneth Symington (Spokane, WA, United States) Objective: To investigate the routine clinical practice of percutaneous gastrojejunostomy(PEG) with a jejunal(J) extension tube insertion and component replacements of the device and associated safety. Background: Levodopa-carbidopa intestinal gel(LCIG, carbidopalevodopa enteral suspension in US) administered via PEG-J and an external pump is a long-term treatment option for patients with advanced Parkinson’s disease(aPD). Limited data exists on PEG-J component utilization/replacements during LCIG treatment in a ‘real-world’ setting. Methods: DUOGLOBE is an ongoing multinational observational study with 3-year follow-up. This interim analysis included aPD patients who had the PEG-J insertion procedure from 55 global sites. The type of tubing used (AbbVie proprietary tubing [proprietary company tubing] vs other tubing types, frequency of and reasons for tube replacements, and Serious Adverse Events (SAEs) were assessed. This analysis was not powered for statistical comparisons. Results: This interim dataset had 188 patients with PEG-J tube placement (mean duration of PEG-J exposure=515.8 days) and178 patients with PEG-J tubing. At initial PEG-J placement, 83%(n=148) of patients received proprietary company tubing and 17%(n=30) received other tubing types. Most patients (79%,n=141) did not require PEG-J replacement. Patients needing any PEG-J replacement was similar between proprietary company tubing and other tubing types(22%/17%); 5% of patients(n/ N=7/148) with proprietary company tubing required 2 PEG-J replacements(other tubing types,n/N=0/30). In the ﬁrst 12 months, Kaplan-Meier estimates showed 84% of patients did not require tube replacement with proprietary company tubing (other tubing types,89%). Most common reasons for ﬁrst PEG-J replacement for proprietary company tubing was routine preplanned procedure(6%), PEG-J dislocation (4%), occlusion and leaking(each 2%), and other(5%); for other tubing types PEG-J dislocation(10%) was the only reported reason. Overall, 38% of patients experienced an SAE with proprietary company tubing(n=56) and 47% for other tubing types(n=14). Gastrointestinal(GI)-related SAEs were reported in 8(5%) patients with proprietary company tubing and 6 (20%) patients with other tubing types[6 (20%)]; 1(1%) and 2(7%) patients discontinued due to GI-related SAE, respectively. Conclusions: This interim analysis demonstrated that most patients did not require PEG-J tube replacements in a real-world setting and safety was similar to LCIG’s known safety proﬁle. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS 35 Efﬁcacy and Safety of Levodopa-Carbidopa Intestinal Gel in Advanced Parkinson’s Disease Patients Stratiﬁed by Baseline Hoehn and Yahr Stage: Data from the DUOGLOBE Study Jason Aldred (Spokane, WA, United States), James Boyd (Burlington, VT, United States), Lars Bergmann (Ludwigshafen, Germany), Pavnit Kukreja (North Chicago, IL, United States), Lily Yu (North Chicago, IL, United States), Esther Cubo Delgado (Burgos, Spain), Norbert Kovács (Pécs, Hungary) Objective: To assess the impact of baseline disease severity on the efﬁcacy of levodopa-carbidopa intestinal gel (LCIG, carbidopa-levodopa enteral suspension, in the US) on motor ﬂuctuations, non-motor symptoms (NMS), axial symptoms, and quality of life (QoL) in patients with advanced Parkinson’s disease (aPD) in routine clinical practice. Background: There is limited data on the efﬁcacy of LCIG in routine clinical practice on motor, NMS, and QoL in patients with low or high disease severity. Methods: In this interim analysis of the multinational, post-marketing observational study, DUOGLOBE, patients were stratiﬁed into two groups: baseline H&Y stage <3 and >=3. Outcomes included the mean change from baseline to month (M) 12 or 18 of LCIG treatment in OFF time, dyskinesia (UDysRS total score), axial symptoms (UPDRS Part III: sum of items 18, 22, 27-30), NMS (NMSS total score), and QoL(PDQ8 summary index) for patients who had completed indicated follow-up visits as of December 2018. Serious Adverse Events (SAEs) were monitored. Results: This interim analysis included a total of 188 patients with baseline demographics and disease characteristics presented in [Table 1]. At M18 of LCIG treatment, OFF time was signiﬁcantly reduced by -3.3 and -3.9 hours in <3 and >=3 H&Y stage groups compared to baseline (n=29/52; both, P<.001). UDysRS scores were reduced by -5.6 in the <3 stage group (n=32) and signiﬁcantly reduced by -11.1 in the >=3 stage group (n=37;P<.01). In the >=3 H&Y group, axial symptoms signiﬁcantly improved through M12 (-1.5; n=86;P<.01); axial symptom scores increased by 1.4 at M12 in the <3 group (n=47;P<.05). Improvements through M18 in NMS (-21.4 and -19.9; n=31/48; both P<.01) and QoL (PDQ-8: -10.3 and -6.3; n=33/52; both, P<.05) were observed. Overall, SAEs occurred in 39.9% of patients (n=75); 44.8% (n=26) in the <3 group and 37.7% (n=49) in the >=3 group. Conclusions: This interim analysis suggests that patients, independent of H&Y disease severity, treated with LCIG during routine clinical practice experience important beneﬁts in motor complications, NMS, and QoL and was consistent with the established safety proﬁle of LCIG. Additionally, patients with higher disease severity have notable improvements in axial symptoms. These data suggest that LCIG may be beneﬁcial for motor ﬂuctuations over a wide range of PD stages. 36 An Open-Label, Phase 1b Study of the Neuroactive Steroid GABA-A Receptor Positive Allosteric Modulator SAGE-324 in Essential Tremor James Paskavitz (Cambridge, MA, United States), David Nguyen (Cambridge, United States), Min Qin (Cambridge, MA, United States), Angela Wehr (Cambridge, MA, United States), James Doherty (Cambridge, United States), Stephen Kanes (Cambridge, United States) Objective: This single dose, open-label, Phase 1b study assessed the safety, tolerability, pharmacokinetics (PK), and pharmacodynamics (PD) of SAGE-324 in otherwise healthy Essential Tremor (ET) patients. Background: ET is associated with impaired GABAergic signaling in the cerebellum. SAGE-324 is a next-generation neuroactive steroid GABA-A receptor positive allosteric modulator, with a pharmacology distinct from benzodiazepines in development for ET. Methods: ET patients with a qualifying upper limb tremor by The Essential Tremor Rating Assessment Scale (TETRAS; combined upper limb total score =8 on performance subscale Part 4) and not taking ET medications were enrolled. Patients received a single dose of SAGE-324 solution in 2 dose groups (45 mg or 60 mg) and underwent tremor assessments for 24 hours post-dose as inpatients, using TETRAS and upper extremity accelerometry. Follow-up at Days 7 and 14 was outpatient. Plasma PK parameters were evaluated. Safety and tolerability were assessed by adverse event reporting and standard clinical assessments. Results: SAGE-324 was generally well-tolerated in both dose groups (45 mg, n=6 or 60 mg, n=5); no serious or severe adverse events were reported. Both dose groups demonstrated mean reductions from baseline in tremor as measured by TETRAS (45 mg: 26%; 60 mg: 43%) and accelerometry (45 mg: 48%; 60 mg: 55%) that followed plasma concentrations of SAGE-324. Conclusions: In this Phase 1b study, SAGE-324 was generally well tolerated and demonstrated decreases in tremor. A clear PK/PD relationship was observed. This study supports a future Phase 2 study of SAGE324 in ET. 37 Pharmacokinetics of ND0612 Administered at Different Infusion Sites and with Different Cannula Lengths: An Open-Label, Randomized, Cross-Over Study in Healthy Volunteers Tal Birnberg (Rehovot, United States), Ryan Case (West Chester, PA, United States), Tami Yardeni (Rehovot, Israel), Sheila Oren (Rehovot, Israel), Olivia Rosenfeld (Rehovot, Israel), Liat Adar (Ramat Gan, Israel) Objective: To evaluate the impact of subcutaneous (sc) infusion site location and cannula length on levodopa pharmacokinetics (PK) administered as a single 16-hour sc infusion of ND0612 (levodopa/ carbidopa 60/7.5 mg/mL) in healthy volunteers. Background: ND0612 is under development as a non-surgical drugdevice combination providing continuous sc delivery of levodopa/carbidopa for patients with Parkinson’s disease (PD) experiencing motor ﬂuctuations. The impact of infusion sites location and cannula length on PK, dermal safety and tolerability were assessed to offer more options for patients. Methods: This was an open-label, randomized, single-dose, 4-period, crossover study to assess the effect of the infusion site location and cannula length on the PK of ND0612 infused to 24 healthy subjects (16M/8F). Subjects were randomized 1:1:1:1 to one of four sequences. Each subject sequentially received ND0612 at 3 different infusion sites, with the abdomen infused twice, once with a long cannula (the reference route of administration) and once with a short cannula. The outer thigh and back sites were assessed with long cannula. Each of the 4 individual 16-hour dosing periods were separated by 32-hour washout. Blood samples for PK analysis were collected before, during, and after administration of ND0612 up to 12 hours after the end of infusion. Results: Mean plasma drug concentration-vs.-time proﬁles were similar for ND0612 infused with long cannula at the abdomen (reference) and each of the other treatments (abdomen with short cannula, outer thigh with long cannula and back with long cannula). The 90% conﬁdence intervals for all PK parameters were within the pre-deﬁned bioequivalence limits of 80-125% between all tests and the reference. The most common treatment-emergent adverse event (TEAE) was infusion site nodules. No TEAE led to study discontinuation and none were classiﬁed as serious or severe. Conclusions: This Phase 1 study conﬁrms that both the rate and extent of absorption of ND0612 are similar when administered using different infusion site locations and cannula lengths. Infusion to additional areas of the body may offer more comfortable and various locations for improved long-term ND0612 use. 38 A phase 2 Dose-Escalation and Double-Blind Efﬁcacy Study of Ampreloxetine (TD-9855), a Norepinephrine Reuptake Inhibitor, Given Once-Daily to Treat Symptomatic Neurogenic Orthostatic Hypotension Horacio Kaufmann (New York, NY, United States), Italo Biaggioni (Nashville, TN, United States), Ashok Panneerselvam (Nashville, TN, United States), Brett Haumann (Nashville, TN, United States), Ross Vickery (South San Francisco, CA, United States) Objective: To assess acute effects of single-dose ampreloxetine vs placebo in a 2-part multicenter study in symptomatic neurogenic orthostatic hypotension (nOH). MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S21 ABSTRACTS Background: Autonomic failure results in a fall in blood pressure (BP) in the upright position due to inadequate increase in synaptic norepinephrine (NE). Ampreloxetine is a novel NE reuptake inhibitor for the treatment of symptomatic nOH. Methods: In a 5-day dose-escalation study, subjects received placebo on Day 1, followed by ascending doses of ampreloxetine (Days 2, 3, 4, and 5; 2.5, 5, 10, and 20 mg ampreloxetine, respectively, or highest tolerated dose with pressor response). In a 1-day double-blind efﬁcacy study, subjects were randomized to placebo or ampreloxetine (dose determined from dose-escalation study). Efﬁcacy assessments were change from baseline in seated and standing systolic BP (SBP), and Item 1 of Orthostatic Hypotension Symptom Assessment (dizziness, lightheadedness, feeling faint). Results: Of 34 subjects (mean age, 66 yrs), 2, 2, 1, 15, and 13 subjects received ampreloxetine 1, 2.5, 5, 10, and 20 mg, respectively, as maximum dose. Ampreloxetine 10 mg showed a consistent increase in seated SBP relative to placebo (mean [SD] change in seated SBP 4.9 [20.1] mmHg more than placebo 4 hours post-dose). At 20 mg, the pressor response was variable. In the double-blind study (ampreloxetine, n=5 [median dose, 10 mg]; placebo, n=5), increase in seated SBP was more pronounced for ampreloxetine up to 9 hours post-dose (mean difference from placebo, 29.9 mmHg at 4 hours; p <0.05), and 3-minute standing SBP up to 10 hours post-dose (mean difference, 35.0 mmHg at 4 hours). Twice as many subjects on ampreloxetine had symptom improvement. Most common adverse events were headache and urinary tract infection, with no serious events. Conclusions: In subjects with nOH, 10 mg ampreloxetine produced a consistent increase in seated SBP relative to placebo. Subjects randomized to ampreloxetine (median dose 10 mg) showed signiﬁcantly greater increase in seated SBP vs placebo 4 hours post-dose, with similar effect on standing SBP, and double subject-reported symptom improvement. In general, ampreloxetine was well tolerated. These results support assessment of longer-term effects of ampreloxetine in nOH. 39 Nilotinib Increases Brain Dopamine and Lowers CSF Tau and Oligomeric Alpha-Synuclein in Parkinson’s Disease Fernando Pagan (McLean, VA, United States), Michaeline Hebron (Wsahington, DC, United States), Barbara Wilmarth (Washington, DC, United States), Yasar Torres-Yaghi (Washington, DC, United States), Abigail Lawler (Washington, DC, United States), Elizabeth Mundel (Washington, DC, United States), Nadia Yusuf (Washington, DC, United States), Nathan Starr (Washington, United States), M. Waseem Anjum (Germantown, MD, United States), Shahnaz Miri (Washington, DC, United States), Steven Nakano (Silver Spring, MD, United States), Amelia Carwin (Arlington, VA, United States), Myrna Arellano (Washington, United States), Wangke Shi (Washington, United States), Sanjana Mulki (Washington, United States), Tarick Kurd-Misto (Washington, DC, United States), Sara Matar (Washington, United States), Xiaoguang Liu (Washington, DC, United States), Jaeil Ahn (Washington, United States), Charbel Moussa (Washington, DC, United States) Objective: This is a single center phase II, randomized, double-blind, placebo-controlled study to evaluate the effects of nilotinib, a potentially disease modifying drug in Parkinson’s disease (PD). Background: Our preclinical evidence indicate that nilotinib increases brain dopamine levels and reduces toxic tau and alphaSynuclein via autophagy in models of neurodegeneration. Methods: We predicted that nilotinib alters exploratory biomarkers via inhibition of Abl in the CSF after 12-month daily nilotinib treatment in 3 groups, including placebo (n=21), 150mg nilotinib (n=21) and 300mg nilotinib (n=20). Results: We did not detect any plasma or CSF Abl inhibition. The 150 mg nilotinib group shows an increase in dopamine metabolites homovanillic acid (159.80nM, 90% CI, 7.04-312.60, p=.04) and 3.4-Dihydroxyphenylacetic acid (4.87nM, 90% CI 1.51-8.23, p=.01) and the 300 mg nilotinib group shows an increase in 3-4-Dihydroxyphenylacetic acid (7.52nM, 90% CI 2.35-12.69, p=.01). The 150 mg nilotinib but not the 300 mg group shows reduction of alpha-synuclein oligomers (-0.04pg/ml, 90% CI -0.08- -0.01, p=.03). A signiﬁcant reduction of hyper-phosphorylated tau is seen in the 150 mg S22 nilotinib (-10.04pg/ml, 90% CI -17.41- -2.67, p=.01) and the 300 mg nilotinib (-12.05pg/ml, 90% CI -19.21- -4.90, p=.01) groups. Conclusions: This study met its objectives that nilotinib robustly alters CSF dopamine metabolism and misfolded proteins, independent of Abl inhibition. Taken together, our data will guide the development of a phase III study to investigate the effects of nilotinib using dopamine metabolites as biomarkers in response to nilotinib in PD. 40 A Novel Small Molecule Tyrosine Kinase Inhibitor (GUtinib) Preferentially Targets Discoidin Domain Receptors and Reduces Toxic Proteins in Neurodegeneration Alan Fowler (Washington, United States), Kaluvu Balaraman (Washington, United States), Michaeline Hebron (Wsahington, DC, United States), Wangke Shi (Washington, United States), Xiaoguang Liu (Washington, DC, United States), Yasar Torres-Yaghi (Washington, DC, United States), Fernando Pagan (McLean, VA, United States), Jaeil Ahn (Washington, United States), Christian Wolfe (Washington, United States), Charbel Moussa (Washington, DC, United States) Objective: Our laboratory demonstrated that multi-kinase inhibitors like nilotinib and bosutinib are optimal agents since they target both Abl and Discoidin Domain Receptors (DDR1/2) and Abl and SRC tyrosine kinases, respectively. These two agents are currently in phase II in DLB (nilotinib and bosutinib), PD and AD (nilotinib). Background: Tyrosine kinase inhibition (TKi) is a potential new strategy to target misfolded protein degradation in neurodegenerative diseases, including Alzheimer’s disease (AD), Parkinson’s disease (PD) and Lewy Body Dementia (DLB). Methods: We evaluated several animal models of neurodegeneration for human alpha-Synuclein, amyloid-beta and hyper-phosphorylated tau. Results: Proﬁling the tyrosine kinase targets of several FDA-approved drugs shows that agents with high potency to Abl alone are ineffective and cannot clear toxic proteins, but a multikinase inhibition like Abl/SRC and DDRs is optimal for misfolded protein clearance. Here we report a novel tyrosine kinase inhibitor (GUtinib) that has the highest speciﬁcity to DDR1/2 and exhibits a high efﬁcacy to clear human alphaSynuclein, amyloid-beta and hyper-phosphorylated tau in several animal models of neurodegeneration. Conclusions: Our data indicate that GUtinib preferentially targets DDR1 and 2, but not Abl, and induces autophagy at very low concentrations and higher efﬁcacy than other multikinase inhibitors. Further toxicology experiments will be conducted to obtain pre-investigational new drug (pre-IND) status, and if the safety of GUtinib is acceptable this agent will be tested in ﬁrst-in-human early development clinical trials. 41 The Lessebo Effect in Parkinson’s Disease: Insights from Individual Patient Data Meta-Analyses Tiago Mestre (Ottawa, ON, Canada), Raquel Lobo (Lisboa, Portugal), Nilza Gonçalves (Lisbon, Portugal), Anthony Lang (Toronto, ON, Canada), Joaquim Ferreira (Torres Vedras, Portugal) Objective: To evaluate the outcome speciﬁcity and temporal evolution of the lessebo effect in Parkinson’s disease (PD). Background: Prior studies show that the inclusion of a placebo arm in a clinical trial may reduce the observed efﬁcacy of the study intervention. This phenomenon is called lessebo effect. The lessebo effect was recently documented in PD, but little is known. Methods: One-stage individual patient data meta-analyses of randomized double-blinded controlled trials conducted in PD to 1) assess the symptomatic effect on motor symptoms, and 2) determine a diseasemodifying effect in early PD. We accessed clinical trials available in the Parkinson Study Group (CALM-PD, TEMPO) and the NINDS PDDOC project (ELLDOPA, FS1, FSTOO, QE2, DATATOP, PRECEPT). After data harmonization and creation of a common dataset, we evaluated the association between different probabilities of placebo allocation and a change in efﬁcacy outcome measures from baseline in active treatment arms exclusively (motor UPDRS, UPDRS part II, time to start of dopaminergic treatment) controlling for pertinent confounders. We MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS used mixed modelling with repeated measures and a Cox proportional hazards model, as appropriate. A P value<0.05 was deemed signiﬁcant. Results: In PD trials conducted to assess a symptomatic effect on motor symptoms (n=815; participants of placebo-controlled trials, n=524; participants of active-comparator trials, n=291), the betweengroup difference in the adjusted mean change from baseline of the motor UPDRS was an improvement of 6.0 units (95% CI: 5.1 - 7.0), and 1.8 units (95% CI: 1.4 - 2.3) in the UPDRS part II at weeks 24-26 of a treatment period, in favour of the active-controlled trials. There were similar between-group differences at weeks 3-4 and weeks 9-14. In PD trials conducted for a disease-modifying effect (n=1149; participants of trials with P (placebo) = 0.25, n=877; participants of trials with P(placebo) = 0.33, n= 272) the adjusted hazards ratio (P(placebo) = 0.25 vs. 0.33) of the time to onset of dopaminergic therapy from baseline was 1.15 (95% CI: 0.92 - 1.43). Conclusions: The current results suggest that in PD the lessebo effect is measurable, clinically signiﬁcant and persistent up to 6 months in the context of symptomatic treatment for motor symptoms. Its presence in disease-modifying trials requires further evaluation, due to the absence of trials without a placebo arm. The prospective assessment of the lessebo effect is warranted in PD to improve knowledge translation of clinical trials into clinical practice. 42 Multimodal balance training with rhythmical cues in Parkinson’s disease: A randomized clinical trial Tamine Capato (Sao Paulo, Brazil), Nienke de Vries (Nijmegen, Netherlands), Joanna IntHout (Nijmegem, Netherlands), Egberto Barbosa (Sao Paulo, Brazil), Jorik Nonnekes (Nijmegen, Netherlands), Bastiaan Bloem (Nijmegen, Netherlands) Objective: (1) To study the effectiveness of balance training with and without rhythmical auditory cues; (2) to verify the effectiveness of the training in long term. Background: Balance impairments in Parkinson’s disease (PD) improves only partially with dopaminergic medication. Therefore, non-pharmacological interventions such as physiotherapy are important elements in clinical management. External cues are often applied to improve gait, however, their effects on balance are unclear. Methods: We performed a prospective, single-blind, randomized clinical trial. We screened 201 volunteers by telephone; 154 were included and assigned randomly into three groups: (1) balance training with rhythmical auditory cues (multimodal balance training); (2) standard balance training without rhythmical auditory cues; and (3) control intervention involving a general education program. Training was performed for 5 weeks, two times/week. Primary outcome was the Mini-BESTest (MBEST) score immediately after the training period. Assessments were performed by a single, blinded assessor at baseline, immediately postintervention, and after one and 6-months follow-up. Results: Immediately post-intervention, multimodal training was more effective than standard training (difference 3.5 (95% Conﬁdence Interval (CI) 2.2; 4.8)), p<0.001). Patients allocated to both active interventions improved compared to controls (MBEST estimated mean difference versus controls 6.6 (CI 5.2; 8.0), p<0.001 for multimodal training; and 3.0 (CI 2.7; 5.3), p<0.001 for standard training). Improvements were retained at one-month follow-up for both active interventions, but only the multimodal training group maintained its improvement at 6-months. Conclusions: Both multimodal training and standard training improve balance, but multimodal training – adding rhythmical auditory cues to standard balance training– has greater and more sustained effects. 43 A Randomized Clinical Trial of Multimodal Balance Training with Rhythmical Cues: Effects on Freezing of Gait in Parkinson’s Disease Tamine Capato (Sao Paulo, Brazil), Nienke de Vries (Nijmegen, Netherlands), Joanna IntHout (Nijmegem, Netherlands), Jordarche Ramjith (Nijmegen, Brazil), Egberto Barbosa (Sao Paulo, Brazil), Jorik Nonnekes (Nijmegen, Netherlands), Bastiaan Bloem (Nijmegen, Netherlands) Objective: To investigate whether the multimodal training is more effective in improving balance and gait in freezers versus non-freezers. Background: Non-pharmacological interventions are increasingly used in the clinical management of gait problems in Parkinson’s disease (PD). However, the effects of cueing training on freezing of gait are suboptimal. We postulate that multimodal balance training might have an additional effect on freezing. Methods: 154 PD patients (Hoehn and Yahr stage 1-3 while ON medication) were assigned randomly to three groups: (1) balance training with rhythmical auditory cues delivered by a metronome (multimodal balance training); (2) standard balance training without rhythmical auditory cues; and (3) control intervention (educational program). Training was performed for 5 weeks, 2x/week. The primary outcome was the Mini-BESTest (MBEST) score directly after the training period. Assessments were performed by a single, blinded assessor at baseline, directly post-intervention, and after one and 6-months follow-up. Treatment effects for freezers and non-freezers were analyzed with a linear mixed model, adjusted for MBEST score, UPDRS scores and levodopa equivalent dose at baseline. Results: Multimodal training signiﬁcantly improved MBEST score post-intervention in both freezers and non-freezers compared to controls (P<0.001). Both patient groups retained their improvement at 1 and 6-months follow-up. Multimodal training signiﬁcantly improved freezing of gait. Functional mobility (timed up and go) improved following both active interventions in the freezers, without difference between both interventions. All improvements were retained until 6-months follow-up. Conclusions: Balance training combined with cueing is particularly effective in improving balance and gait in freezers. This abstract was presented at the 2019 International Congress of Parkinson’s Disease and Movement Disorders in September 2019. 44 Efﬁcacy of Melatonin for Sleep Disorders in Parkinson’s Disease Doniyorbek Daminov (Tashkent, Uzbekistan), Shakhnoza Mukhiddin Qizi (Tashkent, Uzbekistan) Objective: The aim of this study to evaluate efﬁcacy of melatonin in sleep disorders in patients with Parkinson’s disease. Background: Sleep problems may be an early sign of Parkinson’s disease, even before motor symptoms have begun. Emerging evidences suggest that secretion of endogenous melatonin signiﬁcantly decrease in patients with PD. Inadequate secretion of melatonin may main cause of sleep disturbances in PD. Methods: The study included 43 patients with mean age (60 7.7) with a diagnosis of PD without dementia. The average duration of BP is 7.5 years. Patients made up two observation groups. In 23 patients of group I (study group) it was recommended to take Levodopa/carbodopa with melatonin (Melaxen 3 mg). In group II (comparison group), there were 20 patients who took only Levodopa/carbidopa. Sleep disturbance was measured using the Parkinson Disease Severity Scale (PDSS) rating scale, Euphoria sleepiness - Excessive Sleep Scale (ESS), the cognitive function rating scale for PD- SCales for Outcomes in PArkinson’s disease-COGnition (SCOPA-Cog) a quality of life questionnaire under the PD - Parkinson’s Disease Quality of Life (PDQ-39). Results: Sleep disorders was observed in 64.3% of patients, in a third of them almost daily. The study showed the effectiveness of synthetic melatonin (Melaxen, 3 mg tablets) in patients with PD. Against the background of daily intake of Melaxen at a dose of 3 mg / day at bedtime for 6 weeks, there was an improvement in sleep on the PDSS scale (p <0.005) by 27% in I group, a decrease in daytime sleepiness on the ESS scale by 8.7%, in 78% of patients the increased duration of sleep for 1.5 0.5 hours; in most patients (72%), the number of nocturnal awakenings decreased by 50%, the time of falling asleep by 56%, the frequency of insomnia episodes decreased from 3 to 1 times a week (61%). Conclusions: Our clinical research demonstrate that a long time administration of synthetic forms melatonin may improve sleep architecture in Parkinson’s disease. On the other hand Melatonin cannot reduce daytime sleepiness signiﬁcantly. References: Videnovic A, Noble C, Reid KJ, Peng J, Turek FW, Marconi A, et al. Circadian melatonin rhythm and excessive daytime sleepiness in Parkinson disease. JAMA Neurol. 2014;71(4):463–469. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S23 ABSTRACTS Clinicopathological Correlations 45 Pseudoathetosis as an Early Manifestation in a Patient with Multiple Sclerosis (MS) Isabelle Pastor Bandeira (Joinville, Brazil), Washigton Luiz De Medeiros Junior (Joinville, Brazil), André Eduardo Franzoi (Joinville, Brazil), Marina Giacomet (Joinville, Brazil), Laura Parolin (Joinville, Brazil), Paulo Roberto Wille (Joinville, Brazil), Marcus Vinicius Gonçalves (Joinville, Brazil) Objective: The aim of this paper is to report a case of pseudoatetosis and multiple sclerosis, in which movement disorders may be considered a case of open-labeling for demyelinating diseases. Background: Multiple sclerosis (MS) is usually diagnosed in young adults aged 30 to 40 years. The onset of the disease is characterized by episodes of neurological dysfunction that usually recover [1]. Movement disorders are possible clinical manifestations of MS patients. The study of these disorders is of important clinical impact, since early diagnosis signiﬁcantly improves the treatment of patients with the disease [2,3].Pseudoathetosis is a movement disorder that can be developed from lesions at all levels of proprioceptive sensory pathways. Hammond introduced this term in 1881, which comes from the Greek word athetosis meaning “without ﬁxed position” [4]. In 1975 the term pseudoathetosis was introduced by Dooling and Adams to refer to movement disorder characterized by involuntary, slow and writhing movements. The disorder differs clinically from athetosis. The pseudoathetosis is manifested by loss of proprioception, whereas in athetosis there is no sensory loss [5].Various conditions that involve the posterior column have been reported to cause pseudoathetosis. These conditions include myelitis, spinal cord infarct, trauma, tumors, spondylotic myelopathy, leprosy, neuromyelitis optica spectrum disorder (NMOSD), and vitamin B12 deﬁciency [6,7].The main lesion areas are the posterior column of the spinal cord, parietal cortex, thalamus, and peripheral nerves [8,9].Reports of MS with pseudoathetosis are extremely rare. We report a case that was diagnosed as MS but was associated with pseudoathetosis. Methods: Clinical Case description. Results: A 29-year-old woman complained of sensory loss in her left arm. Neurological examination revealed hemiataxis and pseudoathetosis in the upper left limb. At the moment, the course was pulsotherapy with solumedrol for the next 5 days. Imaging exams showed demyelinating lesions in the spinal cord [IMAGE]. Patient was discharged with the diagnosis of active RRMS. Conclusions: Multiple Sclerosis and other conditions that involve the posterior column have been reported to cause spinal pseudoathetosis [10]. Pseudoathetosis is also a very rare symptom in MS. PotulskaChromik and colleagues reported a case of a 43-year-old woman with MS who presented transient involuntary movements of the right hand S24 with coexisting loss of proprioception. Based on their research, they showed that this symptom is usually due to lesion of the proprioception pathway resulting in the dys-function of the integration of the deep sensation with motor function with location in the striatum [10]. Spitz et al described a 34-year-old MS patient who also presented pseudoathetosis as a movement disorder. She had sudden onset of involuntary movements in both hands and on her physical examination there was reduced deep sensation in both hands. In their study, they emphasized the importance of having an accurate evaluation of deep sensitivity whenever dealing with a patient with abnormal athetotic movements of extremities, since proprioception loss from different causes may lead to pseudoathetosis [9]. In conclusion, pseudoathetosis may be considered a case of open-labeling for MS. This is rare, but we recommend that in the presence of pseudoathetosis [10], should be considered the diagnostic hypothesis of MS, especially in young patients. References: Brownlee WJ, Hardy TA, Fazekas F, et al. Diagnosis of multiple sclerosis: progress and challenges. Lancet. 2017 Apr 1;389 (10076):1336-1346.Cameron MH, Nilsagard Y. Balance, gait, and falls in multiple sclerosis. Handb Clin Neurol. 2018;159:237-250.Woods JM. Reﬂections on 50 Years of Neuroscience Nursing: Movement Disorders, Neuromuscular Disease, and Multiple Sclerosis. J Neurosci Nurs. 2018 Feb;50(1):5-12.Sharp FR, Rando TA, Greenberg SA, Brown L, Sagar SM. Pseudochoreoathetosis: movement associated with loss of proprioception. Arch Neurol 1994;51:1103–1109Dooling EC, Adams RD. The pathological anatomy of post hemiplegic athetosis. Brain 1975;98:29–45. Seok HY, Jang SH, You S. Neuromyelitis Optica Spectrum Disorder Presenting with Pseudoathetosis. J Clin Neurol. 2018 Jan;14(1):123-125. Abboud H, Yu XX, Knusel K, et al. Movement disorders in early MS and related diseases: A prospective observational study. Neurol Clin Pract. 2019 Feb;9(1):24-31.Lo YL, See S. Images in clinical medicine. Pseudoathetosis. N Engl J Med. 2010 Nov 4;363(19):e29. Spitz M, Aluízio Costa Machado A, Carvalho R, Maia F, Haddad M, Calegaro D et al. Pseudoathetosis: Report of three patients. Movement Disorders. 2006;21(9):1520-1522.Potulska-Chromik A, Rudzinska M, Nojszewska M, Podlecka-Pietowska A, Szczudlik A, ZakrzewskaPniewska B et al. Original article Clinical and neuroimaging correlation of movement disorders in multiple sclerosis: case series and review of the literature. Folia Neuropathologica. 2014;1:92-100. 46 Myasthenia Gravis and Parkinson’s Disease: Correlation or Causation? Kalea Colletta (Orland Hills, IL, United States), David Kvarnberg (Hines, IL, United States), Jasvinder Chawla (Hines, IL, United States) Objective: We describe 2 patients with co-incident Myasthenia Gravis (MG) and Parkinson’s disease (PD). Both patients’ initial manifestation was MG followed by PD. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Background: MG and PD are unrelated disease entities rarely coexisting in one patient. MG is an autoimmune neuromuscular junction (NMJ) disorder and PD is due to dopamine loss in the substantia nigra. There is an increase in reported cases; is there a link? Methods: Recently, 2 patients were found to have co-existing MG and PD at Hines VA. Patient 1 presented to neurology for dysphagia, hypophonia with a nasal quality, and “tired tongue” with an otherwise unremarkable exam. Anti-acetylcholine receptor antibody (a-AChRAb) and repetitive stimulation on EMG was positive. Within 2 years the patient developed parkinsonian features and had a positive DAT scan. Patient 2 was diagnosed at an outside hospital with MG and started on prednisone and pyridostigmine with good control. However, 4 years later he began exhibiting symptoms of gait imbalance, resting tremor, and hypophonia, consistent with PD. He was started on Sinemet with signiﬁcant improvement of the aforementioned symptoms. Results: There are 20 reported cases of co-incident MG and PD (including our 2 patients). a-AChRAbs were positive in 60% of cases, which questions the impact of a-AChRAbs on the central nervous system (CNS). Despite a-AChRAbs being thought to only affect peripheral muscular a1 subunits, several studies described cross-reactivity with CNS antigens, namely CNS acetylcholine receptor (AChR) a9 and a7 subunits. This would account for CNS manifestations of MG including sleep and cognitive issues, autonomic dysfunction, pain and anosmia, which are identical to the nonmotor symptoms of PD. Furthermore, the a7 subunit has been implicated as a subtype involved in both CNS AChR binding deﬁcits seen in PD and in the protective effects of nicotine on PD. Conclusions: With the increased number of reported co-incident cases of MG and PD, the identical non-motor symptoms present in both disorders, and the majority of cases presenting with positive a-AChRAbs, it seems more than just coincidence. Given cross-reactivity between peripheral a1 muscular subunits and CNS a9 and a7 AChR subunits, there is evidence that CNS AChR dysfunction does in fact occur with MG. Furthermore, the negative impact of MG on CNS AChRs, especially the a7 subunit, may propagate the development of PD as functioning nicotinic AChRs and nicotine are neuroprotective against PD. Given the growing body of evidence of co-incident MG and PD, further investigation is necessary. References: Marano, Massimo, et al. “A rare cause of axial worsening in Parkinson’s disease: A case of myasthenic pseudo-parkinsonism.” Clinical neurology and neurosurgery 179 (2019): 1-3.Quik, Maryka. "Smoking, nicotine and Parkinson’s disease." Trends in neurosciences 27.9 (2004): 561-568. Cognition and Cognitive Disorders 50 Sleep EEG Delta Power is Associated with Cognitive Function in Parkinson’s Disease Adeel Memon (Birmingham, AL, United States), Kimberly Wood (Alabaster, AL, United States), Raima Memon (Birmingham, AL, United States), Allen Joop (Birmingham, AL, United States), Jennifer Pilkington (Birmingham, AL, United States), Adam Gerstenecker (Birmingham, AL, United States), Kristin Triebel (Birmingham, AL, United States), Marcas Bamman (Birmingham, AL, United States), Svjetlana Miocinovic (Atlanta, GA, United States), Amy Amara (Birmingham, AL, United States) Objective: This novel study investigated the relationship between slow wave sleep (SWS) EEG delta power and cognition, measured with a comprehensive neurocognitive assessment in patients with Parkinson’s disease (PD). Background: Cognitive dysfunction affects up to 80% of PD patients (1) and is one of the most disabling non-motor symptoms. Prior work suggests a relationship between sleep and cognition. SWS (<4Hz) may contribute to brain plasticity and cognition (2). Methods: This cross-sectional study evaluated 31 PD participants with polysomnography (PSG) and a Level II comprehensive neurocognitive battery as deﬁned by the MDS Task Force on PD-mild cognitive impairment (MCI). PSGs included EEG, from which delta frequency (0.5-4Hz) spectral power was analyzed in frontal leads (F3 or F4) in artifact-free epochs of NREM stage 3 (N3). Scores for individual cognitive tests were used to calculate z-scores based on normative values. Zscores for each test in a domain were averaged to obtain domain scores, and domain scores were averaged to determine the Composite Cognitive Score (primary outcome). Correlations between N3 delta power and cognitive performance were analyzed with Pearson correlation coefﬁcients. Results: N3 delta power was correlated with better cognitive performance as measured by the Composite Cognitive Score (r=0.506, p=0.004). At the domain level, higher N3 delta power was correlated with better performance in executive function (p=0.003), memory (p=0.034), and language (p=0.035) domains. No signiﬁcant correlations were observed between N3 and attention/working memory (p=0.20), visuospatial skills (p=0.56), or processing speed (p=0.062) domains. When the sample was stratiﬁed by PD-MCI diagnosis, N3 delta power was signiﬁcantly higher in normal cognition participants (n=11) compared to those with PD-MCI (n=22) (Z=2.11, p=0.035). Conclusions: N3 delta power is correlated with global cognitive function in PD. Exploratory analyses suggest that this relationship is driven by associations between higher delta power and better performance in domains of executive function, memory, and language. Thus, N3 delta power may serve as a non-invasive marker of cognitive function in PD patients. Further research to determine potential therapeutic implications may explore the impact of pharmacological or nonpharmacological enhancement of SWS on cognitive outcomes. References: 1. Hely, MA, Reid WG, Adena MA, Halliday GM, Morris JG. The Sydney multicenter study of Parkinson’s disease: the inevitability of dementia at 20 years. Mov Disord 2008; 23(6): 837-44.2. Fogel, S., Martin, N., Lafortune, M., Barakat, M., Debas, K., Laventure, S., Latreille, V.,Gagnon, J.F., Doyon, J., Carrier, J., 2012. NREM sleep oscillations and brain plasticity in aging. Front. Neurol. 3, 1e7. 51 Mild Cognitive Impairment and Deﬁcits in Activities of Daily Living in Individuals with Parkinson’s Disease Ana Paula Loureiro (Curitiba, Brazil), Bruna Yamaguchi (Curitiba, Brazil), Adriano Silva (Curitiba, PR, Brazil), Vera Israel (Curitiba, Brazil) Objective: This study aimed to determine whether cognitive impairment is related to the decline in the ability to perform activities of daily living (ADL). Background: Cognitive impairment is a common non-motor symptom of Parkinson’s disease (PD), which has been associated with functional decline. Methods: Individuals with idiopathic PD were assessed with the Montreal Cognitive Assessment (MoCA) used to detect mild cognitive impairment. For motor aspects of experiences of daily living some components of the MDS-Uniﬁed Parkinson’s Disease Rating Scale (MDSUPDRS) – Subscale II, were checked. Results: 49 volunteers, 28 men (59,2%) and 21 women, Hoehn & Yahr HY1= 16; HY2= 14; HY3= 13; HY4= 6. Mean age 63,18 12,56 years old took part in the study. MoCA screening mean 21,02 4,63, MDS -UPDRS II (ADL) mean 13,08 5,73. Correlations were found between MoCA and the MDS-UPDRS eating tasks (rho = 0.39; p < 0.005); dressing (rho = 0.40; p < 0.004) and hygiene (rho = 0.29; p < 0.041). Conclusions: Our ﬁndings indicate that those individuals with worse performance on the MoCA are associated with greater dependence in some aspects of ADL. This impairment in the primary and/or the secondary tasks occurs because the two tasks compete for similar demands for their processing. These results reinforce the importance of cognitive stimulation associated with motor training during goal-setting in physical therapy treatment because physical exercise with cognitive demand can strengthen and improve motor circuits. References: Rosenthal E, Brennan L, Xie S, Hurtig H, Milber J, Weintraub D, Karlawish J, Siderowf A. Association between cognition and function in patients with Parkinson disease with and without dementia. Mov Disord. 2010 Jul 15;25(9):1170-6.Hoops S, Nazem S, Siderowf AD, Duda JE, Xie SX, Stern MB, Weintraub D. Validity of the MoCA and MMSE in the detection of MCI and dementia in Parkinson disease. Neurology. 2009 Nov 24;73(21):1738-45 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S25 ABSTRACTS 52 Comparative Analysis of Cognitive Proﬁle of Parkinson’s Disease patients with Subthalamic Nucleus Deep Brain Stimulation and Healthy Subjects: Preliminar Results Eduarda Barbosa (Rio de Janeiro, Brazil), Jose Nasser (Rio De Janeiro, Brazil), Helenice Charchat Fichman (Rio de Janeiro, Brazil) Objective: Compare cognitive performance of patients with PD receiving subthalamic nucleus Deep Brain Stimulation (STN-DBS) and healthy volunteers. Background: Subthalamic Nucleus Deep Brain Stimulation (STNDBS) has become an alternative in treatment of movement disorders [1], including Parkinson Disease. Due to not only presenting motor symptoms, it is necessary to assess the non-motor symptoms, in this case, cognition. Methods: Descriptive analysis and t-test comparing results from patients with PD receiving STN-DBS and healthy group. Results: Descriptive analysis and t-test comparing both groups: STNPD with 32 subjects with a mean age of 63.63 years (10,01) and average schooling of 8.17 years ( 4,11) and controlled group (CG) with 33 subjects with a mean age of 71,33 ( 6,78) and average schooling of 15,36 years ( 4,08). The preliminary results are shown in images 1 to 3. Regarding global cognitive functioning, the control group, as expected, presented better performance, as well as verbal ﬂuency; however, in speciﬁc aspects such as naming, learning, late memory and recognition, there was no statistically signiﬁcant difference. Regarding the visual constructive ability and executive functions, the control group presented worse but not statistically signiﬁcant performance. The control group presented better performance in both simple and choice median reaction time, but in the total time there was no statistically signiﬁcant difference. The same was true for implicit learning, with better control group results, but no statistically signiﬁcant difference. Conclusions: Despite the better results from the control group subjects, there were no statistically signiﬁcant differences between them and Parkinson’s disease patients who underwent implantation of deep brain stimulation. This is a very interesting result that deserves further exploration. This paper presents preliminary results of a research that intends to better investigate these aspects. References: Nasser, JA; Falavigna, A; Alaminos, A; Bonatelli, AP; Ferraz, F. (2002) Estimulaç~ao Cerebral Contínua (DBS) Subtalâmica para Controle do Tremor. Arquivos de Neuropsiquiatria, 60(2-B). S26 Drug-Induced Movement Disorders 47 Treatment Responses with Long-Term Valbenazine in Patients with Tardive Dyskinesia Carlos Singer (Fort Lauderdale, FL, United States), Stephen Marder (Los Angeles, CA, United States), Cynthia Comella (Chicago, IL, United States), Khodayar Farahmand (San Diego, CA, United States), Roland Jimenez (San Diego, CA, United States) Objective: To evaluate the range of responses in adults with tardive dyskinesia (TD) who received once-daily treatment with valbenazine (VBZ) for 48 weeks. Background: VBZ, a highly selective vesicular monoamine transporter 2 (VMAT2) inhibitor, is approved for the treatment of TD in adults. VBZ clinical trials used rigorous deﬁnitions of response, including =50% improvement from baseline in the Abnormal Involuntary Movement Scale (AIMS) total score (sum of items 1-7) and score =2 (“much improved” or better) on the Clinical Global Impression of Change-Tardive Dyskinesia (CGI-TD) or Patient Global Impression of Change (PGIC). However, patients who did not meet these thresholds still experienced meaningful levels of response. Therefore, data from KINECT 4 (NCT02405091) were analyzed post hoc to determine the range of responses in patients who received 48 weeks of open-label treatment. Methods: Key inclusion criteria were as follows: ages 18 to 85 years; DSM-IV diagnosis of schizophrenia, schizoaffective disorder, or mood disorder; neuroleptic-induced TD for =3 months prior to screening; stable psychiatric status (Brief Psychiatric Rating Scale score <50); no high risk of active suicidal ideation or behavior. VBZ dosing was initiated at 40mg, with escalation to 80mg at Wk4 based on clinical assessment of TD and tolerability; dose reduction to 40mg was allowed if 80mg was not tolerated. Response ranges at Wk48 were deﬁned as follows: AIMS, =10% to 100% total improvement from baseline (per site investigator); CGI-TD, score =3 (“minimally improved” or better) or =2 (“much improved” or better); and PGIC, score =3 or =2. Results: 103 participants had available assessments at Wk48 (40mg=20; 80mg=83 [9 with dose reduction]). At Wk48, 94.2% of all participants had =30% improvement in AIMS total score and 86.4% had =50% improvement. Response rates for the remaining AIMS thresholds ranged from 9.7% (100% response) to 97.1% (=10% response). Almost all participants had a global score =3 at Wk48: CGI-TD, 99.0%; PGIC, 98.1%. Most had a global score =2: CGITD, 92.2%; PGIC, 88.3%. Conclusions: After 48 weeks of treatment with once-daily VBZ, >85% of KINECT 4 participants had a clinically meaningful AIMS response (=30% improvement) or a robust AIMS response (=50% improvement). Most participants had a global rating of “minimally improved” or better, and >88% had a global rating of “much improved” or better. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS 48 Withdrawn by Author Dyskinesia 53 Mindfulness Intervention for Paroxysmal Dyskinesia and Electroderma Response Amir Ramezani (Sacramento, CA, United States), Philippe Levy (Sacramento, United States), Richard Wanlass (Sacramento, United States), Robert McCarron (Irvine, United States), Samir Sheth (Sacramento, United States) Objective: Describe clinical activity of skin conductance in response to mindfulness intervention in a case of Paroxysmal Dyskinesia. Background: Paroxysmal dyskinesia is group of uncommon movement disorder that preset with dystonia, chorea, athetosis, or a combination of these features (Waln and Jankovic, 2015). Non-pharmacological behavioral interventions are recommended to managing chronic conditions, such as Paroxysmal Dyskinesia, however, little research or descriptive cases are available. Pickut et al (2015) conducted randomized control trial of mindfulness interventions for patients living with Parkinson’s disease. Results showed improvements on pain scores, ability to observe internal mental processes, and movement abilities. Targeting nonpharmacological intervention to detect and manage precipitating factors remains an important strategy in disease management (Waln and Jankovic, 2015). Methods: Literature review, psychophysiological recording, and case study. Results: Paroxysmal Dyskinesia episodes appeared to correspond to electrodermal activity as measured by skin conductance. Mindfulness interventions appeared to reduce electrodermal response during the practice. Conclusions: Skin conductance was observed to be a useful way to measure Paroxysmal Dyskinesia episodes and response to mindfulness intervention. Mindfulness intervention was observed to reduce the intensity of the episode and the electrodermal activity. References: Pickut B, Vanneste S, Hirsch MA, Van Hecke W, Kerckhofs E, Mariën P, Parizel PM, Crosiers D, Cras P. (2015). Mindfulness Training among Individuals with Parkinson’s Disease: Neurobehavioral Effects. Parkinsons Dis., 816404Waln, O., & Jankovic, J. (2015). Paroxysmal movement disorders. Neurol Clin, 33(1), 137-152. doi:10.1016/j.ncl.2014.09.014 54 Reduced Dyskinesia and OFF Time in PD Patients with DBS Following Switch From Amantadine IR to ADS-5102 (Amantadine) Extended Release Capsules: Analysis of 2-Year Open-Label Trial (EASE LID 2) Caroline Tanner (San Francisco, CA, United States), Pinky Agarwal (Kirkland, WA, United States), Dustin Chernick (Emeryville, CA, United States), Andrea Formella (Emeryville, CA, United States), Jean Hubble (Emeryville, CA, United States) MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S27 ABSTRACTS Objective: To evaluate motor ﬂuctuations and safety outcomes among PD patients with DBS who switched directly from immediaterelease (IR) amantadine to ADS-5102 (amantadine) extended release capsules (Gocovri®, Adamas Pharmaceuticals, Inc.) upon enrollment in the 2-year open-label EASE LID 2 trial. Background: ADS-5102, the only FDA-approved drug for LID in PD, also reduced OFF time in 2 pivotal trials.[1,2] The EASE LID 2 trial (NCT02202551) showed long-term antidyskinetic effect and included 24 patients with DBS who were using amantadine IR at enrollment.[3,4] Methods: Of 223 patients in EASE LID 2, 61 had DBS and 24 of these 61 were taking amantadine IR (DBS + Amantadine IR) [Figure1]. Upon enrollment in this trial, all 24 were switched directly to ADS-5102 (dosed at 137 mg QHS in week 1 and 274 mg QHS thereafter). Adverse events (AEs) and persistence on treatment were evaluated in this trial, and efﬁcacy was measured by the change in dyskinesia and OFF time from baseline through end of study (week 100), as assessed using the MDS-UPDRS Part IV Total and item scores. All analyses were conducted post-hoc. Results: Of 24 DBS + Amantadine IR patients enrolled, 19 (79.2%) completed 52 weeks and 15 (63%) completed 100 weeks of treatment. There were 9 patients who withdrew, 4 (16.7%) due to AEs. The most common AEs (>10%) were fall (29%), peripheral edema (29%), hallucination (25%), local swelling (13%), urinary tract infection (13%), and osteoarthritis (13%). At baseline, the mean MDS-UPDRS Part IV total score in this subgroup was 9.8. Following ADS-5102 treatment the MDS-UPDRS Part IV total score decreased by a mean 2.7 points, from baseline to week 8 (the ﬁrst study visit), and the effect was maintained through week 100 (mean change, -2.8) [Table1]. Both daily duration and functional impact of dyskinesia and OFF, measured by Part IV items 4.1 to 4.4, improved by week 8, and remained improved through week 100. Conclusions: This exploratory analysis suggests that ADS-5102 was well tolerated and provided durable improvement in dyskinesia and OFF among PD patients with DBS who switched directly from amantadine IR. The results are consistent with previously reported improvements in dyskinesia and OFF among all patient groups in this 2-year open-label study and suggest that ADS-5102 treatment may provide beneﬁt for PD patients who were incompletely responsive to DBS, amantadine IR, or both. References: 1.Pahwa R, Tanner CM, Hauser RA, Isaacson SH, Nausieda PA, Truong DD, Agarwal P, Hull KL, Lyons KE, Johnson R, Stempien MJ. ADS-5102 (Amantadine) extended-release capsules for levodopa-induced dyskinesia in Parkinson disease (EASE LID study): A randomized clinical trial. JAMA Neurol. 2017;74:941-949.2.Oertel W, Eggert K, Pahwa R, Tanner CM, Hauser RA, Trenkwalder C, Ehret R, Azulay JP, Isaacson S, Felt L, Stempien MJ. Randomized, placebocontrolled trial of ADS-5102 (amantadine) extended-release capsules for levodopa-induced dyskinesia in Parkinson’s disease (EASE LID 3). Mov Disord. 2017;32:1701-1709.3.Hauser RA, Pahwa R, Tanner CM, Oertel W, Isaacson SH, Johnson R, Felt L, Stempien MJ. ADS-5102 (Amantadine) Extended-Release Capsules for Levodopa-Induced Dyskinesia in Parkinson’s Disease (EASE LID 2 Study): Interim Results of an Open-Label Safety Study. J Parkinsons Dis. 2017;7:511-522.4.Isaacson S28 SH, Fahn S, Pahwa R, Tanner CM, Espay AJ, Trenkwalder C, Adler CH, Patni R, Johnson R. Parkinson’s Patients with Dyskinesia Switched from Immediate Release Amantadine to Open-label ADS-5102. Mov Disord Clin Pract. 2018;5:183-190. 55 PD Patient Diaries Demonstrated ADS-5102 (Amantadine) Extended Release Capsules Improved ON Time Without Dyskinesia: Results From Pooled Phase 3 Clinical Trials Robert Hauser (Tampa, FL, United States), Ryan Walsh (Phoenix, AZ, United States), Dustin Chernick (Emeryville, CA, United States), Jean Hubble (Emeryville, CA, United States) Objective: To evaluate the extent to which the reported ADS-5102 (amantadine) extended release capsules (Gocovri®, Adamas Pharmaceuticals, Inc.)-associated improvement in “ON time without troublesome dyskinesia” resulted from an increase in the amount of ON time without dyskinesia. Background: ADS-5102, the only FDA-approved treatment for LID in PD, improved dyskinesia and OFF time in clinical trials[1,2]. The trials demonstrated an improvement in “ON time without troublesome dyskinesia”, a construct composed of two PD diary states: ON time with nontroublesome dyskinesia and ON time without dyskinesia [ﬁgure1]. Methods: Patients enrolled in phase 3 trials (EASE LID [NCT02136914] or EASE LID 3 [NCT02274766]) recorded time spent in the following PD diary states at baseline and endpoint (week 12): asleep, OFF, ON with troublesome dyskinesia, ON with nontroublesome dyskinesia, and ON without dyskinesia. We conducted MMRM analyses of the time spent in both ON with non-troublesome dyskinesia and ON without dyskinesia, following wake-up, for 162 of 198 (82%) enrolled patients (ADS-5102 n=85 and placebo n=77) with completed diaries at both timepoints, to evaluate changes in these states from baseline to week 12. Results: At baseline, patients experienced a mean 8.3 hours of “ON time without troublesome dyskinesia” (4.6 hours with non-troublesome dyskinesia and 3.7 hours without dyskinesia). At week 12, ON time with non-troublesome dyskinesia decreased by 0.7 hours for ADS-5102 vs. 0.3 hours for placebo (LS mean treatment difference: -0.5 hours, P=0.030), while ON time without dyskinesia increased by 4.7 vs. 1.4 hours (LS mean treatment difference: +2.9 hours, P<0.0001). The combination of these two diary states gives the previously reported LS mean placebo-corrected increase of 2.5 hours in the composite “ON time without troublesome dyskinesia”; this increase corresponds with reductions in both troublesome dyskinesia and OFF time (LS mean treatment difference: -1.5 hours, and -1.2 hours, respectively).[3] Conclusions: This post hoc analysis of PD diary data showed that ADS-5102 treatment more than doubled the daily time patients spent ON without dyskinesia. These results suggest that the ADS-5102 treatment effect was driven by an increase in ON time without dyskinesia, as opposed to simply reducing the severity of dyskinesia from troublesome to non-troublesome. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS References: 1.Pahwa R, Tanner CM, Hauser RA, Isaacson SH, Nausieda PA, Truong DD, Agarwal P, Hull KL, Lyons KE, Johnson R, Stempien MJ. ADS-5102 (Amantadine) extended-release capsules for levodopa-induced dyskinesia in Parkinson disease (EASE LID study): A randomized clinical trial. JAMA Neurol. 2017;74:941-949.2. Oertel W, Eggert K, Pahwa R, Tanner CM, Hauser RA, Trenkwalder C, Ehret R, Azulay JP, Isaacson S, Felt L, Stempien MJ. Randomized, placebo-controlled trial of ADS-5102 (amantadine) extended-release capsules for levodopainduced dyskinesia in Parkinson’s disease (EASE LID 3). Mov Disord. 2017;32:1701-1709.3. Hauser RA, Kremens DE, Elmer LW, Kreitzman DL, Walsh RR, Johnson R, Howard R, Nguyen JT, Patni R. Prevalence of Dyskinesia and OFF by 30-Minute Intervals Through the Day and Assessment of Daily Episodes of Dyskinesia and OFF: Novel Analyses of Diary Data from ADS-5102 Pivotal Trials. J Parkinsons Dis.2019;9:591-600. 56 Frequency of Dyskinesia as a Function of Baseline Dyskinesia in Patients with Parkinson’s Disease Treated with Istradefylline, an Adenosine A2A Receptor Antagonist Nobutaka Hattori (Tokyo, Japan), Takanobu Nomura (Tokyo, Japan), Phyllis Salzman (Wyncote, PA, United States), Hiroki Kitabayashi (Tokyo, Japan), Masatake Ishiuchi (Tokyo, Japan), Keizo Toyama (Tokyo, Japan), Akihisa Mori (Tokyo, Japan) Objective: To assess the effect of baseline dyskinesia on the efﬁcacy and safety of istradefylline treatment. Background: Istradefylline is an adenosine receptor antagonist indicated as adjunctive treatment to levodopa/carbidopa in adult patients with Parkinson’s disease (PD) experiencing OFF episodes. Methods: Patients with PD receiving levodopa with or without other approved anti-Parkinson medications and experiencing wearing-off were randomized to istradefylline, 20 or 40 mg/day, or placebo for 12 weeks. In a pooled, post-hoc analysis of two double-blind Phase 2b/3 clinical studies, subgroups were deﬁned by presence or absence of baseline (preexisting) dyskinesia. Presence of baseline dyskinesia and change in daily OFF time were assessed using patient-completed 24-hour ON/OFF diaries. Adverse events (AEs) were recorded throughout. Results: The analysis included 723 patients. Baseline characteristics were similar across treatment groups (placebo, 20 mg/day, 40 mg/day). At baseline, 286 patient diaries recorded ON time with dyskinesia, and 437 recorded no dyskinesia. Among patients with baseline dyskinesia, dyskinesia was reported more frequently as an AE than in patients without baseline dyskinesia (Figure). However, istradefylline’s efﬁcacy in reducing OFF hours/day was unaffected. At the Week 12/last visit, reduction from baseline in OFF hours/day was similar in patients with and without baseline dyskinesia and greater with istradefylline than with placebo. Overall, istradefylline was well-tolerated.Conclusions: These results demonstrated that the presence of dyskinesia at baseline was associated with more frequent reporting of dyskinesia as an AE during istradefylline treatment. However, the presence or absence of baseline dyskinesia had little inﬂuence on the ability of istradefylline to reduce daily OFF time. Istradefylline offers an adenosine A2A receptor-mediated, non-dopaminergic mechanism for patients with PD receiving levodopa and other PD medications who are experiencing motor complications, regardless of the presence of baseline dyskinesia. 57 Paroxysmal Non-Kinesigenic Dyskinesia Disorder Secondary to Systemic Lupus Erythematosus: A Case Report Dante Oropeza (Puebla, Mexico), Adriana Juárez Nájera (Puebla, Mexico), Maricruz Velazquez Vaquero (Puebla, Mexico) Objective: To evaluate a case of an adult Paroxysmal Nonkinesigenic Dyskinesia Disorder secondary to Systemic Lupus Erythematosus. Background: Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease affecting any organ system of the body and occurs predominantly in women of gestation age. Movement disorders are rare in SLE patients, the most common of which is chorea with 1.3% prevalence. Methods: Case report with neurological, neurophysiological, neuropsychological and laboratory examinations. Results: A 35-year-old woman presented to our clinic for evaluation and treatment of involuntary movements in the right hand which had begun about 2 months earlier. She also referred to changes in behavior with a tendency to aggressiveness and violence. On neurological examination, rhythmic irregular movements that increase with stress were observed in the little, ring and index ﬁngers of the right hand. On neurophysiological evaluation, the EEG did not show epileptic grafoelements, the NCVs demonstrate a sensorimotor neuroconduction within normal ranges and the EMG suggested a miorhythmia of subcortical-supraspinal origin. MRI showed a decrease in the volume of the white matter at the frontal level and the absence of the left posterior communicating artery. On neuropsychological examination, a Dysexecutive Syndrome associated with the dorsolateral prefrontal cortex was found. On laboratory tests, the antinuclear and anti-RNP C antibodies were positive. After 8 months since the motor disorders appeared, she was diagnosed with SLE and pregabalin, atomoxetine and desvenlafaxine was administrated. Conclusions: Immune-mediated movement disorders occur in children and adults. We report a new movement disorder in adults secondary to SLE related to a decrease in the volume of the white matter in the frontal cortex and a Dysexecutive Syndrome. This report serves to expand the understanding of the etiology and comorbidities of movement disorders associated with autoimmune diseases. References: Baizabal-Carvallo, J. F., & Jankovic, J. (2012). Movement disorders in autoimmune diseases. Movement Disorders, 27(8), 935-946. Liu, F.-C., Huang, W.-Y., Lin, T.-Y., Shen, C.-H., Chou, Y.C., Lin, C.-L., Kao, C.-H. (2015). Inverse Association of Parkinson Disease With Systemic Lupus Erythematosus: A Nationwide Populationbased Study. Medicine, 94(46), e2097. 58 Epidemiological Characteristics of Levodopa-Induced Dyskinesia in a Mexican-Mestizo Population Rodolfo Abundes-Corona (Mexico City, Mexico), Oscar Esquivel-Zapata (Monterrey, Mexico), Susana Lopez-Alamillo (Villahermosa, Mexico), Amin Cervantes (Mexico City, Mexico), Mayela Rodriguez Violante (Mexico City, Mexico) Objective: To describe the clinical and epidemiological characteristics of a mexican-mestizo population with Parkinson’s disease (PD) and levodopa-induced dyskinesia (LID) at tertiary care center. Background: Motor ﬂuctuations and dyskinesia are the main adverse events of levodopa treatment in patients with PD. (1) LID, can occur at any time of the ON period. Patients with young-onset PD are at higher risk of developing LID. Gender differences have been reported, but an increased risk has not been demonstrated. (2) Methods: Cross-sectional, observational study. We included PD patients with LID from the REMEPARK (Mexican’s Parkinson’s Disease Registry). Electronic ﬁle data was analyzed to identify subtype of LID (peak dose, end dose or diphasic). The MDS-UPDRS parts II and III were used to identify motor subtype. Results: 130 PD patients (median age 66, 24-97, 55.4% female) were recruited from our cohort. The median years of levodopa treatment was 7 (1-37), and the median dosage at the moment of the visit was 750mg daily (100-2,500). The most prevalent subtype of LID was Peak Dose dyskinesia (45%), followed by End Dose (30%). LID subtype was not MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S29 ABSTRACTS identiﬁable in 31% of the patients. The most common motor subtype that presented dyskinesia was PIGD, 77.7%. Conclusions: Dyskinesia phenotype and their general epidemiological characteristics in PD patients of Mexican-mestizo ancestry are consistent with what has been previously described in the global population. Of the 130 patients with dyskinesia, 77% corresponded with PIGD subtype, in relation to a more severe phenotype, a longer duration of the disease, and a longer time of Levodopa treatment. References: Fabbrini G, Brotchie JM, Grandas F, Nomoto M, Goetz CG. Levodopa-induced dyskinesias. Mov Disord. 2007;22(10):1379–89. 2. Kelly MJ, Lawton MA, Baig F, Ruffmann C, Barber TR, Lo C, et al. Predictors of motor complications in early Parkinson’s disease: A prospective cohort study. Mov Disord. 2019;34(8):1174–83. characterized by involuntary cramping of muscles of the hand, forearm, or upper arm selectively triggered by writing.Chorea-athetosis is one of the major types of involuntary movement disorders originating from dysfunctional neuronal networks interconnecting the basal ganglia and frontal cortical motor areas. The syndrome is characterised by a continuous ﬂow of random, brief, involuntary muscle contractions and can result from a wide variety of causes. Unilateral and pseudo-athetosis are derived not from disturbance of the basal ganglia but from impaired sensory pathways. Methods: A 44-year-old right-handed female, was referred to our institution on 2018 because of a 3-year history of involuntary movements of the left upper limb, associated with writing difﬁculty on the opposite hand of the same evolution time.She had been receiving venlafaxine 75 mg for 6 months by then. Previously treated with escitalopram for mood disorders and pregabalin in increasing doses up to 150 mg per day due to cervical pain. The physical examination showed choreoathetoid movements in the left upper limb, associated with homolateral dystonic posture and dystonic task speciﬁc posture on the right upper limb at writing. No mirror movements.Cervical MRI showed C5-C6, C6-C7 protrusions that partially occupied the anterior epidural space and insinuates on both neuroforams bases with left predominance. EMG of the upper limbs with tremor registry showed distal synchronous movements of antagonists muscles on the right upper extremity with a frequency of 6-8 Hz and C7 root involvement without signs of current denervation. Somatosensory evoked potentials within normal parameters. Results: We arrived to the diagnosis of left choreoathetosis and right writer’s cramp due to root compression by cervical herniation. Conclusions: The present report illustrates a case of choreoatetosis on one limb and writer’s cramp on the contralateral one, due to root compression by a cervical hernia. There are some case series and reports that studied choreoathetoid movements or dystonic postures due to proprioceptive sensory loss, caused by lesions anywhere along proprioceptive sensory pathways (pseudochoreoathetosis), but they are usually unilateral and unique regarding the type of movement. This is one of the ﬁrst cases described where the same injury causes two different types of movements on two different parts of the body. References: Frank R. Sharp, MD; Thomas A. Rando, MD, PhD; Steven A. Greenberg, MD, Pseudochoreoathetosis. Movements Associated With Loss of Proprioception. E.K. Tan, MD; Y.L. Lo, MD; L.L. Chan, FRCR; S.J. See, MD; A. Hong, FRCS; and M.C. Wong, FRCP,Cervical disc prolapse with cord compression presenting with choreoathetosis and dystonia. 2 Man Carrying a Diagnosis of “Parkinson’s Disease” with a Prolonged History of Stuttering Speech - A Case Report Prashant Natteru (Jackson, MS, United States), Juebin Huang (Jackson, MS, United States) Dystonia 1 Choreoatetosis and Writer’s Cramp Associated with Radicular Compression by Cervical Hernia Natalia Gonzalez Rojas (La Plata, Argentina), Javier Ziliani (La Plata, Argentina) Objective: To report a patient who developed choreoathetoid movements on the left arm, and task speciﬁc dystonia (writer’s cramp) on the right one due to a radicular compression by cervical herniation. Background: Dystonias are a diverse group of movement disorders characterized by sustained or intermittent muscle contractions causing abnormal, often repetitive, movements, postures, or both. Writer’s cramp is one of the more recognized forms of focal task speciﬁc dystonia, is S30 Objective: To report the clinical, radiographic and genetic features of an atypical case of Pantothenate Kinase-Associated Neurodegeneration (PKAN). Background: PKAN, often caused by mutations in the pantothenate kinase (PANK2) gene, is the most common disorder associated with abnormal brain iron accumulation in the basal ganglia. Classic PKAN has an early onset of progressive dysarthria, rigidity, dystonia, and choreoathetosis, whereas atypical PKAN has an onset in the 2nd-3rd decade, a slower insidious course with speech defects and psychiatric disturbances. As the phenotypic spectrum of PKAN varies widely, recognizing and diagnosing PKAN can be challenging. Methods: Case report. Results: A 54-year-old right-handed Hispanic man presented with hand tremor, forceful eye closure and gait disturbance for ten years. He also reported having a stutter since age 15 years which has worsened in recent years. He carried a diagnosis of Parkinson’s disease since his forties and had been taking carbidopa/levodopa for several years with no clear improvement. A brain MRI done 7 years ago reported unremarkable. There was no family history of similar conditions. Neurological examination revealed severe blepharospasm, oromandibular dyskinesia, palilalia, bilateral hand tremor, mild rigidity, bradykinesia and a shufﬂing gait with frequent freezing. Brain MRI this time around showed abnormal signal MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS changes in the basal ganglia consistent with the “eye-of-tiger” sign. An [123I] ioﬂupane SPECT study demonstrated normal uptake in bilateral caudate and putamen. PANK2 genetic testing revealed a heterozygous pathogenic variant in exon 6 (Gly521Arg) and a variant with uncertain signiﬁcance on exon 2 (Asp265Glu), strongly supporting a diagnosis of PKAN caused by compound heterozygous mutations. Conclusions: The unrecognized initial presentation of stuttering speech 30 years prior to his other movement disorder symptoms and the initially normal MRI brain study constitute forme fruste features of atypical PKAN which contributed to the delay of his diagnosis. References: Thomas, M., Hayﬂick, S.J., Jankovic, J. Clinical heterogeneity of neurodegeneration with brain iron accumulation (Hallervorden-Spatz syndrome) and pantothenate kinase-associated neurodegeneration. Mov Disord. 2004;19:36–42- Marshall RD, Collins A, Escolar ML, et al. Diagnostic and clinical experience of patients with pantothenate kinase-associated neurodegeneration. Orphanet J Rare Dis. 2019;14(1):174. Published 2019 Jul 12. doi:10.1186/ s13023-019-1142-1Hayﬂick SJ, Kurian MA, Hogarth P. Neurodegeneration with brain iron accumulation. Handb Clin Neurol. (2018) 147:293–305. 10.1016/B978-0-444-63233-3.00019-1. 3 A New Treatment for Cervical Vertigo with Botulinum Toxin Ib Odderson (Seattle, WA, United States) Objective: To report a new and unique treatment for cervical vertigo with botulinum toxin. Background: Cervical vertigo and cervicogenic dizziness can be quite debilitating and few treatments are available. The diagnosis is controversial, and often made by exclusion of other conditions such as benign paroxysmal positional vertigo. Similarly, the pathology and treatments are not well deﬁned. Methods: Case report. Results: A 54-year-old woman was hit by wave in the back ten years earlier. She subsequently developed neck pain 1-5/10 and her vertigo spells were especially associated with tightness and muscle tension in the left trapezius. She did not have BPPV and a course of prednisone and two cervical epidural steroid injections were all with short-term beneﬁts. A cervical MRI showed only mild L C3-4 foraminal stenosis.An EMG exam conﬁrmed dystonia in several neck muscles, and a diagnosis of cervical dystonia was made. She received a total 100 u of incobotulinumtoxinA to selected dystonic neck muscles. She has subsequently experienced consistent resolution of her vertigo symptoms and pain for a total of 6 treatments so far. Conclusions: This report presents a new and unique treatment for CV with chemodenervation of posterior cervical muscles. Especially, physicians treating patients with posttraumatic vertigo should be aware of the potential beneﬁts from chemodenervation with botulinum toxin. A possible explanation is that chemodenervation reduced muscle spasms and pain, and thereby reduced the proprioceptive input with improvement of her vertigo. 4 Remission in Oromandibular Dystonia Sahyli Perez Parra (Atlanta, GA, United States), Laura Scorr (Atlanta, GA, United States), Hyder Jinnah (Atlanta, GA, United States), Stewart Factor (Atlanta, GA, United States) Objective: To report the clinical characteristics of a case series of idiopathic oromandibular dystonia who experienced remission (OMD) Background: OMD is a focal dystonia involving lower face, jaw and tongue, which is more prevalent in women. It produces signiﬁcant impairment in quality of life. Treatment is complex and response is variable. Methods: Series of ﬁve cases. Results: Five patients with idiopathic OMD experienced clinical remission. Mean age at onset was 54.4 6.5 years. Mean duration of disease was 5 3.5 years. All patients were women and had jaw-opening OMD with only one patient presenting with a combination of jawopening and deviation. All patients also had lingual protrusion dystonia. Dystonia in other regions was found in 2 patients, one with blepharospasm and one with cervical dystonia. On average 14.4 3.4 sessions of botulinum toxin therapy were performed with either Onabotulinumtoxin A, Incobotulinumtoxin A or Abobotulinumtoxin A. Mean duration of remission was 16.4 21.0 months. Conclusions: To our knowledge, remission of OMD has not been previously reported. Remission in cervical dystonia has been described, but usually in the ﬁrst year after onset of the disease. In our cases the duration of the disease was far longer. It will be important to investigate the frequency of remission and whether there are any clear predictors. References: 1. J.G. Nutt, M.D. Muenter, A. Aronson, L.T. Kurland, L.J. Melton 3rd. Epidemiology of focal and generalized dystonia in Rochester, Minnesota. Mov. Disord., 3 (1988), pp. 188-194 2. P. Gonzalez-Alegre, R.L. Schneider, H. Hoffman. Clinical, etiological, and therapeutic features of jaw-opening and jaw-closing oromandibular dystonias: a decade of experience at a single treatment center. Tremor. Other Hyperkinet. Mov. (N Y), 4 (2014), p. 231 5 Measuring the Brain Activity in Upper Limb Dystonia During the Finger-Tapping Task: A Comparison Between Functional Magnetic Resonance and Near Infrared Spectroscopy Artur José Paulo (Santo Andre, Brazil), Danilo De Faria (Sso Paulo, Brazil), Joana Balardin (S~ ao Paulo, Brazil), Renata Proa Lucca (S~ ao Paulo, Brazil), Carlos Baltazar (S~ ao Paulo, Brazil), Joao Ricardo Sato (S~ ao Paulo, Brazil), Vanderci Borges (Sao Paulo, Brazil), Sonia Maria Cesar de Azevedo Silva Moura MagalhÃ (Sao Paulo, Brazil), Henrique Ferraz (Sao Paulo, Brazil), Patricia Maria Carvalho Aguiar (S~ ao Paulo, Brazil) Objective: To explore brain activation using functional near-infrared spectroscopy (fNIRS) comparing with functional magnetic resonance (fMRI) in individuals with upper limb dystonia. Background: fNIRS is a new low-cost technique that allows to register cortical activation in unconstrained environments in task that induces dystonic postures (e.g. writing, playing instruments). This is the ﬁrst study that compares fNIRS and task-fMRI in a highly selected sample of patients with upper limb dystonia. Methods: Twenty-seven patients with idiopathic right upper limb dystonia and twenty-six age-matched controls, all right-handed, were assessed during three ﬁnger-tapping conditions (right hand, left hand and both hands), four blocks each. Functional images were acquired in a 3.0 T equipment (Siemens® - PRIA) and in a portable 8x8 fNIRS system (NIRx Medical Technologies). Differences between patients and controls were assessed by a two-tailed t-test for independent samples considering each voxel (fMRI) or each channel (fNIRS). Results: Brain areas associated with ﬁnger tapping with right and left hand from fMRI and fNIRS are shown in Figure 1. We observed lateralization in BOLD and Deoxy-Hemoglobin (Hb), but with regard to Oxy and Total-Hb we found a more bilateral pattern. Upper limb dystonia patients showed lower Oxy-Hb [Figure2.A] and Total-Hb [Figure2.B] in left frontal cortex and ipsilateral somatosensory areas during ﬁnger tapping with the right (affected) hand. Conversely, we found increased activation (fMRI) in right cerebellar lobules in dystonia patients under the same condition, as shown in Figure 3. We did not detect differences between groups on the remaining conditions (fMRI), neither cortical differences in terms of Deoxy-Hb in any condition (fNIRS). Conclusions: To our knowledge, this is the ﬁrst study comparing fMRI and fNIRS in dystonia. We found similar cortical maps comparing Deoxy-Hb (fNIRS) and BOLD (fMRI) signals in both patients and control. With fMRI, we detected a higher cerebellar activation ipsilateral to the dystonic. Through Oxy-Hb and Total-Hb (fNIRS) we found lower activation in right somatosensory and left frontal cortex in dystonia. Due to its portability, fNIRS enables measurements of cortical activity in natural conditions, opening new possibilities to explore the pathophysiology of dystonia and other movement disorders. References: Balardin, J. B., Zimeo Morais, G. A., Furucho, R. A., Trambaiolli, L., Vanzella, P., Biazoli Jr, C., & Sato, J. R. (2017). Imaging brain function with functional near-infrared spectroscopy in unconstrained environments. Frontiers in human neuroscience, 11, 258. Rothkirch, I., Granert, O., Knutzen, A., Wolff, S., Gövert, F., Pedersen, A., & Witt, K. (2018). Dynamic causal modeling revealed dysfunctional effective connectivity in both, the cortico-basal-ganglia and the cerebello-cortical motor network in writers’ cramp. NeuroImage: Clinical, 18, 149-159. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S31 ABSTRACTS S32 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS 6 Dystonia-parkinsonism Syndrome in GM1 Type 3 Gangliosidosis Gustavo Franklin (Curitiba, Brazil), Nayra Lima (Curitiba, Brazil), Helio Teive (Curitiba, Brazil) Objective: To describe a singular case of man who presented as a generalized dystonia and parkinsonism, with no family history of neurological diseases, that after extensive investigation was diagnosed as GM1 Type 3 Gangliosidosis. Background: GM1 gangliosidosis is an autosomal recessive disorder due to acid beta-D-galactosidase deﬁciency. Type 3 is characterized by later onset and and variable symptoms, from movement disorders, skeletal changes, cognitive impairment and many others. Clinical descriptions of this form are rare, particularly in non-japanese patients. Generalized dystonia remained the predominant feature throughout the disease course and was often associated with akinetic–rigid parkinsonism (1). Classic signs may give a diagnostic clue, such as facial grimacing, and MRI changes, with wish bone ﬁndings (2).Here, we describe a patient with genetic conﬁrmation GM1 gangliosidosis with preserved cognitive and without the described classical signs. Methods: Case report. Results: A 23 years-old Caucasian man presented with hip abnormalities, scoliosis, bilateral femur head necrosis by the age of 7 years-old. In that time, he went to bilaterally surgical approach. At 14 years-old starts the development of dystonia in superior arms, which progressed to dysarthria, blepharospasm, cervical and truncal dystonia.The clinical examination of the patient showed a short stature, with a disproportionate headto-trunk ratio. Presented yet a generalized dystonia and a marked bradykinesia of limbs with signiﬁcant gait disability. There was no oromandibular dystonia or oculomotor abnormalities. In the diagnostic investigation, the MRI was unremarkable. A genetic panel was performed, identifying a mutation in the GLB1 gene. Conclusions: GM1 gangliosidosis must be considered as a cause of early-onset generalized dystonia, particularly in patients with short stature and skeletal dysplasia. References: Yoshida K, Oshima A, Sakuraba H, et al. GM1 gangliosidosis in adults: clinical and molecular analysis of 16 Japanese patients. Ann Neurol 1992;31:328 –3322. Maciel R O H, Pedroso J L, Barsottini O G P. Facial grimacing as a clue for the diagnosis of GM1 type 3 gangliosidosis. Arq. Neuro-Psiquiatr. 2011; 69(2b): 406-407. 3. Muthane U, Chickabasaviah Y, Kaneski C, et al. Clinical features of adult GM1 gangliosidosis: report of three Indian patients and review of 40 cases. Mov Disord 2004;19:1334 –13414. Roze, E., Paschke, E., Lopez, N., Eck, T., et al Roubergue, A. (2005). Dystonia and parkinsonism in GM1 type 3 gangliosidosis. Movement Disorders, 20(10), 1366–1369.5.Hirayama M, Kitagawa Y, Yamamoto S, et al. GM1 gangliosidosis type 3 with severe jaw-closing impairment. J Neurol Sci 1997;152: 99 –101. 7 Deep Brain Stimulation (DBS) as Treatment of Childhood Onset Dystonia: Experience of 13 Chilean Patients Daniela Munoz (Santiago, Chile), Monica Troncoso (Santiago, Chile), David Aguirre (Santiago, Chile), Emilia Zambrano (Santiago, Chile), Ramiro Zepeda (Santiago, Chile), Sebastian Monsalves (Santiago, Chile), David Mendez (Santiago, Chile), Rodrigo Catalan (Santiago, Chile), Andres De La Cerda (Santiago, Chile), Olga Benavides Canales (Santiago, Chile), Roque Villagra (Santiago, Chile), Valentina Naranjo (Santiago, Chile), Maria Jose Hidalgo (Santiago, Chile), Isadora Ruiz (Santiago, Chile), Alvaro Retamales (Temuco, Chile), Kay Gittermann (Punta Arenas, Chile), Eliana Jeldres (Rancagua, Chile) Objective: The aim of this study is to evaluate outcome of DBS treatment in patient with chilhood onset dystonia. Background: Dystonia is a painful and disabling disorder often refractory to drug treatment. Deep brain stimulation (DBS) in the internal pale globe (Gpi) has been proposed as an effective treatment of patients with refractory dystonia Methods: We study chilhood onset dystonia patients treated with DBS between the years 2014-2019. Dystonia severity was meassured preDBS and 1, 3, and 6 month after DBS. We used Burke-Fahn-Mardsen Dystonia Scale (BFMDRS), applying its 2 subscales: motor and disability. All patients have video registration of each control. Results: 13 patients were analyzed. All of them with bilateral Gpi DBS. 8 men, 5 women. Average age dystonia onset was 8.5 years. Etiologies: Primary dystonia 5 patients (1 DYT1, 1 DYT 5, 1 DYT MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S33 ABSTRACTS 24, 1 mutation SLC6A3 gene, 1 mutation KMT2B gene); 5 Secondary dystonia (2 PKAN, 1 Kernicterus, 2 late dystonia); 3 unknown etiology. Each patient was evaluated with BFMDRS scale pre-DBS and in controls of 1, 3 and 6 months. The average pre-DBS BFMDRS score was 71.4 / 21.5pts on motor / disability scales respectively. The average score in controls of 1, 3 and 6 months was 35.2 / 15.2pts, 29.5 / 12.2pts and 29.2 / 11.9pts respectively. The average improvement percentage was 61.2% on motor scales and 52.3% on disability scales. When we analyzed each group, the percentages of improvement were 63.4% / 66.6% primary dystonia, 58.6% / 53.65% secondary dystonia and 55.2% / 41% in dystonia of non-precise etiology. 3 patients achieved 100% improvement of dystonic symptoms, all of them were patients with primary dystonia. Conclusions: Our study conﬁrms the efﬁcacy of this therapy, especially in patients with primary dystonia of genetic origin. This is the ﬁrst Chilean series that describes the response of pediatric patients with dystonia to DBS. References: Bronte-Stewart H, Taira T, Valldeoriola F, Merello M, Marks WJ Jr, Albanese A, Bressman S, Moro E. Inclusion and exclusion criteria for DBS in dystonia. Mov Disord. 2011 Jun;26 Suppl 1:S5-16.Jinnah HA, Alterman R, Klein C, Krauss JK, Moro E, Vidailhet M, Raike R. - - Deep brain stimulation for dystonia: a novel perspective on the value of genetic testing. J Neural Transm 2017 Apr;124(4):417-430.Moro E, LeReun C, Krauss JK, Albanese A, Lin JP, Walleser Autiero S, Brionne TC, Vidailhet M. Efﬁcacy of pallidal stimulation in isolated dystonia: a systematic review and meta-analysis. Eur J Neurol. 2017 Apr;24 (4):552-560. 8 Childhood Onset of Spinocerebellar Ataxia 3: Tongue Dystonia as an Early Manifestation Nester Mitchell (Grenville, Grenada), Gaynel LaTouche (St.Patrick, Grenada), Beverly Nelson (St.George, Grenada), Karla Figueroa (Salt Lake City, United States), Ruth Walker (Bronx, NY, United States), Andrew Sobering (St. George’s, Grenada) Objective: We describe a 10-year-old Afro-Caribbean female who presented with tongue dystonia as an early manifestation of spinocerebellar ataxia 3 (SCA3). Background: SCA3 is an autosomal dominant neurodegenerative disorder with a CAG trinucleotide repeat expansion in exon 10 of the ATXN gene. Clinical ﬁndings ascribe to cerebellar degeneration, movement disorders, parkinsonism and dystonia. Methods: Neurological evaluations were performed at a pro bono movement disorders clinic in a resource-limited community. Due to the small population size of the Caribbean island where our patient and family reside our IRB has requested that we do not reveal their precise nationality or geographic locations. DNA isolated from whole blood was analyzed by PCR ampliﬁcation of the ATXN3 CAG repeat region gene followed by capillary electrophoresis with internal standards. Results: The patient developed problems with gait and speech at age 10. She was doing reasonably well in school, albeit with limitations due to motor incoordination. Physical examination approximately 1 year after symptom onset revealed a dystonic, hypertrophic tongue, dystonic hand and foot posturing, marked nystagmus of eye movements, mild dysdiadochokinesis, and an ataxic gait. Mini-mental examinations revealed no cognitive decline. Brain MRI was grossly normal. Capillary electrophoresis of PCR ampliﬁed products of the repeat region in ATXN3 revealed 84 and 22 CAG repeats conﬁrming diagnosis of SCA3. Conclusions: The primary feature of SCA3 is ataxia due to cerebellar degeneration. Dystonia and other manifestations may occur due to brainstem degeneration and dysfunction of oculomotor, pyramidal pathways, motor neurons, and peripheral nerves. To our knowledge, tongue dystonia not been previously reported in SCA3. Possible treatment options for tongue dystonia are limited and carry signiﬁcant risks. The absence of specialists, laboratories for genetic testing and socioeconomic constraints creates a signiﬁcant challenge in diagnosing and managing patients with movement disorders on a small resource limited Caribbean island. References: Childhood-onset spinocerebellar ataxia 3: tongue dystonia as an early manifestation. Tremor Other Hyperkinet Mov. 2019. doi: 10.7916/tohm.v0.704. Manuscript in press. S34 9 Delay in Diagnosis and Impact of Dystonia in Patient’s Disability Renata Gabriela Vargas (Brasilia, Brazil), Talyta Grippe (Brasília, Brazil), Gustavo Bachtold (Brasilia, Brazil), Flavio Pereira (Brasilia, Brazil), Marcelo Lobo (Guará Brasilia, Brazil), Ariely Borges (Brasilia, Brazil), Francisco Cardoso (Belo Horizonte, Brazil) Objective: To evidence the time of diagnosis conﬁrmation in dystonic patients and other impacts of the disease in the patient’s lives. Background: The diagnosis of dystonia is mostly based on clinical signs, and the diagnosis and etiological deﬁnition of this disorder remain a challenge. Besides the clinical problems of involuntary abnormal postures and repetitive movements frequently associated with pain, patients with dystonia present with a wide range of social disabilities and impairments in health-related quality of life. Methods: A transversal study was conducted with 37 patients with the diagnosis of primary or secondary dystonia, followed in a tertiary movement disorders outpatient clinic in Brazil. The patients were asked about the time between the onset of symptoms and the deﬁnitive dystonia diagnosis personally or by phone. The number of diagnostic errors, the length of treatment, the patient’s ability to continue occupational activities, and the patient’s view of treatment were assessed. Results: 37 patients were interviewed, with a mean age of 49.5 (18 – 81) years. Regarding the diagnostic delay, there was an interval from 3 months to 10 years for diagnostic certainty, and 78% (n = 29) of these patients were diagnosed after 1 year or more from the onset of symptoms. Inaccurate diagnoses were given to 32% (n = 12) of these patients at ﬁrst glance, 19% (n=7) of the patients had the symptoms attributed to psychogenic issues. Moreover, 68% (n = 25) were not diagnosed at the ﬁrst visit. Regarding occupational activities, 54% (n = 20) were retired due to disability secondary to dystonia, 18% (n = 7) were already retired when diagnosed and only 28% (n = 10) keep following activities within limitations. In this study, 67% (n = 25) patients have more than 10 years of botulinum toxin treatment, while 33% (n = 12) range between 3 and 9 years of treatment. Finally, 72% (n = 27) of respondents effectively realize the beneﬁts of toxin treatment. Conclusions: This study highlights the struggling time between the symptoms and the diagnosis of dystonia and also estimates the economic impact of the disease in a population previously work actively. It is essential to acknowledge all the difﬁculties regarding this condition in order to establish better goals for better assistance to the dystonic patient. References: Balint B, Mencacci NE, Valente EM, et al. Dystonia. Nat Rev Dis Primers. 2018 Sep 20;4(1):25.2. MordinM, Masaquel C, Abbott C,et al. Factors affecting the health-related quality of life of patients with cervical dystonia and impact of treatment with abobotulinum toxin A (Dysport): results from a randomised, doubleblind, placebo controlled study. BMJ Open. 2014 Oct 16;4(10):e005150. 10 Striatal Injury in Early X-Linked Dystonia Parkinsonism Affects Both Matrix and Striosomes Jeff Waugh (Dallas, TX, United States), Norbert Brueggemann (Luebeck, Germany), Nutan Sharma (Charlestown, MA, United States), Hans Breiter (Chicago, IL, United States), Anne Blood (Charlestown, MA, United States) Objective: To assess the integrity of the striosome and matrix compartments of the human striatum in vivo using diffusion tensor imaging in two forms of dystonia: idiopathic cervical dystonia (ICD) and earlyphase X-linked dystonia-parkinsonism (XDP). Background: A postmortem histochemical study of XDP suggested that tissue loss may occur ﬁrst or most severely in the striosome compartment, with relative preservation of the matrix. This controversial hypothesis has not been tested directly in vivo. Methods: We acquired high-resolution 3T structural and diffusion MRI sequences in 17 XDP patients (mean duration: 2.9 years), 17 ICD patients, and matched healthy controls. We generated probabalistic diffusion tractography using FSL-probtrackx2, classiﬁcation targets mode, to quantify the differential probability of connection of each striatal voxel with striosome-weighted or matrix-weighted targets (deﬁned using animal tract tracing studies). Striatal voxels were categorized as “striosomelike,” “matrix-like,” or mixed-connectivity. After parcellating all striatal MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS voxels we compared radial and axial diffusivity (RD, AD) between XDP, ICD and controls. Results: In the early phase of XDP, structural MRI demonstrates 38-44% volume reduction in caudate, putamen, and pallidum (p<4x10-6, each region). These regions in ICD had volumes similar to those of healthy controls. Markers of free-water movement (RD, AD) were higher in XDP in both striatal compartments, but were elevated more in matrix-like (71-77% increase, p <3.4x10-6) than in striosomelike voxels (45-56% increase, p<1.6x10-7). In XDP, RD and AD had little correlation with age or dystonia severity, but correlated positively with disease duration in both compartments, with higher rates of increase in matrix-like voxels. RD and AD were no different in ICD patients and controls. Conclusions: Striatal atrophy in early XDP is not restricted to the striosomal compartment: striatal volume loss (>40%) substantially exceeded the total expected volume of the striosomal compartment (15%). Likewise, diffusivity was signiﬁcantly increased in both striatal compartments, but was elevated more in matrix. Diffusivity increased with disease duration, paralleling progressive neuronal loss; the rate of increase was greater in matrix. These ﬁndings suggest that striatal atrophy in the early phase of XDP affects both striatal compartments, and may preferentially impact the matrix compartment. 11 Hyperexcitability Neurophisiological Measures in Dystonic Patients Natália Cunha (Brasília, Brazil), Talyta Grippe (Brasilia, Brazil), Rubens Fernandez (Brasilia, Brazil), Francisco Cardoso (Belo Horizonte, Brazil), Raphael Boechat (Brasilia, Brazil) Objective: To compare the neurophysiological tests which measure cortical excitability between dystonic patients and health controls. Background: Dystonia is a disease that is believed to have an increase in brain excitability that can be shown in neurophysiological studies, such as: an increase in the silent cutaneous period duration and a tendency to decrease H max/M max ratio. Methods: Two groups of patients were studied: a control group and a dystonic group. We perfomed neuroconductive tests, to exclude patients with any other diseases from the peripheric nervous system. Other neurophysiological tests (silent cutaneous period (CuSP) and H reﬂex/ Maximum M wave (H max / M max) ratio were also performed to evaluate the patients. The results were reviewed by an experienced blind examiner and the numeric data was evaluated with the JASP software, using the Mann-Whitney test. Results: Thirteen dystonic patients and 59 normal controls were evaluated. The neuroconductive studies were unremarkable. The cutaneous silent period (CusP) duration was not statistically different comparing dystonic and control patients. The CusP latency was shorter in the dystonic comparing to the controls in the right (p=0,03) and left side (p=0,04). The relation Hmax/Mmax was lower in the dystonic patients regarding the right (p=0,007) and the left side (p=0,02) comparing to controls.[table1] Conclusions: This study shows the differences between dystonic and controls in the neurophysiological tests, corroborating the hyphotesis that there is a measurable hyperexcitability in dystonia. And helping to further deﬁne the normative values for these measures, allowing future comparisons References: 1. Fahn S. Concept and classiﬁcation of dystonia. Adv Neurol. 1988; 50:1-8. Suppl_5.S1 2. Morgante F, Klein C. Dystonia. Continuum (Minneap Minn). 2013 Oct;19(5 Movement Disorders):1225-41. 3. Steeves TD, Day L, Dykeman J, Jette N, Pringsheim T. The prevalence of primary dystonia: a systematic review and metaanalysis. Mov Disord. 2012 Dec;27(14):1789-96 4. Albanese A, Bhatia K, Bressman SB, et al. Phenomenology and classiﬁcation of dystonia: a consensus update. Mov Disord 2013; 28:863-873 5. Conte, A, et al. Nonmotor symptoms in patients with adult-onset focal dystonia: sensory and psychiatric disturbance. Parkinsonism and Related Disorders, 2015,1-4. 6. Berman BD, Junker J, shelton e, et al. J Neurol Neurosurg Psychiatry 2017;88:595-602. 7. Lange F, Seer C, Salchow C, Dengler R, Dressler D, Kopp B (2016) Meta-analytical and electrophysiological evidence for executive dysfunction in primary dystonia. Cortex 82:133-146 8. Czekóová K, Zemánková P, Shaw DJ, Bareš M. Social cognition and idiopathic isolated cervical dystonia . p; A, Rizos A, Jost W, Warner TT, Chaudhuri KR (2014) Nonmotor symptoms and focal cervical dystonia: observations from 102 patients. Basal Ganglia 4(3):117-1209. Eichenseer SR, Stebbins GT, Comella CL. Beyond a motor disorder: a prospective evaluation of sleep quality in cervical dystonia. Parkinsonism Relat Disord 2014;20(4):405-8. 10. Fabbrini G et al. Diffusion Tensor Imaging in patients with cervical dystonia and blepharospasm. European Journal of Neurology 2008, 15: 185-189 11. Hallet M. Neurophysiology of Dystonia: The Role of Inhibition. Neurobiol Dis. 2011 May ; 42(2): 177-184. 12. Berardelli A, et al. Pathophysiology of blepharospasm and oromandibular dystonia. Brain. 1985; 108:593-608 13. Espay AJ, et al. Cortical and spinal abnormalities in psychogenic dystonia. Ann Neurol. 2006; 59:825- 34. 14. Antelmi E, Erro R, Rocchi L et al. Neurophysiological correlates of abnormal somatosensorytemporal discrimination in dystonia. Mov Disord. 2017 Jan;32(1):141-148. 15. Stephen Tisch, MBBS, Limousin, Patricia et al. Changes in Blink ReﬂexExcitability after Globus Pallidus Internus Stimulation for Dystonia. Mov. Disord. 2006.16. Nakashima K, Rothwell JC, Thompson PD, et al. The blink reﬂex in patients with idiopathic torsion dystonia. Arch Neurol 1990;47: 413– 416.17. Aramideh M, Ongerboer de Visser BW. Brainstem reﬂexes:elec- trodiagnostic techniques, physiology, normative data and clinical applications. Muscle Nerve 2002;26;14 –30.18. Esteban A. A neurophysiological approach to blink reﬂexes: blink reﬂex.Neurophysiol Clin 1999;29:7–38.19. Basso MA, Powers AS, Evinger C. An explanation for reﬂex blink hyperexcitability in Parkinson’s disease: I, superior colliculus. J Neurosci 1996;16:7308 –7317. 20. S.L.Pullman, B, Ford et al. Cutaneos electromyographic silent period ﬁndings in brachial dystonia. Neurology, 1996. 21. Misiaszek, John E. The H-reﬂex as a tool in neurophysiology: its limitations ans uses in understanding nervous system function. 12 Non-Motor Symptoms in Patients with Primary Craniocervical Dystonia Talyta Grippe (Brasília, Brazil), Gustavo Bachtold (Brasilia, Brazil), Matheus Moreno (Brasilia, Brazil), Natália Cunha (Brasília, Brazil), Francisco Cardoso (Belo Horizonte, Brazil) Objective: To characterize and evaluate the proﬁle of nonmotor symptoms of patients with primary dystonia in a referral center in Brazil. Background: Dystonia is a disease deﬁned by co-contraction of antagonist’s muscles. However, there is a high prevalence of nonmotor symptoms, which often precede motor symptoms and may be overlooked by doctors and patients. Methods: This was a prospective, cross-sectional, descriptive study. Questionnaires were applied to evaluate non-motor symptoms, including MOCA - cognition assessment, MMSS - general assessment of nonmotor symptoms and HADS - anxiety and depression assessment. Results: A total of 21 patients with primary cervicofacial dystonia answered the questionnaire, 52% men and 48% women. From the HADS scale cutoff point, 45% were classiﬁed as anxiety, and 45% as depressive. Besides, patients have a signiﬁcant cognitive impairment, with an average MOCA of 21.1 (11 - 27), which is lower than the average Brazilian population score of 26. The NMSS score showed the presence of 66% of patients with cardiovascular symptoms. 95% with sleep symptoms, 90% with mood change, 55% with perception symptoms, 77% with attention change, 66% gastrointestinal, 77% urinary and 50% with sexual dysfunction. Conclusions: There is a high prevalence of non-motor symptoms in patients with dystonia in the evaluated sample, showing that this aspect should always be explored and addressed by the attending physician. References: TORRES, Julie Ann Kristy L.; ROSALES, Raymond L. Nonmotor symptoms in dystonia. In: International review of MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S35 ABSTRACTS neurobiology. Academic Press, 2017. p. 1335-1371.NORRIS, Scott A. et al. Clinical and demographic characteristics related to onset site and spread of cervical dystonia. Movement Disorders, v. 31, n. 12, p. 1874-1882, 2016.BEZERRA T C, et al. Epidemiological and clinical aspects of a sample of Brazilian patients with primary dystonia and the impact of the new classiﬁcation on their clinical evaluation. Arq Neuropsiquiatr. 2018;76(12):821-826. Education in Movement Disorders 94 The Use of Levodopa-Carbidopa Intestinal Gel in Parkinson Disease: Impact of Medical Clinician-Patient Medical Education on Knowledge and Conﬁdence Among Neurologists Thomas Finnegan (Glenside, PA, United States), Catherine Murray (New York, United States), Michael Okun (Gainesville, FL, United States), Irene Malaty (Gainesville, FL, United States) Objective: To evaluate the effectiveness of an online educational intervention to improve knowledge and conﬁdence among neurologists regarding optimal patient selection and adverse events associated with LCIG. Background: Deep brain stimulation (DBS) and levodopa carbidopa intestinal gel (LCIG) are a consideration for Parkinson disease (PD) modulatewhen patients have motor ﬂuctuations and dyskinesia in Parkinson disease (PD) that cannot be adequately managed with medication. Among the surgical interventions available for patients with PD is levodopa-carbidopa intestinal gel (LCIG). Data show has suggested that neurologists lack knowledge and conﬁdence regardinglating to the clinical use of LCIG. Methods: The online continuing medical education (CME) activity format consisted of a semi-structured 30-minute video discussion with two movement disorder physician experts and two patients with PD. Educational effect was assessed by comparing a matched sample of neurologists’/PCPs’ responses to four identical questions presented before and directly after exposure to the intervention. A chi-square test was used to identify signiﬁcant differences between pre- and post-assessment responses. Cramer’s V was used to calculate the effect size of the online education. Data from the participants were collected between February 5, 2019 and March 14, 2019. Results: Participation in the CME intervention improved knowledge as reﬂected by the effect size among neurologists receiving education (n=205; V=.170). The following areas showed signiﬁcant (P <0.05) prevs post-educational improvements: First, the identiﬁcation of patients who may beneﬁt from LCIG therapy (110% relative pre-vs post education improvement) and second distinguishing potential adverse events of S36 LCIG versus DBS (23% relative pre-vs post education improvement). The education did not result in a signiﬁcant pre-post change regarding contraindications for the use of LCIG. After the activity, 39% of neurologists reported improved conﬁdence in selecting an appropriate surgical intervention for PD patients. Conclusions: The results indicated that the easily disseminated CME-certiﬁed 30-minute video discussion between physician experts and patients was effective at improving knowledge regarding the use of the LCIG for the management of PD. Future education efforts should continue to address the similarities and differences between available surgical options for the management of PD, including the contraindications to surgical interventions. References: Okun MS and Malaty IAC. When Good Isn’t Good Enough: Patient and Clinician Perspectives in the Surgical Management of PD. 2019. https://www.medscape.org/viewarticle/907850. 95 Central American Movement Disorders Work Group (CAMDWG)/MDS-PAS Afﬁliate Society, and the Impact in the MDS-PAS Educational Programs: 2011-2019 Jose Ricardo Lopez-Castellanos (Little Rock, AR, United States), Jose LopezContreras (San Salvador, El Salvador), Ernesto Cornejo-Valse (Quezaltepeque, La Libertad, El Salvador), Fernando Gracia (Panama, Panama), Norbel Roman-Garita (San Jose, Costa Rica), Walter Diaz (Managua, Nicaragua), Alex Medina (Tegucigalpa, Honduras), Heike Hesse (Tegucigalpa, Honduras), Jose Lopez-Contreras (San Salvador, El Salvador), Marcia Castillo (Distrito Nacional, Dominican Republic) Objective: To describe the activities carried out by CAMDWG supported by MDS-PAS Educational programs and its achievements in Central America and the Dominican Republic. Background: Continuous medical education is essential to improve fund of knowledge in healthcare professionals worldwide. In Central America, high quality education in Movement Disorders is insufﬁcient due to limited resources. Methods: We reviewed all educational activities that were carried out by CAMDWG with the support of MDS-PAS Educational Committee in Central America and the Dominican Republic. Results: From 2011 to 2019, CAMDWG developed 8 educational activities supported by MDS-PAS Educational Programs: 2 Ambassador Programs, 5 Developing World Education Programs, 3 MDS Supporting Meetings, and 1 Virtual Professor Program. Total attendance for all combined events was 2,775. 75.9% (N: 2,106) Neurologist and Non-Neurologist, 24.1% (N: 669) patients and caregivers. CAMDWG achieved a greater attendance when the events were developed in conjunction with a local or regional conference. Resources provided by MDS-PAS were optimized by carrying out more than 2 simultaneous events for NonNeurologists, Neurologists, patients and caregivers. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Conclusions: The Central American Movement Disorders Work Group (CAMDWG) is an important regional strategic initiative to disseminate knowledge in Movement Disorders supported by MDS-PAS Educational Programs. References: KESSLER, D.; LIDDY, C. Self-management support programs for persons with Parkinson’s disease: An integrative review. Patient Education and Counseling. 2017. 97 96 The Long-Term Effects of a Multidisciplinary Program Based on Centered People Care Model for Health Education of People Living with Parkinson’s Disease Maria Elisa Piemonte (Sao Paulo, Brazil), Cynthia Dias (Sao Paulo, Brazil), Andressa Costa (Santo André, Brazil), Erika Okamoto (S~ ao Paulo, Brazil), Adélia Sartori (S~ ao Paulo, Brazil), Camila Silva (S~ ao Paulo, Brazil) Objective: The aim of this study was to investigate the long-term improvements in the health knowledge of the people living with PD after a multidisciplinary health education program. Background: According to the centered people care model advocated by the World Health Organization, for caring for people with chronic disease like PD, the Health education is not only critical to improving knowledge but encouraging health change actions. Methods: The multidisciplinary health education program consisted of 9 in-person lectures offered by professionals from different areas monthly to groups of 10 people. A survey to evaluate the knowledge improvement of key learning points of lectures before (BL), immediately (AL) and 30 days (30dAL) after each lecture was applied for a blinded examiner. The survey was consisted of 18 question (2 per lecture) where the minimum level (score 0) indicated the absence of knowledge and maximal level (score 5) indicated the full knowledge of lectures’ key points. The results were analyzed by ANOVA for repeated measure considering the factor the assessment points (BLXALX30dAL). Results: Twenty people with conﬁrmed diagnosis of idiopathic PD, 6 in stage 2, 8 in stage 3 and 6 in stage 6 of disease evolution according to Hoehn and Yahr Classiﬁcation, with 67 (SD = 9.4) years of age, 11.8 (SD = 6.9) years of education, 22.16 (SD = 6.4) in Montreal Cognitive Assessment, and 6.2 (SD = 4.2) in Geriatric Depression scale were included in this study. The ANOVA showed signiﬁcant short and long-term improvements in the survey’s scores for 16 of the 18 investigated issues (p <.001). Most importantly, for 12 of the 18 key points, people with PD reached a full level of long-term learning. Conclusions: The multidisciplinary health education program consisted of in-person lectures on how to manage the PD alterations was able to improve the level of knowledge on key points for management of motor and non-motor symptoms of PD, in people in initial to moderate stage of disease evolution without dementia. Experience with a Pro-Bono Movement Disorders Clinic in a Low-Income Country in the West Indies Andrew Sobering (St. George’s, Grenada), Nester Mitchell (Grenville, Grenada), Ashley Yearwood (True Blue, Grenada), Dolland Noel (St. George’s, Grenada), Ruth Walker (Bronx, NY, United States) Objective: To describe the impact of a once-yearly neurology outreach clinic on a small resource-limited island nation. Background: In developed countries, people with movement disorders typically have access to medical services aimed at diagnosis and management. Most low-income nations lack these resources due to socioeconomic disparities and/or geographic isolation. Methods: The St. George’s University Visiting Professors program funded travel and accommodation for a movement disorders-trained neurologist to perform a yearly movement disorders clinic on a small Caribbean island. Clinical evaluations and management recommendations were performed at the clinic and also during home visits. When appropriate, puriﬁed DNA was shipped to international collaborating research laboratories for genetic testing for triplet repeat expansion disorders, and exome sequencing. Continuing medical educational seminars help to train local physicians. Results: Over 4 years we offered more than 57 consultations. Clinical diagnoses included; 18 Parkinson disease (PD), 9 Huntington disease (HD), 4 spinocerebellar ataxia 3 (SCA3), 7 essential tremor (ET), 2 focal dystonia, 1 progressive supranuclear palsy. In the majority of cases medication adjustments were made, and in some cases the diagnosis was revised (e.g. from PD to ET). Annual follow-up visits were encouraged; we saw 20 patients more than once. Consultations were offered to 4 unaffected family members at risk of developing either HD or SCA3. As-yetundiagnosed disorders include early-onset spasticity/dystonia and ataxia accompanied by extreme photo sensitivity. In addition, we identiﬁed one patient with infantile-onset seizure and dystonia caused by homozygous loss-of-function mutations in SYNJ1 (OMIM 604297). Conclusions: Evaluation by a movement disorders-trained physician is critical for diagnosis and optimization of management for chronic conditions such as PD, and can make a signiﬁcant contribution, even if available only annually. In addition, diagnosis of genetic disorders and genetic counseling can be facilitated. A local DNA puriﬁcation lab, or saliva collection kits, allow collaboration with overseas research labs to enable genetic diagnosis when appropriate. Challenges to successful implementation include administrative support and ensuring that patients who are MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S37 ABSTRACTS unlikely to beneﬁt from a movement disorders consultation are not offered an appointment. 98 Development and Impact of the Hispanic Parkinson’s Advisory Council on the Education and Recruitment of the Hispanic PD Population in Clinical Research Anna Naito (Miami, FL, United States), Rebeca DeLeon (Miami, United States), Fernando Cubillos (Miami, FL, United States), Clarissa MartinezRubio (Miami, FL, United States), Camila Gadala-Maria (Miami, FL, United States), Karlin Schroeder (New York, NY, United States), Ruby Rendon (New York, United States), James Beck (New York, NY, United States), Ignacio Fernandez Mata (Cleveland, OH, United States) Objective: Develop a strategy to increase education and awareness of clinical research among the Hispanic Parkinson’s disease (PD) population in the US and increase Hispanic representation in the Parkinson’s Foundation’s clinical research studies. Background: The vast majority of clinical research studies for PD in the US largely constitute the Caucasian population and is not representative of the total PD population in the US, which may limit the understanding of the biological basis of PD. Methods: The Parkinson’s Foundation has launched a new Hispanic outreach initiative that will consist of convening a leadership advisory committee to strategize how to best engage the Hispanic PD population, and develop education, recruitment materials and events to increase participation in clinical research studies for PD. The initiative will form a national leadership committee that includes Movement Disorders Specialists who treat Hispanic populations, study coordinators/social workers and Hispanic People with Parkinson’s (PwP). The goal is to standardize outreach strategies and educational materials to increase awareness and participation in clinical research studies. The advisory committee will consist of national leaders of Movement Disorders clinicians and coordinators anchored at sites with high Hispanic presence, as well as Hispanic PD advocates. Results: The critical barrier for increasing the diversity in the PD population are: 1) limited access to translated educational resources or events in their native language and 2) being treated by a Movement Disorders clinician. The deliverables for this initiative include generating standardized educational materials for Hispanic PD education/recruitment for clinical studies, and to increase overall Hispanic enrollment by 10% for two of the Parkinson’s Foundation’s ﬂagship clinical research studies: Parkinson’s Outcomes Project (POP) and PDGENEration. Conclusions: The Hispanic Parkinson’s Advisory Council will aim to increase Hispanic representation in the PD population and empower the Hispanic PD community through education and advocacy. This work will lead to increased diversity in PD clinical research studies, which will widen our understanding of the biological basis of PD. Ultimately, the results of this initiative will lead to improved treatments and development of novel therapeutic compounds for PD. Emerging and Experimental Therapeutics 93 Mechanistic PK/PD Model of Neuroactive Steroid GABAA Positive Allosteric Modulation and Effects on TETRAS Assessment in Essential Tremor David Nguyen (Cambridge, United States), Anne Smith (Cambridge, MA, United States), Min Qin (Cambridge, MA, United States), Ethan Hoffmann (Cambridge, MA, United States), James Paskavitz (Cambridge, MA, United States), Angela Wehr (Cambridge, MA, United States), Suki Malhi (Cambridge, MA, United States), Amy Bullock (Cambridge, MA, United States), Abdul Sankoh (Cambridge, MA, United States), Albert Robichaud (Cambridge, MA, United States), Stephen Kanes (Cambridge, United States), James Doherty (Cambridge, United States), Michael Quirk (Cambridge, United States) Objective: Create a model of exposure-response (PK/PD) relationships for target engagement and tremor reduction in Essential Tremor S38 (ET) for brexanolone injection and SAGE-217 to predict biomarker and efﬁcacy PK/PD responses for SAGE-324. Background: PK/PD modeling allows knowledge translation across compounds of similar mechanisms, and the generation of testable hypotheses for clinical development. Model-based hypotheses were tested in SAGE-324 Phase 1 signal ﬁnding studies. Methods: A PK/PD model for neuroactive steroid, GABA-A receptor postitive allosteric modulators (PAMs) was generated using ET clinical trial data from brexanolone injection and SAGE-217 to inform SAGE324 development. We tested PK/PD model based hypotheses in three SAGE-324 cohorts: an 8:8 randomized placebo controlled qEEG study that tested the hypothesized PK/PD relationship for qEEG in healthy volunteers; and two open-label Essential Tremor cohorts (n=6 at 45 mg, n=5 at 60 mg) that tested the hypothesized PK/PD relationship for tremor reduction. Results: We found that qEEG and tremor reduction were well described by PK/PD modeling across all three compounds and that the clinically observed PK/PD responses for SAGE-324 were within conﬁdence bounds of the predicted response. The Essential Tremor Rating Scale (TETRAS) scores were modulated in exposure ranges where qEEG signals were observed, with reductions in tremor occurring at less than doubling of qEEG power and at well-tolerated exposures across all three compounds. Conclusions: Our mechanistic modeling platform describing the exposure response-relationship for target engagement (by qEEG) and efﬁcacy (through TETRAS) offers a unique opportunity to establish and utilize a common mechanism of action for neuroactive steroid-based GABA-A receptor PAMs in ET to predict responses for novel compounds in development. The results from our SAGE-324 Phase 1 signal ﬁnding study in ET patients were consistent with the PK/PD model predictions. This conﬁrmation derisks our modeling approach and supports a mechanistic PK/PD approach for informing Phase 2 trial development. Epidemiology 88 Screening of Risk Factors Associated with Movement Disorders in the Population of Santo Domingo de Heredia, Costa Rica Norbel Roman (San Jose, Costa Rica), Carolina Boza (San José, Costa Rica) Objective: To develop a screening for movement disorders (MD) and associated risk factors in the population of Santo Domingo de Heredia, Costa Rica. Background: Parkinson’s disease (PD) is considered the second most common neurodegenerative disease in elderly population. It is characterized by the loss of motor, cognitive, behavioral and other functions that lead to disability. In Costa Rica, an 8% national prevalence of PD is estimated. The toxicological and pesticide component has been reported in Costa Rica, as a risk factor of suffering from PD 2.6 times compared to non-exposure. Methods: Descriptive correlational study, 101 older adults selected “door to door” from the community of Santo Domingo de Heredia, an urban and rural area with one of the highest rates of aging in the country and economy based on coffee plantations. We developed a screening instrument evaluated and approved by the Parkinson Working Group of Central America, in addition to the 10/66 protocol from ADI. We analyzed data related to pesticide exposure, presence of symptoms associated with MD, cognitive functioning and risk factors associated with PD and dementia. Results: 101 adults over 65 years (average=75 years), 60% women. The most reported risk factor was high blood pressure (33.7%), followed by diabetes (27.7%), depression (20.8%), cardiovascular disease (19%) and cerebrovascular disease (9%). One third (36.5%) reported a history of work in agriculture, mainly coffee cultivation (88.6%), with 10% prolonged use of pesticides (>20 years). The presence of motor symptoms associated with MD was reported in 20% of those interviewed, the most frequent being bradykinesia, balance problems and gait alteration. Nonmotor symptoms were reported in 5%, with sleep disorders being the most frequent. Only one participant reported having a diagnosis of PD conﬁrmed by a neurologist. Regarding cognitive functioning, 5.5% have MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS mild cognitive impairment and 5.5% moderate impairment (possible dementia). Conclusions: Costa Rica is one of the areas in the world where a greater prevalence of movement disorders worldwide is estimated. There are no national statistics regarding the situation in our country. The results found in this pilot plan demonstrate a high prevalence of risk factors for neurodegenerative diseases in the elderly population of the community of Santo Domingo de Heredia. This ﬁrst approach reﬂects the need to develop public health strategies for risk reduction, as well as to activate actions with the support of international organizations. References: Wesseling C & Steeland K. Parkinson’s and Alzheimer’s diseases in Costa Rica: a feasibility study toward a national screening program. Global health action, 2013. Volume 6, Issue 1.GBD Colls. Global, regional and national comparative risk assessment of 79 behavioral, environmental and occupational, and metabolic risks or clusters of risks, 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015. Lancet, 1659-1724.Steenland K, Wesseling C, Román N, Quirós I, Juncos JL. Occupational pesticide exposure and screening tests for neurodegenerative disease among an elderly population in Costa Rica. Environ Res. 2013 Jan;120:96-101.Steenland K, Mora AM, Barr DB, Juncos J, Roman N, Wesseling C. Organochlorine chemicals and neurodegeneration among elderly subjects in Costa Rica. Environ Res. 2014 Oct;134:205-9. 89 Withdrawn by Author 90 Utilization of Public Health Services by People with Parkinson’s Disease Elaine Gregório (Florianópolis, Brazil), Rafaela Myra (Florianópolis, Brazil), Bárbara Dos Santos (Florianópolis, Brazil), Talita Kuhn (Florianopolis, SC, Brazil), Rodrigo Kanabben (Florianópolis, Brazil), Micheline Henrique Koerich (Florianópolis, Brazil), Alessandra Swarowsky (Fargo North Dakota, United States) Objective: The purpose of this study was to investigate the clinical and functional characteristics of users with Parkinson’s disease (PD) as well as the health services used by them in Primary Care in a city the south of Brazil. Background: Parkinson’s disease (PD) is a chronic and progressive neurodegenerative condition [1]. It affects the elderly population [2] and signiﬁcantly interferes with the family environment, social [3,4] and independence level of the individuals [5], increasing the use and expenses of health services [6,7]. Methods: This is a descriptive and study that analyzed 273 medical records of patients with PD, it occurred from January 2016 to December 2017. Data were extracted from the Information System of the city, through the analysis of the medical records registered with ICD-10 / G20, and analyzed using descriptive statistics. Results: The majority of the users were elders (92%), men (52.7%), married (65.2%) and had incomplete elementary school (28.2%). The most commonly reported signs and symptoms of PD were: resting tremor (65.2%), rigidity (57.1%) and bradykinesia (35.9%) and the main associated comorbidities were: systemic arterial hypertension (46.5%) and diabetes (16.8%). Approximately 17.2% were bedridden and 8.4% used wheelchairs for locomotion. There was a list of falls in 37% of the medical records (30.4% reported having some kind of consequence after falls). The most used health services were: medical consultations (100%), medication distribution (85%) and immunization (81.7%). Less than half of the users participated in groups at the basic health units (BHU) or in the community and approximately 40.3% of the individuals received a home visit by the Family Health Strategy (FHS). Conclusions: The results addressed in this research may contribute to understanding how care is offered and reorient health proposals and actions for this population in the municipality. References: 1.Wirdefeldt K, Adami HO, Cole P, Mandel J. Epidemiology and Etiology of Parkinson´s Disease: A Review of the Evidence. European Journal of Epidemiology, 2011; 26 ( S1): S1- S58. 2. Dorsey ER Constantinescu R, Thompson JP, Biglan KM, Holloway RG, Kieburtz K, Marshall FJ, Ravina BM, Schiﬁtto G, Siderowf A, Tanner CM. Projected number of people with Parkinson Disease in the most populous nations, 2005 through 2030. Neurology, 2007; 68 (5): 384-6. 3. Portilho VMC et al. Projecto ReNACE. Convivencia de Pacientes y Familiares con Familiares con la Enfermedad de Parkinson: Resultados Preliminares de la Fase I. Revista Cientíﬁca la Sociedade Española de Enfermeria Neurologica, 2012; v.0, (36): 31-38. 4. Salcedo AZ, Senosiain García JM, Riverol Fernández M, Anaut Bravo S, Díaz de Cerio Ayesa S, Ursúa Sesma ME5, M.C. Portillo MC. Elementos Clave em el Processo de Convivencia con la Enfermedad de Parkinson de pacientes y familiares cuidadores. Anales del Sistema Sanitario De Navarra, 2012; 37 (1):.69-79. 5. Savitt JM.; Dawson, VL.; Dawson TM. Diagnosis and Treatment of Parkinson Disease: Molecules to Medicine. The Journal of Clinical Investigation, 2006; 116 (7): 1744-54. 6. Dodel RC. et al. costs of Drug Treatment in Parkinson´s Disease. Movement Disorders, 1998; 13 (2): 249-54. 7. Siderowf AD, Holloway RG, Stern MB. CostEffectiveness Analysis in Parkinson´s Disease: Determining the value of Interventions. Movement Disorders, 2000; 15 (3): 439-45. 91 Parkinsonism and Parkinson’s Disease in Latin America: A 10/66 Group Population Base Study Jorge Llibre Guerra (La Habana, Cuba), Ivonne Jimenez (San Juan, Puerto Rico), Mariella Guerra (Lima, Peru), Daysi Acosta (Santo Domingo, Dominican Republic), Ana Luisa Sosa Ortiz (Mexico City, Mexico), Aquiles Salas (Caracas, Venezuela), Ana Rodriguez Salgado (La Habana, Cuba), Juan Llibre-Guerra (La Habana, ACT, Cuba), Matthew Prima (London, United Kingdom), Martin Prince (London, United Kingdom), Juan Libre-Rodriguez (La Habana, Cuba) Objective: To estimate the prevalence and main risk factors for Parkinson’s disease in a cohort of individuals aged 65 years or older in Latin America. Background: Parkinson’s disease (PD) is the second most common neurodegenerative disorder and carries a signiﬁcant global impact. However, most of the studies on PD prevalence, and overall impact in society has been limited to High-Income countries with few epidemiological studies in Low and Middle-Income Countries (LMIC). Methods: The 10/66 Dementia Research Group collected information on neurological symptoms via the NEUROEX assessment in population-based surveys of older adults living in LMIC. Data from 11,614 adults participating in the baseline assessment of the 10/66 study and living in Cuba, Dominican Republic, Peru, Puerto Rico, Venezuela, and Mexico were analysed. Diagnosis of PD was established using diagnosis algorithms according to UK Parkinson’s Disease Society Brain Bank’s clinical criteria. The diagnoses algorithm, based on speciﬁed criteria, were reviewed by neurologists to increase reliability and tested in the in Cuban sample. Crude and age-adjusted prevalence were determined overall and by age group, sex, period, and region. Results: Among a population cohort, 65 years or alder from six countries in LA, the prevalence rate of Parkinsonism per 1000 population was 8.04, and the prevalence of PD was 2.15 ( 95% CI 1.1-2.6 ). Prevalence estimates of PD increased with age from 1.1 to 5.3 per (65-69 vs 80 years or older, p<0.001). Age-adjusted prevalence rates were lower for women than for men. No signiﬁcant differences were found across countries, except for lower prevalence in Urban Peru (1.3). Among those identity with PD, less than 50% received a previous diagnosis (selfreported PD) or seek neurological attention. Conclusions: Frequency of Parkinsonism in a LA-based population was higher than in HIC, probably related to a higher frequency of vascular Parkinsonism. The reported prevalence of PD is similar to the one on developed countries, and the rate increases with age. A signiﬁcant proportion of cases with PD did not have a previous diagnosis or never seek neurological attention. Therefore, hospitals/clinics registries cannot be assumed representative of our true prevalence in our region. These ﬁndings underscore the need to improve public health programs for population that is currently undergoing rapid demographic aging and epidemiological transition. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S39 ABSTRACTS 92 Inﬂuences of White Matter Changes in Parkinsonian Signs in an Elderly Community-Dwelling Brazilian Population: The Pietà Study Thiago Vale (Juiz de Fora, Brazil), Maira Barbosa (Belo Horizonte, Brazil), Elisa França Resende (Belo Horizonte, Brazil), Karoline Carmona (Belo Horizonte, Brazil), Henrique Guimar~ aes (Belo Horizonte, Brazil), Rogerio Beato (Belo Horizonte, Brazil), Ana Paula Santos (Belo Horizonte, Brazil), Francisco Cardoso (Belo Horizonte, Brazil), Paulo Caramelli (Sao Paulo, Brazil) Objective: To evaluate the inﬂuences of white-matter hyperintensities (WHM) on the motor scores of the Uniﬁed Parkinson’s disease Rating scale (UPDRSm) in a Brazilian community-dwelling population of oldest-old individuals from the Pietà study. Background: WHM on T2-weighted brain MRI of the elderly population has been identiﬁed as a cause of motor impairment over time. Previous studies have shown that a high WMH volume were associated with mild parkinsonian signs (MPS), incident parkinsonism and dementia. There are only a few studies on this association in a community-dwelling population of oldest-old individuals. Methods: This is a cross-sectional analysis of 144 individuals with acceptable FLAIR brain MRI images from the Pietà study, a community-based survey of brain aging, carried out in Caeté, Southeast Brazil, from January through April, 2008. All subjects underwent a thorough functional, clinical, psychiatric and neurological evaluation, including the Functional Assessment Staging, Pfeffer’s Functional Activities Questionnaire, the UPDRSm, and a Brief Cognitive Screening Battery, consisting of the Mini-Mental State Examination, category ﬂuency test (animals/minute), clock drawing test and picture drawings memory test. Severity of WMH on axial FLAIR images were accessed using the Fazekas scale (absent/mild changes [Fazekas 0 and 1], moderate changes [Fazekas 2] and severe changes [Fazekas 3]. Results: Sample was comprised mostly by women (61.1%), with median age (interquartile range) of 79 (77-82) years and median of 3 (1-4) years of schooling. Seventy-nine (54.9%) patients had no cognitive impairment, 30 (20.9%) had cognitive impairment non-dementia (CIND) and 28 (19.4%) had dementia. Eighteen patients were classiﬁed as Fazekas 1, 78 as Fazekas 2 and 48 as Fazekas 3. Groups were statistically similar regarding sex, age, schooling years, cognitive proﬁle and UPDRSm scale. We then divided the sample into two groups, according to the median score of 1.5 points on the UPDRSm scale. We observed a marked poorer performance on cognitive tests by the group with UPDRSm score > 1.5 points. We also noted a higher prevalence of CIND and dementia in this group. Conclusions: Severity of WMH in this community-dwelling elderly population was not associated with MPS. However, a poorer cognitive performance was statistically associated with a higher UPDRSm score. Although cerebral small vessel disease manifesting as WMH can occur in asymptomatic elderly individuals, it is related mainly to vascular dementia in this age-group. We did not ﬁnd association between the severity of WMH with either MPS or cognitive decline or dementia. We postulate that the sample size and the mean age of the groups might have accounted for the lack of association. Likewise, when the sample is divided according to the median UPDRSm score of 1.5 points, the subset of individuals with higher UPDRSm scores had more cognitive decline and dementia. References: 1. P. Caramelli, M.T. Barbosa, E. Sakurai, E.L. Dos Santos, R.G. Beato, J.C. Machado, H.C. Guimaraes, A.L. Teixeira, G. Pieta Study, The Pieta study: epidemiological investigation on successful brain aging in Caete (MG), Brazil. Methods and baseline cohort characteristics, Arq Neuropsiquiatr 69(4) (2011) 579-84; 2. L. Pantoni, A. Poggesi, D. Inzitari, Cognitive decline and dementia related to cerebrovascular diseases: some evidence and concepts, Cerebrovasc Dis 27 Suppl 1 (2009) 191-6; 3. F.E. de Leeuw, J.C. de Groot, E. Achten, M. Oudkerk, L.M. Ramos, R. Heijboer, A. Hofman, J. Jolles, J. van Gijn, M.M. Breteler, Prevalence of cerebral white matter lesions in elderly people: a population based magnetic resonance imaging study. The Rotterdam Scan Study, J Neurol Neurosurg Psychiatry 70(1) (2001) 9-14; 4. S.H. Kreisel, C. Blahak, H. Bazner, D. Inzitari, L. Pantoni, A. Poggesi, H. Chabriat, T. Erkinjuntti, F. Fazekas, J.M. Ferro, P. Langhorne, J. O’Brien, P. Scheltens, M.C. Visser, L.O. Wahlund, S40 G. Waldemar, A. Wallin, M.G. Hennerici, Deterioration of gait and balance over time: the effects of age-related white matter change--the LADIS study, Cerebrovasc Dis 35(6) (2013) 544-53; 5. H.M. van der Holst, I.W. van Uden, A.M. Tuladhar, K.F. de Laat, A.G. van Norden, D.G. Norris, E.J. van Dijk, R.A. Esselink, B. Platel, F.E. de Leeuw, Cerebral small vessel disease and incident parkinsonism: The RUN DMC study, Neurology 85(18) (2015) 1569-77; 6. E.P.F. Resende, L. Costa-Silva, K.C. Carmona, T.H. Machado, J.C.B. Machado, H.C. Guimaraes, M.T. Barbosa, A.L. Teixeira, L.C. de Souza, P. Caramelli, Ischemic cerebrovascular burden evaluated by magnetic resonance imaging in an elderly Brazilian community: The Pieta study, eNeurologicalSci 5 (2016) 30-34. Genetics 77 Gaucher and Parkinsonism: Description of a Canadian Cohort Priti Gros (Toronto, ON, Canada), Graeme Nimmo (Toronto, ON, Canada), Dominick Amato (Toronto, ON, Canada), Lorraine Kalia (Toronto, ON, Canada) Objective: To describe the parkinsonian features of a Canadian cohort of type 1 Gaucher disease (GD) patients. Background: GD is a systemic disorder associated with mutations in the glucocerebrosidase (GBA) gene. GBA mutations are the most common genetic risk factors for Parkinson’s disease (PD). A broad spectrum of parkinsonian features can be observed among GD patients (1). Methods: We performed a cross-sectional cohort study of 48 patients with GD. GBA mutations were identiﬁed by DNA sequencing. Information regarding family history, hyposmia and sleep was collected. The Movement Disorder Society Uniﬁed Parkinson Disease Rating Scale (MDS-UPDRS) part I, II and III, as well as the Montreal Cognitive Assessment (MoCA) version 7.1 were performed. Results: Our cohort mean age (+-stdev, standard deviation) was 50.6 (+-15.6) y, 75% were female. The most common GBA mutations were N370S (75%) and L444P (22.3%), 25% were homozygous. Ethnicity of our population was diverse with up to 16 different ethnicities. The proportion of patients of Ashkenazi Jewish descent was 16.7%. A family history of PD was present in 37.5% of the cases. Hyposmia was reported in 18.8%, and features suggestive of REM sleep behavior disorder were reported in 14.6%. 3 patients (6.2%) were diagnosed with PD, 2 out of 3 had the L444P mutation. Mean (+-stdev) scores for each MDSUPDRS part are as follows: part I 9.7 (+-6.5); part II 3.4 (+-5.9); part III 7.7 (+-12.8). For part I, 79.1% had a score above 5, and for part III, 31.2% had a score above 5. The mean (+-stdev) score on the MoCA was 26.2 (+-5.0). The most affected cognitive domain was delayed recall followed by language. Executive function was relatively preserved. Conclusions: This is the ﬁrst description of the spectrum of parkinsonian features in a Canadian GD cohort. Non-motor features including psychiatric, sleep and cognitive disturbances were prominent among our GD patients. A large proportion of GD patients also had mild motor features, although a minority fulﬁlled criteria for PD. We expect that prospective observation of this cohort will reveal whether the presence of these motor and non-motor features represents overlap between the symptoms of GD and PD or features of prodromal PD among GD patients. References: Goker-Alpan O, Lopez G, Vithayathil J, Davis J, Hallett M, Sidransky E. The spectrum of parkinsonian manifestations associated with glucocerebrosidase mutations. Arch Neurol. 2008;65 (10):1353-7. 78 Whole Exome Sequencing for Atypical or Combined Movement Disorders Maria Ruiz Yanzi (Buenos Aires, Argentina), Patricio Millar-Vernetti (Florida, Argentina), Malco Rossi (Buenos Aires, Argentina), Marcelo Merello (Buenos Aires, Argentina) MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Objective: To report our experience with Whole Exome Sequencing (WES) for atypical phenotypes or combined movement disorders in a tertiary Movement Disorders Clinic. Background: Technologies for sequencing DNA are improving constantly, and they allow the diagnosis of rare diseases, detection of new pathogenic genetic variants, and the association of unknown clinical manifestations to already known diseases. Next-generation sequencing enables massive and parallel sequencing of multiple fragments of DNA, allowing sequencing of an individual’s entire genome, exome, or of multiple speciﬁc genes. Whole Exome Sequencing is convenient in many cases, because over 85% of disease-causing mutations are found in exons, and it also permits detecting previously unknown variants. Methods: A retrospective study of patients evaluated with WES was conducted between July 2015 and July 2019. Patients over 18 years old with movement disorders and combined or atypical phenotypes were included, such as parkinsonism with atypical features; ataxias where acquired causes and more frequent forms of trinucleotide expansions were excluded; combined dystonias; hereditary spastic paraplegia; or the combination of multiple phenotypes. Fisher’s exact test was used to compare categorical variables and to assess the diagnostic yield of pure or combined phenotypes. Results: Fifty-four patients (4820 years, range 19 to 93 years; 28 males) underwent WES. The average latency since onset of symptoms to the genetic test was 15 years (range 1 to 72 years). In 29 (54%) of the tests, a positive genetic ﬁnding was found, being 14 (26%) pathogenic variants and 15 (28%) variants of uncertain signiﬁcance. The number of pathogenic variants obtained according to the phenotype were 8/19 (42%) for ataxia, 5/17 (29%) for parkinsonism; 3/3 (100%) for tremor, 2/7 (29%) for hereditary spastic paraplegia, 6/21 (29%) for dystonia; and no pathogenic variants were found in two patients with chorea. Pathogenic variants were more frequently found in dystonias combined with other phenotypes in comparison to pure dystonias (p=0.031). No signiﬁcant differences were found in other phenotypes for either pure or combined forms. Etiological diagnosis enabled starting or modifying evidence-based treatment in 10 cases, representing 71% of cases in which a pathogenic mutation was found in WES, and 18.5% of cases in which the study was performed. Conclusions: In our cohort of patients with atypical or combined phenotypes, an etiological diagnosis was obtained in 26% of the cases, similar to what is described in the literature. In approximately 20% of cases, knowing the etiology of the disease allowed starting evidencebased treatment. WES is an effective test that allows obtaining an aetiological diagnosis in patients with atypical or combined movement disorders and should be considered for use once ﬁrst-line diagnostic tests were performed. References: 1. Key Principles and Clinical Applications of “NextGeneration” DNA Sequencing. Jason M. Rizzo and Michael J. Buck. Cancer Prev Res 2012.2. Improved diagnostic yield compared with targeted gene sequencing panels suggests a role for whole-genome sequencing as a ﬁrst-tier genetic test. Anath C Lionel PhD, Gregory Costain MD, PhD, Christian R Marshall PhD, et al. Genetics in Medicine volume 20, pages 435–443 (2018). 79 Northwestern University Feinberg School of Medicine Parkinson’s Disease and Movement Disorders Center Biorepository: Bringing the Clinic and Lab Together Alejandro Hernandez (Chicago, IL, United States), Robert Modiest (Chicago, United States), Lena Burbulla (Tuebingen, Baden-Wuerttemberg, Germany), Neil Shetty (Chicago, IL, United States), Heidi Friedeck (Chicago, United States), Danny Bega (Chicago, IL, United States), Puneet Opal (Chicago, IL, United States), Roneil Malkani (Chicago, IL, United States), Joanna Blackburn (Chicago, IL, United States), Carolyn Taylor (Chicago, IL, United States), Lisa Kinsley (Chicago, United States), Niccolo Mencacci (Chicago, IL, United States), Steven Lubbe (Chicago, United States), Dimitri Krainc (Chicago, IL, United States), Tatyana Simuni (Chicago, IL, United States) Objective: To establish a biobank of DNA and tissue samples from a population of movement disorder patients, their family members and healthy controls recruited from the Chicago area, where each biospecimen is directly linked to detailed clinical information, medical history and genetic information. Background: A large population of patients with movement disorders and their families are regularly seen in a neurology clinic. This provides an opportunity to gather valuable biological and detailed clinical data per participant. While there is a growing number of large scale DNA databanks, there are still few cohorts that have clinical phenotype information. Methods: Data collection from all consenting participants include at minimum: a DNA sample, family/social history information, MoCA and if they are an affected patient, electronic medical record data containing relevant disease-speciﬁc clinician administered scales. Whole blood or saliva samples are taken for DNA extraction, with plasma and CSF also taken if possible. Clinically relevant results of the genetic testing can be returned to participants. Skin biopsies for IPSCs etc. are also collected from consenting participants. All biospecimens accompanied by deidentiﬁed clinical data are available to external researchers through a structured application process. Results: The Northwestern University Feinberg School of Medicine Parkinson’s disease and Movement Disorders Center Biorepository was launched in 2014. To date 781 participants have been enrolled comprised of: 180 controls (109 familial, 71 non-familial), 457 PD and 145 other movement disorders. DNA is in storage for all participants, in addition 93 skin biopsies and 22 CSF samples have been taken. A majority of the PD samples are currently being screened for known PD mutations, and are also being genotyped on the NeuroChip variant platform. Conclusions: The dataset will be used by investigators to conduct longitudinal observational studies on phenotypic genotypic correlations. The collection of social/environmental, familial, and clinical data coupled with biological samples allows for a wide range of research applications. Growth of the database/biobank is necessary with a goal of collecting DNA data on all consenting clinic patients and at least 500 controls. 80 Novel LRRK2 Variants Contributing to Parkinson’s Disease in Hispanic Patients Karen Nuytemans (Miami, FL, United States), Farid Rajabli (Miami, FL, United States), William Scott (Miami, FL, United States), Corneliu Luca (Miami, FL, United States), Carlos Singer (Fort Lauderdale, FL, United States), Jeffery Vance (Miami, FL, United States) Objective: Assess frequency of known and novel LRRK2 and GBA variants in Caribbean Hispanics. Background: Little information is known about PD genetics in Hispanic patients, as most known variants have been identiﬁed in white, non-Hispanic or Asian patients. Recently, Dr. Mata and colleagues reported on Hispanic-speciﬁc PD variants in known PD gene, GBA, in the LARGE-PD cohort (1,2). Methods: We analyzed DNA of 83 Hispanic PD patients of which 75% identiﬁed as Caribbean (Cuba, PR or DR). We performed Global Screen Assay genotyping to assess global and local ancestry as well as Sanger sequencing on all exons of LRRK2 and GBA with known causal variants (previously identiﬁed in white, non-Hispanic patients) and exons harboring newly reported Hispanic-speciﬁc variants. For those patients carrying a variant, local ancestry was determined using RFMix (3) to assess contribution from European, West African or Amerindian ancestry to disease in these patients, and frequency in Hispanic controls available through the Alzheimer Disease Sequencing Project (ADSP; age-atexam>65y) was assessed. Results: We identiﬁed well-known variants LRRK2 p.G2019S and GBA p.N409S and p.A495P in ﬁve, three and two patients respectively, all on a European local ancestry. We did not observe LRRK2 p. R1441C/G, p.Q1111H or GBA p.N370S, p.L444P and p.K198E. Interestingly, we observed two novel variants in LRRK2; p.P1480L and p. D734N. Neither variant is observed in 765 Hispanic ADSP controls. Local ancestry analyses provide evidence for these variants to be present on the Amerindian or African background respectively. p.D734N had been previously identiﬁed in two individuals in the gnomAD database of the ‘Latino’ or ‘other’ group supporting non-European ancestry. Conclusions: Genetic analyses of PD patients in underrepresented population groups is necessary as genetic information from research in MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S41 ABSTRACTS white, non-Hispanics is not generalizable across race/ethnicity. Our and Dr. Mata’s results suggests that Hispanic-speciﬁc variants contributing to PD in this diverse population exists. Further analyses supporting the pathogenic nature of these variants in known PD genes is needed as well as analyses of non-previously identiﬁed candidate genes. The identiﬁcation of PD variants across race/ethnicity will help diagnoses and understanding of biological mechanism in all patients reducing the bias of research results and subsequent future therapies towards white, non-Hispanics; ultimately preventing further health disparities. References: (1) Cornejo-Olivas M. et al. NPJ Parkinsons Dis. 2017 Jun 2;3:19.(2) Velez-Pardo C. et al. Parkinsonism Relat Disord. 2019 Jun;63:204-208.(3) Maples BK. et al. Am J Hum Genet. 2013 Aug 8;93 (2):278-88. 81 Impact of Offering Genetic Testing and Counseling to People with Parkinson’s Disease in a Clinical Setting Anna Naito (Miami, FL, United States), James Beck (New York, NY, United States), Anne Hall (, United States), Karen Marder (Bronx, NY, United States), Martha Nance (Golden Valley, MN, United States), Michael Schwarzschild (Boston, MA, United States), Tatyana Simuni (Chicago, IL, United States), Anne Marie Wills (Boston, MA, United States), Roy Alcalay (New York, NY, United States) Objective: Facilitate access to Clinical Laboratory Improvement Amendments (CLIA)-approved genetic testing and genetic counseling to people with PD (PWP) and their clinicians. Background: Most PWP as well as their clinicians do not know if they carry genetic mutations linked to PD. The PD GENEration (PD GENE) study will offer CLIA-certiﬁed genetic testing of seven PDrelated genes along with genetic counseling to people with PD. Methods: The study will target enrollment of up to 15,000 participants across 50 sites in the US. Participants will undergo clinical assessments to characterize disease status and severity. DNA will be sequenced for GBA1, LRRK2, PRKN, PARK 7/DJ-1, PINK-1, SNCA, and VPS35, through a CLIA-certiﬁed genetic testing laboratory. In a pilot study, a subset of participants will be randomized to receive genetic test results from their movement disorders neurologist or a centralized genetic counselor. Follow-up at 3 and 12 months post-baseline visit will assess satisfaction and impact among participants and clinicians. The primary endpoint is the number of participants that receive genetic testing and are ready to enroll in gene-speciﬁc clinical trials. Results: Recruitment started in September 2019 at six pilot sites in the US accredited as a Parkinson’s Foundation Center of Excellence (COE) and Parkinson Study Group (PSG) site (NCT# 04057794). PD GENE is a ﬁve-year, multi-center study that will expand to ﬁfty COE and PSG study sites in 2020-21. Knowledge, impact and satisfaction surveys administered to the participant and clinician disclosing genetic testing results will permit assessment of the impact of genetic data on clinical care for PWP and may accelerate referral to clinical research. In the pilot study, secondary objectives include evaluating the impact and feasibility of returning genetic test results through clinician vs. genetic counselor. Conclusions: The PD GENE study will provide a large-scale patient registry that links clinical phenotype with genetic status for up to 15,000 PWP in the US. The study also aims to improve communication of genetic testing results and empower those living with PD by providing further knowledge of their disease through the administration of pre- and post-genetic test counseling. This abstract was presented at the 2019 International Congress of Parkinson’s Disease and Movement Disorders in September 2019. 82 Mitochondrial Membrane Protein-Associated Neurodegeneration (MPAN) Caused by a Heterozygous C19orf12 Mutation Laura Pesantez Pacheco (Houston, TX, United States), Shivika Chandra (Houston, TX, United States) Objective: We report a patient with MPAN caused by a novel C19orf12 heterozygous mutation. Background: Neurodegeneration with brain iron accumulation (NBIA) is a group of neurodegenerative disorders with a speciﬁc hallmark S42 of brain iron accumulation. MPAN is a form of NBIA caused by C19orf12 mutations with an autosomal recessive inheritance. Methods: The patient is a 27-year-old right-handed female from Honduras with unremarkable family and past medical history who was born from non-consanguineous parents. At age 6, she started falling, had difﬁculties with ﬁne motor skills, vision problems and complained of pain, numbness and tingling in legs. By age 10, she developed learning disabilities. Due to progressive gait instability, the patient became wheelchair-bound at age 19 despite physical therapy and Baclofen treatment. By age 21, she had dysarthria, dysphagia, insomnia, paranoia and visual hallucinations. At age 23, levodopa was prescribed with a mild improvement in motor symptoms. At age 27, the patient is non-verbal and wheelchair bound requiring assistance with all ADLs. Results: Neurological exam shows orofacial dystonia, lower extremity weakness, bradykinesia, generalized spastic tone, increased patellar reﬂexes and spastic gait; she had peripheral neuropathy, dysmetria, adiadochokinesia but no ataxia. Ophthalmological evaluation revealed bilateral optic atrophy. EDX studies reported distal axonal polymyopathy. MRI revealed hypodensity on T2 and GRE imaging in the lentiform nuclei. WES identiﬁed a novel single frameshift de novo mutation in C19orf12 (c.315dupC). This variant has not been yet reported in individuals with a C19orf12-related disease and was classiﬁed as a Variant of Uncertain Signiﬁcance. Conclusions: Our patient’s presentation and MRI is consistent with MPAN. Although it is thought that MPAN is an autosomal recessive disease, new evidence suggests that heterozygous de novo pathogenic sequence variants can cause MPAN. The mutation in this woman leads to premature stop codon. Gregory et al. proposed that truncated C19orf12 proteins, impair function of the normal protein produced from the non-mutated allele via a dominant negative mechanism and cause loss of function. This case highlights the worldwide distribution and genotypic heterogeneity of this condition. References: [1] Gregory A, Hartig M, Prokisch H, et al. Mitochondrial Membrane Protein-Associated Neurodegeneration. 2014 Feb 27. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews®. Seattle (WA): University of Washington, Seattle; 1993-2019. [2] Hogarth P, Gregory A, Kruer MC, et al. New NBIA subtype: genetic, clinical, pathologic, and radiographic features of MPAN. Neurology. 2013;80(3):268–275. [3] Schneider SA, Dusek P, Hardy J, Westenberger A, Jankovic J, Bhatia KP. Genetics and Pathophysiology of Neurodegeneration with Brain Iron Accumulation (NBIA). Curr Neuropharmacol. 2013;11(1):59–79. [4] Gagliardi M, Annesi G, Lesca G, Broussolle E, Grazia I, Vaiti V, Gambardella A, Quattrone A. C19orf12 gene mutations in patients with neurodegeneration with brain iron accumulation. Parkinsonism & Related Disorders, 2015;21(7): 813-816. [5] Nobile S, Thulasirajah S, Venkateswaran S. A Novel c19orf12 Mutation in Mitochondrial Membrane Protein-Associated Neurodegeneration. Canadian Journal of Neurological Sciences. 2019;46(5), 610-614. [6] Dušek P, Školoudík D, Roth J, Dušek P. (2018) Mitochondrial membrane protein-associated neurodegeneration: a case report and MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS literature review, Neurocase, 24:3, 161-165. [7] Sparber P, Marakhonov A, Filatova A et al. Novel case of neurodegeneration with brain iron accumulation 4 (NBIA4) caused by a pathogenic variant affecting splicing Neurogenetics 2018;19:257. [8] Al Macki N, Al Rashdi I. A Novel Deletion Mutation of Exon 2 of the C19orf12 Gene in an Omani Family with Mitochondrial Membrane Protein-Associated Neurodegeneration (MPAN). Oman Med J. 2017;32(1):66–68. [9] Selikhova M, Fedotova E, Wiethoff S, Schottlaender LV, Klyushnikov S, Illarioshkin SN, Houlden H. A 30-year history of MPAN case from Russia. Clinical Neurology and Neurosurgery 2017;159: 111-113. [10] Gregory A, Lotia M, Jeong SY, et al. Autosomal dominant mitochondrial membrane protein-associated neurodegeneration (MPAN). Mol Genet Genomic Med. 2019;7(7):e00736. 83 A Rare Case of Heterozygous Aceruloplasminemia Presenting with Tremors and Abducens Nerve Palsy Neeharika Thottempudi (Galveston, TX, United States), Laura Wu (Galveston, TX, United States) Objective: To present a case with tremors and left abducens nerve palsy secondary to heterozygous mutation in the ceruloplasmin gene. Background: Aceruloplasminemia is a rare, autosomal recessive disorder characterized by iron accumulation in the brain and viscera due to mutations in the ceruloplasmin (CP) gene. Heterozygous mutations in CP gene have been thought to be asymptomatic. Methods: Case report. Results: A 61-year-old Caucasian male presented to neurology clinic with acute onset of diplopia. He has a longstanding history of bilateral hand tremors and was previously diagnosed with Wilson’s disease due to low ceruloplasmin levels. He has no family history of Wilson’s disease or tremors. Neurological exam showed left abducens nerve palsy, postural/ action tremors of both arms and postural tremors of both legs. No Kayser-Fleischer rings were noted. Serum ceruloplasmin levels were low at 13 (reference range: 25 – 63 mg/dl). Serum copper level was low but 24-hour urine copper excretion was not elevated (12.0 ug/day, reference range: 3.0-45.0 ug/day). Serum iron and ferritin levels were normal. Acetylcholine receptor antibody and anti-MuSK antibody were negative. Liver function panel was normal. MRI Brain was unremarkable. Genetic test for ATP7A and ATP7B genes were negative. Low Leipzig scores made the diagnosis of Wilson’s disease unlikely. A heterozygous pathogenic variant, c.2066delC (p.Pro689fs), was identiﬁed in CP gene resulting in the diagnosis of symptomatic heterozygous aceruloplasminemia. His diplopia resolved spontaneously after four months. Conclusions: Most common neurological presentations in aceruloplasminemia are cognitive impairment, involuntary movements and cerebellar ataxia. Heterozygous carries of mutation in CP gene have been thought to be asymptomatic but a few cases have been reported showing neurological deﬁcits such as tremors and ataxia. The case we presented here has slowly progressive postural and action tremors of all limbs since his early 20s. No other etiology found to explain his left abducens nerve palsy. Low ceruloplasmin levels can be found in Wilson’s disease, Menkes disease and aceruloplasminemia. Genetic test for CP gene mutation should be considered in such cases especially when patients present with neurological deﬁcits. Health Professional (Non-Physician) Focus 84 Withdrawn by Author 85 The Inﬂuence of Physical Activities on Cognition in Small Groups of Parkinson’s Disease Patients Rachel Guimaraes (Campinas, Brazil), Marcelo Pereira (Piracicaba, SP, Brazil), Luiza Piovesana (Campinas, Brazil), Paula Azevedo (Campinas, Brazil), Luciana Ramalho (Campinas, Brazil), Lilian Gobbi (Rio Claro, Brazil), Fernando Cendes (Campinas, SP, Brazil) Objective: To assess the relation between cognitive performance and physical exercise in Parkinson’s disease patients. Background: Cognitive deﬁcit is a common non-motor symptom in PD, responsible for a huge impact on the quality of life. It‘s known that physical exercises improve cognitive function (1). The ProParki project includes and provides physical activities, aiming to improve quality of life and soften symptoms. This study aim seeks to expose the impact of a physical exercise program in a small group of PD patients when compared to sedentary ones. It‘s can show how efﬁciency is the encouragement of the formation of physical exercises programs for PD patients as an important non-pharmacological therapeutic option for a cognitive deﬁcit. Methods: This was a cross-sectional study. Twenty patients were included and divided into two groups: 10 patients who practice physical activities in the ProPark project and another group with 10 sedentary PD patients from the University of Campinas Clinical Hospital. The ProParki activities were based on muscle-building, balance training and socializing, three times a week and was held in S~ao Paulo State University - UNESP. All patients underwent a SCOPA-COG scale for cognitive function analysis. A GLM was applied to compare the two groups, where SCOPA scores were included as the independent variable, and time of levodopa use, time of disease, age, and education were included as covariates. Results: The ProParki patients had higher SCOPA-COG scores than sedentary individuals. The sub-item memory, was signiﬁcant different between groups. This shows a good impact on PD patient‘s memory, even in a small group. Conclusions: Physical activity might be an important nonpharmacological therapeutic tool to improve non-motor symptoms, like cognitive function, in PD. This study exposed that even in a small groups of patients, the beneﬁts from physical activities were noticed. Improving the quality of life of patients with PD. 86 Expiratory Strength Assessment Using PEAK FLOW in Parkinson’s Disease Rachel Guimaraes (Campinas, Brazil), Mirian Diogo (Campinas, Brazil), Maria Fernanda Sattolo (Campinas, Brazil), Lorena Durans (Campinas, Brazil), Lidiane Paiva (Campinas, Brazil), Fernando Cendes (Campinas, SP, Brazil) Objective: This study aimed to evaluate the effectiveness of cough in Parkinson’s disease (PD) patients from the Parkinson Ativo group at UNICAMP. Background: PD the second most prevalent neurodegenerative disease worldwide, and is deﬁned as a movement disorder, caused by basal ganglia dysfunction, involving the death of dopamine-producing neurons in the substantia nigra and projections to striatum 1, 2. Although the cardinal symptoms are motor, respiratory dysfunction is a common feature, and is a common cause of death in these patients. (art5) 3. Previous studies have shown that PD patients exhibit multiple respiratory changes, such as, reduced ability to perform a rapid and coordinated airway muscles contraction during exertion, chest wall rigidity and cough airﬂow reduction 4. Mucus removal is dependent on the magnitude of the peak ﬂow generated during cough5. Peak cough ﬂow (PFT) is the most reproducible way to measure cough strength, and is a valid tool for assessing and estimating glottic function and the risk of pulmonary complications in patients with neuromuscular disease5. There are no standard values for peak ﬂow measurements, but it is known that values up tp 160 l/min are correlated with extubation failure and indicative of bronchospasm6. Methods: We included patients from the Parkinson Ativo group, from UNICAMP, previously diagnosed with PD according to the London brain bank criteria, age above 18 years, with the capacity to understand verbal commands, and no other neurologic disease. Twelve PD patients. The cough evaluation was performed through the Peak Cough Flow and the patient was asked to perform up to three attempts. Best value was recorded. Results: Thirteen patients were included, (mean age 64.84 + 13.99 years), eight male. We divided patients into mild (n=5), moderate (n=5) and severe (n=3) PD according to the modiﬁed H&Y scale. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S43 ABSTRACTS There was a statistically signiﬁcant difference between groups as determined by one-way ANOVA (F=15.98, p=0.001). A Tukey post hoc test revealed that the peak ﬂow from mild PD (390 + 83.06 l/min) patients was signiﬁcantly different from the moderate (254 + 32.09 l/min, p=0.01) and severe (156.67 + 35.11 l/min, p= 0.01) group. There was no statistically signiﬁcant difference between the moderate and severe groups (p=0.105) . Conclusions: Mild PD patients aren’t at risk for pulmonary complications, and this phase, might be the therapeutic target for respiratory exercises, in order to prevent future aggravations. References: [1] Juste et al, Speech ﬂuency proﬁle on different tasks for individuals with Parkinson’s disease, 2017; [2] Ayres et al, Noninstrumental clinical evaluation for oropharyngeal dysphagia in Parkinson’s disease: systematic review, 2016; [3] Martins et al, Hospital Mortality in Parkinson’s Disease: Retrospective Analysis in a Portuguese Tertiary Centre, 2016; [4] Darling-White et al, The impact of expiratory muscle strenght trainig on speech breathing in individuals with Parkinson’s Disease: A preliminary study, 2017; [5] Freitas et al, Clinical application of peak cough ﬂow: a literature review, 2013; [6] Bach et al, Expiratory Flow Maneuvers in Patients with Neuromuscular Diseases, 2006. 87 The Effect a Six Months Physical Activity Program in a Small Group of Parkinson’s Disease Patients Maria Fernanda Sattolo (Campinas, Brazil), Lorena Durans (Campinas, Brazil), Lidiane Paiva (Campinas, Brazil), Mirian Diogo (Campinas, Brazil), Fernando Cendes (Campinas, SP, Brazil), Rachel Guimaraes (Campinas, Brazil) Objective: This study aimed to evaluate the progression of motor symptoms in Parkinson’s disease (PD) patients who practiced controlled physical activity, designed for people with Parkinson’s (PWP), for six months. Background: PD is one of the most common disorders in the world. It is a progressive neurodegenerative disease. The progression of symptoms may lead to disability, impairing daily activities 2. Physical activity may inﬂuence disease onset, severity and progression.3 There is increasing scientiﬁc evidence on the effectiveness of physical therapy and physical activity as adjunctive treatments for dopaminergic replacement focusing on controlling motor and non-motor PD symptoms, and, consequently, improving quality of life, regardless the stage of the disease Methods: Fourteen patients diagnosed with PD, according to the London brain bank criteria, who could walk independently or using assistive devices were included. All patients belonged to the Parkinson Ativo group, which consists of speciﬁc exercises for PWP. Activities were performed twice a week, with one hour duration. The UPDRS, Time UP and Go (TUG) and H&Y scales were applied at baseline and after 6 months. During the six months, different domains were trained, such as mobility, gait, balance, conditioning and motor coordination. Results: 11 patients (mean age 62,5 years, + 15.52) were assessed at baseline and after 180 days. Patients who attended to less than 10 sessions during the 180 days were excluded (n=3). The mean number of sessions was 20.37 (min 13, max 29). Mean UPDRS at baseline was 35.25 + 10.79. The mean UPDRS-III score at baseline was 16.85 + 10.23, and at 180 days were 13.62 + 7.04. Decrease of UPDRS part III (motor) score of greater than or equal to 5 points is considered clinically signiﬁcant after six months 1. Although there was no signiﬁcant difference between the two assessments, six of 11 patients demonstrated clinically signiﬁcant decrease at six months. Schulman et al.28 reported that a difference of 2.5 in the UPDRS motor score, represents a minimal clinically important difference (CID), with 5.2 for moderate and 10.8 for large CID. At 180, only 2 patients exhibited an increase in UPDRS motor score of more than 2.5, while eight patients exhibited a decrease in UPDRS motor score of more than 2.5.Five out of 8 patients had better performance at the TUG test on the second assessment, after 180 days, and 3 patients took more time to complete the test, however, these differences were not statistically signiﬁcant. Conclusions: Despite not being statistically signiﬁcant, our results suggest that physical activity is a valuable tool to improve patient’s motor limitations. It should always be considered as a treatment for PWP. S44 References: Schrag, A, Sampaio, C, Counsell, N Minimal clinically important change on the uniﬁed Parkinson’s disease rating scale. Mov Disord 2006; 21: 1200–1207. Huntington’s Disease 21 Withdrawn by Author 22 Gender Inﬂuences on Anosognosia Severity in Huntington’s Disease Karina Massruha (Sao Paulo, Brazil) Objective: To investigate anosognosia clinical correlations in Huntington’sdisease (HD). Background: Anosognosia is present when a patient’s perception of obvious disease manifestations differs from that of objective observers. Anosognosia often accompanies Huntington disease, and it is predictive of poor independence and high functional incapacity. Methods: We evaluate unawareness in manifested HD patients through the Patient Competency Rating Scale. Patients had a full neuropsychological and psychiatric evaluation. We seek for correlations with CAG repetitions, CAP score, paternal inheritance, sex, age at symptoms manifestations, HD duration, clinical motor subtypes, mood and behavioral symptoms, and cognitive decline severity. Results: Thirty-ﬁve patients were evaluated (60% female), mean age was 40.1 years (SD 12.7), and mean HD duration was 5.8 years (SD 5.5). Mean number of CAG expansions was 47.2 (SD 9.9). Paternal inheritance was present in 51.4%. Depressive symptoms were present in 56.3%, and anxiety in 53.6%. Anosognosia severity correlated positively with age (Spearman-r 0.43, p<0.05), male gender (T-test; p< 0.01), and UHDRS maximal chorea score (p=0.03). Negative correlations were observed with verbal ﬂuency (Spearman-r -0.44, p=0.021), immediate memory tests (Spearman-r -0.40, p=0.04), and quality of life SF-36 score on mental function (Spearman-r -0.42). Men disclosed more severe anosognosia than women on total score discrepancy and on the following subdomains: attention (p=0.031), ﬂexibility (p<0.001), monitoring (p=0.012), and memory issues (p=0.016). Multivariate linear regression analysis evidenced male gender as the principal predictive factor for anosognosia severity. Conclusions: Unawareness in HD requires further speciﬁc studies to better deﬁne the associations with the disease features at different disease stages. We observed a strong association with male gender and poor quality of life. References: 1. McGlynn S., Schacter D. Unawareness of deﬁcits in neuropsychological syndromes. J Clin Exp Neuropsychol. 1989;11:143–205.2. McCusker EA. Unawareness of motor phenoconversion in Huntington disease. Neurology. 2013;81:1141–7.3. Sitek EJ, Thompson JC, Craufurd D, Snowden JS. Unawareness of Deﬁcits in Huntington’s Disease. J Huntingtons Dis. 2014;3:125–35. 23 Phenotypic Variants of Huntington’s disease Patients in Santa Clara, Cuba Miriam Batule Dominguez (Santa Clara, Cuba), Luis R Crespo Rodríguez (Santa Clara, Cuba), Elién Tatiana Castro Ortiz (Santa Clara, Cuba) Objective: To describe phenotypic variants in Huntington’s disease. Background: Huntington’s disease is a neurodegenerative disease produced by a CAG expansion in the IT15 gene on the short arm of chromosome 4. Greater number of repeats are related with an earlier age of onset and severe course, nevertheless there are some variations even within the same family. Methods: A case serie of seven patients in Santa Clara, Cuba is phenotypically described according with their number of CAG repeats. Results: The ﬁrst ﬁve patients presented with variable number of CAG repeats and an age of onset between their twenties and their ﬁfties. One patient presented with an allele in an intermediate range, of 31, and MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS the other with 60 repeats, he had the earliest age of onset within that family and greater severity of the disease. The symptoms at diagnosis where motors in all of them, but with years they developed psychiatric manifestations and cognitive decline interfering with their daily activities. Two cases from another family had as an onset symptom psychiatric manifestations with repetitive pointless behavior carried out for long periods of time while other main activities where unattended, developing later the rest of symptoms. Epigenetic mechanisms have been implicated in the pathogenesis of the disease: DNA methylation, posttranslational modiﬁcations of histone proteins and some genetics modiﬁers on chromosome 15 and 18. Environmental factors may also inﬂuence this phenotypic variation, including age of onset and severity of disease. Conclusions: The study of this phenotypic variants with the genome analysis may help discerning personalized interventions. References: Duyao M, Ambrose C, Myers R, Novelletto A, Persichetti F, Frontali M, et al. Trinucleotide repeat length instability and age of onset in Huntington’s disease. Nature Genetics. 1993;4(4):387-92. Glajch, KE. Sadri-Vakili, G. Epigenetic Mechanisms Involved in Huntington’s Disease Pathogenesis. Journal of Huntington’s Disease. 2015;4(1):1-15. 24 “Hung-Up Knee Jerk Reﬂex” Description in a Population of Colombian Patients with Huntington’s Disease Juan Vargas Jaramillo (Bogota, Colombia) Objective: Describe the HUKJR in patients genetically diagnosed with HD from CLÍNICOS IPS, as well as its clinical correlation with the Uniﬁed Huntington Disease Classiﬁcation Scale (UHDRS). Background: Hung-Up Knee Jerk Reﬂex (HUKJR) is a clinical sign in the physical examination of Huntington’s Disease (HD)[1]. The reﬂex is associated with the involuntary contraction of the femoral quadriceps, caused by the percussion of the patellar tendon; in the HUKJR the extension of the leg remains elevated for a prolonged moment and relaxes sharply until reaching its original place. This sign is due to a choreic movement caused by delayed sensitivity[2]. The literature describes a prevalence of 80% in HD vs controls[3]. There are no studies in Latin America. Methods: Observational, cross-sectional and descriptive study in 10 patients with HD and 10 healthy controls, attending the IPS outpatient clinic. Results: It was observed that 9/10 patients (90%) with HD presented HUKJR, 7 Men: Age 49.4013.20 and UHDRS 35.2016.20; 3 Women: Age 55.819.21 and UHDRS 55.0924.32. We obtained 10 controls, none of which presented HUKJR, 6 Men: Age 34.4613.26, 4 Women: Age 34.239.97. Conclusions: It is important to consider HUKJR in the physical examination of HD. It could be a pathognomonic sign, it can occur in presymptomatic stages, it has been observed in advanced course of the disease, a decrease in the reﬂex probably by the progression towards Parkinsonism. It is necessary to conduct studies in a larger population and with choreas of other etiologies to conﬁrm or discard the before mentioned. References: 1] Obeso JA, Rothwell JC, Marsden CD. Hung-up tendon jerks in Huntington’s chorea—study of a little sign. Arch Neurobiol 1984; 47:151–6. [2] Hallet M. Physiology of basal ganglia disorders: an overview. Can. J. Neurol. Sci. 1993; 20:177-183. [3] Perez J, Diaz J, Pagonabarraga J, et al. Hung up knee jerk reﬂex in Huntington’s disease: A clinical and neurophysiological study. Mov Disord. 2017; 32-2. Myoclonus 27 Limb-Shaking Associated with Cortical Myoclonus as a Manifestation of a Glioblastoma Juan Vargas Jaramillo (Bogota, Colombia) Objective: Limb-shaking syndrome is uncommon, it involves involuntary movements of the limbs. Myoclonus is a rapid movement caused by the contraction of agonist and antagonistic muscles, the cortical subtype is the most frequent. We present a case that associates these two movements, there are no similar cases reported. Background: A 49-year-old woman was brought to the hospital for a clínica picture of numbness of the right lower limb, accompanied by involuntary movements referred to as "uncontrollable jumps." Upon examination we found mild paresis of the right lower limb, hypoaesthesia and “shaking” movements while sitting, they had some rhythmicity but were irregular, were rhythmic but irregular, the dorsal cube disappeared, there was also an involuntary, regular, fast, rhythmic and brief movement of proximal predominance and in the right iliac fossa. Electromyography showed involuntary, rhythmic and synchronous contractions in the vastus lateral lateralis and biceps femoris muscle, lasting up to 7 milli seconds, ﬁndings also seen with voluntary movements. The paraclinics had no metabolic abnormalities laboratories tests at admisión, including metabolic proﬁle were normal.The electroencephalographic record ruled out epileptiform discharges. These ﬁndings were compatible with the diagnosis of cortical myoclonus associated with limb-shaking. Cerebral angiotomography revealed a left frontal cortical hyperdense lesion, ruled out there were no fundings of vascular stenosis. Resonance MRI with contrast showed a left corticosubcortical lesion with contrast enhancement. The spectroscopy ﬁnding was compatible with high grade glioblastoma. It was movement disorder was managed with clonazepam and carbamazepine, achieving good control. Later she was taken to surgery with total resolution of symptoms. Conclusions: We present the ﬁrst case report of limb-shaking with cortical myoclonus, demonstrating compatible features and ruling out other causes. The response to anticonvulsants was excellent. Treating the cause is the main objective. 28 Focal Oromandibular Myoclonus Associated to Cochlear Stimulation Natalia Gonzalez Rojas (La Plata, Argentina), Gustavo Andres Da Prat de Magalhaes (Buenos Aires, Argentina), Martin Cesarini (La Plata, Argentina), Jose Luis Etcheverry (Caba, Argentina), Alfredo Palavecino (Buenos Aires, Argentina), Emilia Gatto (Buenos Aires, Argentina) Objective: To describe a patient that underwent CID and developed focal oromandibular myoclonus. Background: Facial nerve stimulation (FNS) has been reported as a complication of cochlear implant device (CID), with an estimated frequency between 0.9 to 14.9%. Although, hypoacusia secondary to otosclerosis is one of indication for CID, otosclerosis is among one of the major risk factors for abnormal FNS. FNS can appear immediately after CID or as a delayed complication. FNS can cause involuntary movements involving different facial innervated areas. Results: A 60-year-old female with a diagnosis of severe otosclerosis for more than 20 years underwent CID 3 years ago. No complications occurred after CID and improvement in hearing was achieved. She was referred by her CID technician for a 6-month history of paroxysmal involuntary movements involving the right oromandibular area. After the last CID setting, she noticed transient paresthesias near the right oromandibular area triggered by certain speciﬁc auditory stimulus. Neurological examination revealed a sudden, brief movement of the right orbicularis oris, that increases in frequency and intensity by speciﬁc auditory stimulus.Complementary studies, including routine laboratory testing and axial computed tomography (CT) of the brain were normal. All other potential causes were ruled out. A transient turnoff of the CID supressed involuntary facial movements. The diagnosis of focal myoclonus associated to FNS was done and the CID settings were modiﬁed with improvement of the movement. Conclusions: Although FNS after CID has been reported, movement disorders are among rare possible side effect. The present case illustrates about an uncommon cause of focal facial myoclonus to be included in the list of secondary acquired causes of focal myoclonus. References: 1) The Pathologic Basis of Facial Nerve Stimulation in Otosclerosis and Multi-Channel Cochlear Implantation. Mohammad Seyyedi. Otol Neurotol. 2013 December; 34(9):. doi:10.1097/MAO.0b013e3182979398.2) Facial nerve stimulation after cochlear implantation: our experience. S. Berrettini. ACTA oTorhinolAryngologiCA iTAliCA 2011;31:11-163) Characteristics and MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S45 ABSTRACTS pathogenesis of facial nerve stimulation after cochlear implant surgeries: A single center retrospective analysis from 1,151 patients. Kim, Y.R. PARK (Orcid ID : 0000-0002-6331-8556)4)Revision Cochlear Implantation for Facial Nerve Stimulation in Otosclerosis Marek Polak, PhD; S. Arif Ulubil, MD; Annelle V. Hodges, PhD; Thomas J. Balkany, MD5) Cushing et al.: Facial Nerve Stimulation in Children With Cochlear Implants. Laryngoscope 116: October 2006 Neurodegeneration 64 Prevalence of Neurodegenerative movement disorders in different clinical Practices of Hyderabad, Pakistan Mudassar Arain (Jamshoro, Pakistan) Objective: The main objective of current study was to determine the rate of prevalence of neurodegenerative movement disorders at different clinical practices in Hyderabad, Pakistan. Background: All the body movements are controlled by neurons of brain. Therefore, Neurodegenerative diseases are affect different brain parts progressive that may leads to the improper functioning of nerve cells that control the body movements. These neurodegenerative diseases include Alzheimer’s disease (AD), Parkinson‘s disease, Huntington diseases, multiple system atrophy, essential tremor, myoclonus and Hereditary Spastic Paraplegia. Methods: This is an observational and cross-sectional study. All the patients between 18-80 years of age were included for a period of six months. A total of 200 patients were enrolled who visited at 10 different locations of Hyderabad. The data includes the patient and disease information were recorded in the data sheet. Results: Majority of male patients (71%) were reported as compared with female (29%) during this study and the average age of patients are around 65 years. The most prevalent Neurodegenerative movement disorders were Parkinson’s disease (24%) followed by chorea (17%) and essential tremor (16%). Autonomic complications such as feeling anxious (65%), nocturia (38%) and Low mood and feeling sad(37%), whereas urinary urgency was stated by 29% of patients, memory problems (53%), and Feeling of light-headedness was observed by 52% of patients. The most common problem related to sleep are insomnia (34%).In Motor symptoms mostly muscle stiffness (62%) was reported in patients. Conclusions: Parkinson’s disease is more prevalent in our population. Autonomic complications anxious and Motor symptoms like muscle stiffness are more common inNeurodegenerative movement disorders. In future, these types of studies will be conducted to evaluate the relationship of various factors with Neurodegenerative disease and movement disorders. 65 The Effect of Cancer on Parkinson’s Disease Progression Abhimanyu Mahajan (Cincinnati, OH, United States), Martina Chirra (Cincinnati, OH, United States), Alok Dwivedi (El Paso, United States), Andrea Sturchio (Cincinnati, OH, United States), Elizabeth Keeling (Phoenix, AZ, United States), Luca Marsili (Cincinnati, OH, United States), Alberto Espay (Cincinnati, OH, United States) Objective: To evaluate the extent to which a cancer diagnosis may be associated with slower progression of Parkinson’s disease Background: Cancers and neurodegenerative diseases are biological opposites. Their coexistence implies an interplay between cellular proliferation and cellular degeneration processes. Methods: Retrospective case-control design using electronic medical records from 2013 to 2019. Setting: Movement disorders center at the University of Cincinnati.Participants: Consecutive patients diagnosed with Parkinson’s disease with (cases) and without cancer (controls) evaluated between January 1, 2013 and January 1, 2019. Cases were further divided according to the timing of cancer diagnosis relative to Parkinson’s onset (before and after Parkinson’s). Exposure: Histopathological diagnosis of cancer.Main Outcome and Measure: Time to postural instability, a disability milestone, deﬁned as achieving a Hoehn and Yahr staging of S46 3. All cases were classiﬁed as fast or slow progressors based on a median time to H&Y stage = 3 of 6 years. Results: A total of 125 cases and 125 controls were included. The two most common cancers among cases were skin cancer (68%) and prostate cancer (8%). Cases with cancer diagnosis before Parkinson’s onset had an older age at onset of Parkinson’s disease (80.5 vs 63.7 years, p = 0.0001), older mean age at postural instability (83.7 vs 74 years, p = 0.0005) but a shorter mean time to postural instability (3.2 vs 10.3 years, p = 0.0033). Cases who progressed to postural instability with a faster progression had an older age at onset of PD symptoms (74.4 vs 60.8 years, p<0.001), shorter duration of cancer symptoms at the last visit (2.3 vs 7.7 years, p = 0.002) and lower prevalence of depression. (26% vs 63%, p = 0.031) Conclusions: While the association between cancer and Parkinson’s disease has been evaluated and described before, our study uses milestones developed from granular clinical data from our movement disorders center to address this relationship. While such an inverse relationship between carcinogenesis and neurodegeneration has been proposed in a large population-based cohort of Alzheimer’s disease patients, ours is the ﬁrst study to assess the effect of cancer on disease progression in PD and report a potential protective effect. Cancer diagnosis may delay Parkinson’s onset but either accelerates progression or compounds its overall disability. References: 1.Devine MJ, Plun-Favreau H, Wood NW. Parkinson’s disease and cancer: two wars, one front. Nature reviews Cancer. 2011;11 (11):812-823.2.Ospina-Romero M, Abdiwahab E, Kobayashi L, et al. Rate of Memory Change Before and After Cancer Diagnosis. JAMA network open. 2019;2(6):e196160. 66 An Unusual Presentation of a Misidentiﬁcation Syndrome in a Patient with Lewy Body Dementia Yousaf Ajam (Galveston, TX, United States), Lei Lu (Galveston, TX, United States), Laura Wu (Galveston, TX, United States) Objective: To describe an unusual presentation of delusion in a patient with Lewy body dementia Background: Delusional misidentiﬁcation syndrome (DMS) is a delusional disorder which can be seen in Lewy body dementia (LBD). Capgras syndrome is the most frequent form. Delusion of reduplication of places is rarely reported in patients with LBD. Methods: Case report. Results: A 73-year-old Caucasian woman presented with a 6-year history of progressive cognitive decline and more recently visual hallucinations and gait disturbance with frequent falls in the past 2 years. Her cognitive functions ﬂuctuated throughout the day and from day to day. Patient also presented a behavioral disturbance that she believed she “live [s] in two places” which are identical including the arrangement of the furniture. Patient does not recognize her own apartment after being dropped off the ﬁrst time following an outing and denied that the apartment is hers. Only after driving around the block and returning to her own apartment again can she be convinced that it is her apartment. Neuropsychological testing showed impaired range on most of the cognitive domains including temporal orientation, drawing and copying of a clock face, motor perseveration, semantic ﬂuency and memory. Amnestic neurodegenerative disorder. MRI brain showed age appropriate generalized cortical atrophy. DatScan showed reduced uptake in the right putamen which made the diagnosis of Lewy body dementia more likely. Conclusions: Delusions are not as common as visual hallucination in LBD but can cause signiﬁcant burden in care-givers. Several studies have addressed Capgras syndrome (CS) in LBD but other misidentiﬁcation syndromes are scarcely studied. One large study focused in the prevalence of DMS in neurodegenerative disease enrolled 36 subjects of LBD. Isolated CS was diagnosed in 3 (8.3 %) but none of the individuals with LBD presented with delusions of the reduplication of places.1 Another study enrolled 100 LBD patients and 6 out of the 100 showed reduplication of places.2 DMS can also present in PD cases.3 The pathophysiology of DMS remains unclear. Our case suggested that further study is warranted for further evaluation of DMS in parkinsonism. References: 1. Harciarek M, Kertesz A. The Prevalence of Misidentiﬁcation Syndromes in Neurodegenerative Diseases. Alzheimer Dis Assoc Disord. 2008; 22 (2): 63-9. 2. Yasuhiro N, et al. Classiﬁcation of MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Psychotic Symptoms in Dementia with Lewy Bodies. Am J Geriatr Psychiatry. 2007 Nov;15(11):961-7. 3. Hermanovicz N. Delusional misidentiﬁcation in Parkinson’s disease: report of two cases and a review. Postgrad Med. 2018 Mar;130(2):280-283 67 Abnormal Contents of the Microtubule Associated Protein Tau Mediates Deﬁcits in Motor Coordination and Basal Ganglia Neuronal Dysfunction Maria-Elena Avale (Buenos Aires, Argentina), Ana Damianich (Caba, Argentina), Camilo Mininni (Buenos Aires, Argentina), Carolina Facal (Buenos Aires, Argentina), Delﬁna Loch (Buenos Aires, Argentina), Silvano Zanutto (Buenos Aires, Argentina), Juan Ferrario (Buenos Aires, Argentina) Objective: In this work we investigate motor phenotypes and neurochemical changes in the basal ganglia in a mouse model of tauopathy linked to abnormal tau splicing. We validate a potential therapeutic intervention to preclude disease progression based on RNA reprogramming. Background: The microtubule-associated protein TAU is crucially involved in neuronal functions such as microtubule dynamics and axonal transport. The alternative splicing of tau primary transcript produces isoforms with 3 or 4 microtubule binding repeats (3R and 4R), present in equal amounts in the normal adult human[1]. Tauopathies are a group of neurodegenerative diseases, with presence of insoluble tau aggregates and loss of tau function. Several tauopathies such as Frontotemporal dementia and Progressive Supranuclear Palsy (PSP) affect the basal ganglia thus leading PD symptoms. In those pathologies the endogenous 3R:4R tau balance is frequently altered, due to genetic or epigenetic mechanisms [2]. For translational studies, mouse models reﬂecting deﬁcits in tau splicing are essential to evaluate potential therapeutic interventions for those tauopathies. Here we investigated motor phenotypes and neurochemical changes in the basal ganglia of a mouse model of tauopathy linked to tau mis-splicing to assess potential therapeutic interventions. Methods: Transgenic mice bearing 3R:4R imbalance (Htau mice), and their wild type and Tau knock-out controls were tested in the open ﬁeld and rotarod to assess motor coordination. Anxiety behaviour and behavioural inhibition was measured in the elevated plus maze. Western blot and immunohistochemistry were performed to assess tau pathology in the basal ganglia. To modulate the 3R:4R tau ratio in htau mice a trans-splicing RNA reprogramming strategy was used as we previously reported [3][4]. Trans-splicing molecules were delivered by estreotaxic injection of lentiviral vectors at 3 months old. Phenotypic rescue was assessed 6 months after treatment. Firing rates of striatal neurons was assessed in living mice using electrophysiological recording with tetrodes. Results: Both TauKO and htau mice showed poor performance in the rotarod compared to WT mice, while spontaneous locomotion open ﬁeld and inverted grid tests were normal. Analysis of tau isoforms contents indicated that 3R >4R tau in the PFC and striatum of htau mice, although tau deposits were detected in the cortex but not in the striatum of htau mice. Modulation of tau isoforms imbalance by trans-splicing in the striata of young mice precluded motor deﬁcits in the htau model while other phenotypes such as cognitive impairment or behavioral inhibition were not rescued. In addition, striatal neuronal ﬁring which was restored to normal levels after trans-splicing modulation of tau isoforms imbalance. Conclusions: Either lack of tau function or tau isoforms imbalance (gain of toxic function) are detrimental for motor activity, however our results evidence that the (dys) functional consequences of tau 3R:4R imbalance would have a stronger impact in the development of motor impairments than mutations that induce the lack of function. In addition the results obtained using tau isoforms modulation in the htau model rise the potential use of RNA reprogramming to correct tau mis-splicing in human tauopathies. References: 1. Spillantini MG, Goedert M (2013) Tau pathology and neurodegeneration. Lancet Neurol 12:609–622. doi: 10.1016/ S1474-4422(13)70090-5 Neuroimaging and Neurophysiology 68 Dynamic Functional Connectivity Patterns in Parkinson’s Disease Patients with Depressive Symptomatology Maria Diez-Cirarda (Toronto, Canada), Iñigo Gabilondo (Barakaldo, Spain), Naroa Ibarretxe-Bilbao (Bilbao, Spain), Juan Carlos Gomez (Barakaldo, Spain), Jinhee Kim (Toronto, Canada), Olaia Lucas-Jiménez (Bilbao, Spain), Sang Soo Cho (Toronto, Canada), Rocio Del Pino (Bilbao, Spain), Javier Peña (Bilbao, Spain), Natalia Ojeda (Bilbao, Spain), Marian Acera (Bilbao, Spain), Alexander Mihaescu (Toronto, Canada), Mikaeel Valli (Toronto, Canada), Alberto Cabrera-Zubizarreta (Galdakao, Spain), María Angeles Gómez-Beldarrain (Bilbao, Spain), Antonio Strafella (Toronto, ON, Canada) Objective: The objective of the present study was to evaluate the brain dynamic functional connectivity (FC) patterns of Parkinson’s disease (PD) patients with depressive symptomatology. Background: Depression symptomatology is frequently observed in PD patients even in prodromal stages. Previous studies have shown relationships between static FC alterations and depressive symptoms in PD [1], but to date, no study has evaluated the temporal properties of connectivity in PD patients with depression. Methods: Fifty-seven PD patients and 63 healthy controls (HC) underwent a clinical, cognitive and depression assessment evaluated by Geriatric Depression Scale (cut-off score = 5) [2]. Resting-state functional MRI (rs-fMRI) and T1-weighted MRI were also acquired. Dynamic FC analyses were performed in GIFT toolbox. Dynamic FC analyses included connectivity differences in each dynamic state, and the dynamic FC indexes were also analyzed: fractional time window (i.e. total time spent in one dynamic state); mean dwell time (i.e. mean time spent in one dynamic state before switching to another state); and MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S47 ABSTRACTS state transitions (i.e. number of changes between states). MANCOVA analyses were performed to test differences between groups. Results: Thirty-four PD patients with depression, 23 PD patients without depressive symptoms and 63 HC were analyzed. Dynamic FC analyses revealed two dynamic connectivity States. State I was characterized by within-network coupling and State II showed stronger connectivity between-networks (Fig.1). PD patients with depression showed reduced fractional time window in State 1 compared to PD patients without depression and HC, while State II occurred at a higher rate (p<.05) (Fig. 2). In addition, PD patients with depression showed shorter dwell time in State 1, while dwell time in State 2 was increased compared to PD patients without depression and HC (p<.05). Also, the number of state transitions was increased in PD patients with depression compared to HC (p<.001). Conclusions: This study was the ﬁrst to analyze dynamic FC patterns in PD patients with and without depression symptomatology. Results suggest that presence of depressive symptoms in PD patients was related to alterations in the temporal properties of connectivity, showing a shorter dwell time pattern of the state with weaker connectivity while remained more time in the state with stronger connectivity. Instead, PD patients without depression showed no dynamic FC alterations. These ﬁndings may add relevant information about brain functional alterations in PD patients with depressive symptomatology and help in the design of symptom-speciﬁc treatments for PD patients. References: 1. Wen, M. C., Chan, L. L., Tan, L. C. S., & Tan, E. K. (2016). Depression, anxiety, and apathy in Parkinson’s disease: insights from neuroimaging studies. European journal of neurology, 23(6), 1001-1019. 2. Greenberg, Sherry A. “The geriatric depression scale (GDS).” Best Practices in Nursing Care to Older Adults 4.1 (2019): 1-2. 69 Hypomimia in Progressive Parkinson’s Disease: Neural Strutural Correlations with Abberations in Ponto-Cortical Connectivity Ganesh Elumalai (Kakinada, India), Panchanan Maiti (Memphis, TN, United States), Christina Vadiyala (Georgetown, Guyana), Geethanjali Vinodhanand (East Bank Demerera, Guyana), Prathiksha Singuru (Georgetown, Guyana) Objective: Parkinson’s disease (PD) is a movement disorder with classic motor signs consisting of bradykinesia, rigidity and tremor. Loss of asymmetrical facial expression and facial emotional response that is “Hemi hypomimia” comes as manifestation of bradykinesia. Background: Hemi hypomimia is rare sign in early stage of PD. The motor symptoms of PD are mostly due to progressive degeneration of dopaminergic neurons. Asymmetric motor symptoms are linked to long term disease progression. Methods: Diffusion ﬁbre tractography performed using Diffusion Tensor Imaging in 4 groups of 120 data sets each, obtained from different individuals. These groups include a control group and three PD affected S48 groups from different stages of severity. The ﬁbre tractography used in the analyzation of progressive degeneration of neural structural connectivity between the cortex (Brodmann area 4, and a part precentral gyrus) to facial motor nuclei (in Pons). Results: Disruption in the neural communication in the Pontocortical region assumed to be associated with Hypomimia. Our present study exhibits rigid deterioration of the tract in females than males. Approximately ninety percent of the tract is deteriorated in ﬁrst two years of PD in females. The results obtained are asymmetrical with unequal levels of deterioration on right and left hemispheric tract. Conclusions: The hypomimia, also called facial masking, is well marked symptoms in Parkinson’s disease (PD). But, till now, very little information known about how this motor impairment relates to neural structural correlations. The current study suggests that monitoring the neural connectivity between the cortex (Brodmann area 4, and a part precentral gyrus) to facial motor nuclei (in Pons) facilitating to monitor the progression of PD or a mode of staging on the basis of disease progression. References: [1] Sarah D. Gunnery., et al. The Relationship between the Experience of Hypomimia and Social Wellbeing in People with Parkinson’s Disease and their Care Partners. J Parkinsons Dis. 2016 Jun 3; 6(3): 625-630. [2] Ozekmekçi S., et al. Hemihypomimia, a rare persistent sign in Parkinson’s disease: follow up of 11 patients. J Neurol. 2007 Mar;254(3):347-50. 70 Diffusion Fiber Tractography Connectivity Analysis for Olfacotry Neural Networks Associated with Cognitive Impairements in Parkinson’s Disease Ganesh Elumalai (Kakinada, India), Panchanan Maiti (Memphis, TN, United States), Harshita Chatterjee (East Bank Demerera, Guyana), Geethanjali Vinodhanand (East Bank Demerera, Guyana) Objective: Disruption in the neural connections between orbitofrontal cortex (OFC) and olfactory cortex and analyze their consistency ranging from control group to Parkinson’s disease (PD) affected groups of different stages of progression. Background: The neural connections between OFC region with the olfactory cortex could potentially alter normal processing of stimuli causing changes in one’s response to them. This would give rise to olfaction associated cognitive dysfunction, which is often reported to precede motor symptoms seen in PD. Methods: Diffusion ﬁbre tractography performed using Diffusion Tensor Imaging in 4 groups of 40 data sets each, obtained from different individuals. These groups include a control group and three PD affected groups from different stages of severity. Results: Disruption of neural connections known to cause disturbances in cognitive, behavioural and emotional functions. The difference noted in ﬁbres of control group and PD affected group is statistically signiﬁcant with a p-value of 0.00001, the average number of ﬁbres noted in MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS a normal individual was 56 while that in a PD affected person was 16. The difference noted in ﬁbres among different stages of progression was also signiﬁcant with a p-value of 0.00001. Conclusions: The OFC plays an important role in drawing perceptions from sensory stimuli. The visual aspect of the same has been greatly explored, but olfactory aspect still remains a matter of curiosity. A study done on this topic states that the OFC plays a major role in olfactory perception, and the right OFC is relatively more dominant. Based on the results obtained we may suggest that olfactory stimuli dependent cognitive impairment in PD patients can be monitored and used to track the progression of the impairment and the disease References: [1] Stevenson RJ., Attuquayeﬁo T. Human olfactory consciousness and cognition: its unusual features may not result from unusual functions but from limited neocortical processing resources. Front. Psychol., 01 November 2013 | https://doi.org/10.3389/fpsyg.2013.00819.[2] Li W., et al. Right Orbitofrontal Cortex Mediates Conscious Olfactory Perception. Psychol Sci. 2010 Oct; 21(10): 1454–1463. 71 Temporal-Parietal Junction Neural Connectivity Analysis in Olfactory Spatial Attention Deﬁcit in the Progression of Parkinson’s Disease Ganesh Elumalai (Kakinada, India), Panchanan Maiti (Memphis, TN, United States), Harshita Chatterjee (East Bank Demerera, Guyana), Geethanjali Vinodhanand (East Bank Demerera, Guyana) Objective: Variations in the neural connections conscientious for facilitating spatial recognition of olfactory stimuli as observed in people diagnosed with Parkinson’s disease (PD) compared to those not affected by PD, and during the progression of the same. Background: Olfactory dysfunction is an early clinical marker before motor and cognitive declines in PD. The temporal-parietal junction (TPJ) contributes to spatial recognition, and lesions in this region or disruption associated with lack of spatial attention. Methods: The presents study employs the diffusion ﬁbre tractography using Diffusion Tensor Imaging. The neural connection between the olfactory cortex and TPJ have been studied in four different groups of people including a control group, and three groups of people diagnosed with PD from widely spaced stages of progression of the disease. The study has been conducted with MRI scans obtained from 40 different people for each group. Results: Olfactory dysfunctions are observed in approximately nighty percent of people affected by PD. A signiﬁcant difference in the number of ﬁbres connecting the two regions was noted between the control group and the groups affected with PD. A p-value of 0.037 was obtained. As the stages progressed, a decrease by 20 ﬁbres on an average was noted and a p-value of 0.0001 was obtained. Conclusions: Disruption in the neural communication in the TPJ region proved to be associated with lack of spatial attention to various stimuli including visual, auditory, and less popularly olfactory. Upholding the hypothesis, a signiﬁcant difference in the number of ﬁbres have been seen between the control and PD affected groups, and the further decline has been observed in the progressive stages of PD. This suggests that monitoring the neural connectivity between the olfactory cortex and TPJ or monitoring the neural connections facilitating one’s spatial attention to olfactory stimuli can provide for a way to monitor the progression of PD or a mode of staging on the basis of olfactory dysfunction progression. References: [1] Lamb MR., et al. Attention and interference in the processing of global and local information: Effects of unilateral temporalparietal junction lesions. Neuropsychologia. 1989; 27(4). 471-483. [2] Friedrich FJ., et al. Spatial attention deﬁcits in humans: a comparison of superior parietal and temporal-parietal junction lesions. Neuropsychology 1998; 12(2). 193-207. 72 A Rare Observation on the Primary Gustatory Cortex Neuralconnectivity Analysis in Parkinson’s Disease Progression Ganesh Elumalai (Kakinada, India), Panchanan Maiti (Memphis, TN, United States), Harshita Chatterjee (East Bank Demerera, Guyana), Geethanjali Vinodhanand (East Bank Demerera, Guyana) Objective: Dysgeusia, a least established non-motor symptom in PDs. The study analyze the consistency of neural connections of the primary gustatory cortex (PGC), ranging from control group to progressive stages of Parkinson’s disease (PD). Background: Various researchers failed to observe the Dysgeusia symptom in PD patients, but certain studies also state that taste acuity is seen to be enhanced in these patients. We aimed to identify the neural connections in PGC and regions associated in PDs. Methods: The present used diffusion ﬁbre tractography on structural Magnetic Resonance Imaging (MRI) scans, we observed the number, length and volume of ﬁbres connecting PGC and its associated regions. Comparisons were made among 4 groups, each group containing 40 MRI scans obtained from different individuals, one control group and three PD affected groups of spaced stages of progression. Results: PD patients experienced a more intense response to gustatory stimuli of low concentration as compared to control groups. A statistically signiﬁcant difference in ﬁbres was noted between control and PD affected groups, with a p-value of 0.0008, but difference noted in ﬁbres among different stages of progression was not signiﬁcant. In most PD affected MRIs the number of ﬁbres noted was as low as zero. From the results observed, we may derive that while there is a signiﬁcant decrease in the ﬁbres in PD affected group and we may conclude that PD does affect gustatory functions, there is a very little decline in ﬁbres from initial stages to advanced stages. Conclusions: Previous studies have presented that dysgeusia noted in PD patients is not related to the disease itself but is a result of olfactory loss which leads to decreased gustatory function and trigeminal functions. From the results observed, a statistically signiﬁcant difference in ﬁbres was noted between control and PD affected groups. Therefore, although we may use monitoring of gustatory neural connections as a form of preliminary diagnosis, it may not serve the purpose of progression monitoring. However, in order to conﬁrm the same, a similar study using functional MRI scans instead of structural MRI scans may provide better insight. References: [1] Kashihara K., et al. Frequency and characteristics of taste impairment in patients with Parkinson’s disease: results of a clinical interview. Intern Med. 2011;50(20):2311-5.[2] Goncalves CVF. Dysgeusia in Parkinson’s Disease: A Systemic Review. Faculdade de Medicina da Universidade de Coimbra. April 2016. 73 Decline in Cholinergic Basal Forebrain Volume in Early Parkinson’s Disease Matthew Barrett (Charlottesville, VA, United States), Justin Murphy (Charlottesville, VA, United States), Jeffrey Zhang (Charlottesville, VA, United States), Jamie Blair (Charlottesville, United States), Joseph Flanigan (Charlottesville, VA, United States), Scott Sperling (Charlottesville, VA, United States), James Patrie (Charlottesville, VA, United States), Thomas Druzgal (Charlottesville, VA, United States) Objective: To examine change in cholinergic basal forebrain volume in early Parkinson disease (PD) and determine which baseline clinical features predict this change. Background: Degeneration of the cholinergic basal forebrain is a feature of advancing PD and contributes to cognitive impairment and other neuropsychiatric symptoms. Methods: We analyzed data for 97 PD participants from the Parkinson’s Progression Markers Initiative with longitudinal MRI scans at baseline, 1 year, 2 years, and 4 years. Using probabilistic maps of the cholinergic basal forebrain, cholinergic nucleus 4 (Ch4) grey matter density (GMD) was calculated for each MRI. GMD measurements were modeled as a function of surveillance time by way of random coefﬁcient regression (RCR). Results: Mean baseline Ch4 GMD was 0.473 (95% CI: [0.463, 0.484]) and the mean change between baseline and 4 years was -0.025 (95% CI: [-0.031, -0.019]), or -5.21% (95% CI: [-6.67%, -3.88%]), which is equivalent to -1.30% per year (95% CI: [-1.65, -0.98%]). Using RCR, the predicted rate of change in Ch4 GMD was -0.00053 units/ month (95% CI: [-0.00066, -0.00040 unit/month], p<0.001), which is equivalent to -1.35% per year (95% CI: [-1.49, -1.22%]). Using RCR, the predicted rate of change in the MoCA score per month was -0.0048 (95% CI: [-0.00153, 0.0058], p=0.32). See Figure 1. Ch4 GMD and the MoCA score were signiﬁcantly correlated at baseline (rs=0.24; 95% CI:[ 0.04, 0.42]; p=0.018) and the rate of change in Ch4 GMD and rate of change in MoCA score were signiﬁcantly correlated (rs=0.26; 95% CI: MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S49 ABSTRACTS [0.06, 0.44], p=0.010). After controlling for false positive discovery error, the rate of change in Ch4 GMD between baseline and 4 years was signiﬁcantly associated with age (r=-0.35; 95% CI: [-0.52, -0.16], p<0.001) and the University of Pennsylvania Smell Identiﬁcation Test (UPSIT) total score (r=0.36; 95% CI: [0.17, 0.53]; p<0.001). A baseline Ch4 GMD adjusted multivariate linear regression model showed that greater age was associated with greater decline in Ch4 GMD between baseline and 4 years and that a higher UPSIT score was associated with less decline in Ch4 GMD between baseline and 4 years. Conclusions: Ch4 GMD linearly declines in early PD and decline in Ch4 GMD is correlated with decline in MoCA score. Greater age and impaired olfaction at baseline are associated with more rapid decline in Ch4 GMD over 4 years in early PD. 74 Cortical Activity During the Writing Task in Dystonia - A Study with Functional Near Infrared Spectroscopy (fNIRS) Renata Dalle Lucca (S~ ao José Dos Campos - SP, Brazil), Joana Balardin (S~ ao Paulo, Brazil), Danilo De Faria (Sso Paulo, Brazil), Artur José Paulo (Santo Andre, Brazil), Joao Ricardo Sato (S~ ao Paulo, Brazil), Carlos Baltazar (S~ ao Paulo, Brazil), Vanderci Borges (Sao Paulo, Brazil), Sonia Silva (Sao Paulo, Brazil), Henrique Ferraz (Sao Paulo, Brazil), Patricia De Carvalho Aguiar (Sao Paulo, Brazil) S50 Objective: This study aimed to investigate the cortical activity in focal right upper limb dystonia patients using functional near infrared spectroscopy (fNIRS) during writing. Background: Techniques such as fMRI and PET impose important physical constraints. Latter advances in functional near infrared spectroscopy offer a new possibility for investigating cortical areas and the neural correlates of complex motor behaviors non-invasively under naturalistic experimentation. Methods: Twenty-one patients with right upper limb idiopathic dystonia (6 with task-speciﬁc dystonia) and twenty-one healthy volunteers matched for age and years of education were submitted to a simple righthand writing task paradigm that consisted of 4 epochs of alternating writing/resting blocks. To test a priori hypotheses that brain activation to simple handwriting in cortical sensorimotor and supplementary motor regions would be less speciﬁc in patients than in controls, we deﬁned right and left primary motor (M1) and somatosensory (S1) and supplementary motor area (SMA) ROIs. Differences between groups on changes in both deoxy and oxy-Hemoglobin (HbO2) and for each ROI were then compared using Mann Whitney tests. Results: Channels exhibiting increased HbO2 for the writing task compared to the resting condition in the patient and control groups are described in Fig. 1. In controls, a lateralization predominantly to the left side was observed in most of the central channels covering the primary MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS motor and somatosensory cortices. A more bilateral pattern of activation was observed in patients. However, in a direct channel-wise comparison between groups, no signiﬁcant differences were observed in any channel. In the ROI-based group analysis, between-group differences were observed in two of the ﬁve ROIs: left M1 (p = 0.022) and S1 (p = 0.022) exhibited increased HbO2 in patients in relation to controls. Conclusions: Overactivity ﬁndings on M1 as S1 are in agreement with previous studies of the writing task in non-ecological conditions1,2,3, reinforcing the role of these areas in dystonia. As symptoms in dystonia are very speciﬁc to the task, we believe the pattern of brain activation is as much as speciﬁc, therefore the importance of an experimental set that mimics real life conditions. To our knowledge, this is the ﬁrst study to measure cortical activity during ecological writing (subjects sited in anatomical position, in less noisy environment, using traditional paper and pen) in focal upper limb dystonia patients, outlining the potential of fNIRS for the study of the movement disorders in unconstrained environments. References: 1. Delnooz, C.C.; Helmich, R.C.; Medendorp, W.P.; Van de Warrenburg, B.P.; Toni, I. Writer’s cramp: Increased dorsal premotor activity during intended writing. Hum. Brain Mapp., 2011, 0. 2. Odergren, T., Stone-Elander, S., & Ingvar, M. (1998). Cerebral and cerebellar activation in correlation to the action-induced dystonia in writer’s cramp. Movement Disorders, 13(3), 497–508. https://doi. org/10.1002/mds.8701303213. Carbon, M., Argyelan, M., Habeck, C., Ghilardi, M. F., Fitzpatrick, T., Dhawan, V., … Eidelberg, D. (2010). Increased sensorimotor network activity in DYT1 dystonia: a functional imaging study. Brain : a journal of neurology, 133(Pt 3), 690–700. doi:10.1093/brain/awq017 75 Unraveling the Cellular Mechanism Involved in the Neuroprotective Effect Mediated by Ilex Paraguariensis (Yerba Mate) Juan Ferrario (Buenos Aires, Argentina), Soledad Anesetti (Buenos Aires, Argentina), Hernan Hauche (Buenos Aires, Argentina), Alejandra Bernardi (Buenos Aires, Argentina), Pedro Ballestero (Buenos Aires, Argentina), Melina Bordone (Buenos Aires, Argentina), Oscar Gershanik (Buenos Aires, Argentina) Objective: To investigate the molecular mechanisms triggered by exposure to yerba mate [YM]) extract which may help to understand neuronal survival. Background: The consumption of some beverages, as coffee, tea and YM have been inversely associated with incidence of PD. In accordance, our group demonstrated that YM extract provides survival to dopaminergic neurons in vitro. Methods: As follow up of our previous work (Bernardi et al. 2019), we are investigating the cellular and molecular mechanisms regulated by YM treatment, which may be responsible of the observed neuroprotective effects. We have used two in vitro models to reach this goal: 1) We have studied cell growth, differentiation and regulation of key molecular markers by western blot on the human neuroblastoma SHSY5Y cells under YM treatment. We have treated cells with different concentrations and at different time points of YM. 2) We have analyzed the expression of those markers in dopaminergic neurons from mouse mesencephalic primary cultures. Cell cultures were either processed for western blot or ﬁxed and immunostained following standard procedures. Results: We found that yerba mate extract induces a mitogenic effect on the SH-SY5Y cell lines but it does not induces differentiation. Moreover, we measured the protein levels and phosphorylated status of key elements of energy signaling and autophagy (AMPK and p70S6K?), mitogenesis and differentiation (EGR-1) and the multifaceted ERK protein by Western Blot. We found that YM regulates these markers in a different manner. These results will be contrasted with the regulation of these markers triggered by caffeine, theobromine and chlorogenic acid, three components highly present in yerba mate extract. Conclusions: These initial results suggest that YM stimulates neuronal growth and has a role in cell energy homeostasis and/or autophagy. Because YM shares several active principles with coffee, our ﬁndings provide the ﬁrst insights to understand how these popular beverages may be inﬂuencing neuronal homeostasis impacting in their health and therefore in the evolution of neuronal diseases such as Parkinson’s disease. Much further work is still necessary to conﬁrm, interpret and translate our ﬁndings to understand the mechanism of neuronal survival and give support to the epidemiological studies involving these stimulant drinks. References: Bernardi A, Ballestero P, Schenk M, Ferrario M, Gómez G, Rivero R, Avale E, Taravini I, Gershanik O, Guerrero S, Ferrario JE. Yerba mate (Ilex paraguariensis) favors survival and growth of dopaminergic neurons in culture. Mov Disord. 2019 Jun;34 (6):920-922. doi: 10.1002/mds.27667. 76 Prefrontal Cortex Activation is Facilitated by Levodopa During Dual Task Walking in Parkinson’s Disease Lilian Gobbi (Rio Claro, Brazil), Diego Orcioli-Silva (Rio Claro, Brazil), Rodrigo Vitorio (Rio Claro, Brazil), Priscila Nóbrega-Sousa (Rio Claro, Brazil), Núbia Conceiç~ ao (Rio Claro, Brazil), Victor Beretta (Rio Claro, Brazil), Ellen Lirani-Silva (Caieiras, Brazil) Objective: To investigate the effect of dopaminergic medication on prefrontal cortex (PFC) activity and gait parameters during dual task (DT) walking in people with Parkinson’s disease (PD). Background: Although dopaminergic medication improves DT walking in people with PD, the underlying neural mechanisms are not yet fully understood. As prefrontal cognitive resources are involved in DT walking, evaluation of the PFC is required. Methods: Twenty individuals with PD and 30 healthy older people performed ﬁve trials of two walking conditions: single and dual task (walking while performing a digit vigilance task). Motor symptom severity of individuals with PD were assessed under ON and OFF medication MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S51 ABSTRACTS Table 1. Participant characteristics (Mean SD). Parkinson’s Control disease group Age (years) 69.8 5.9 68.0 5.6 Sex (Male/Female) 15/5 15/15 Height (cm) 166.210.2 163.48.9 Weight (Kg) 74.511.0 76.7 13.9 MMSE (0-30) 26.9 1.8 27.71.6 MDS-UPDRS III - OFF (0-132) 35.2 11.3 MDS-UPDRS III - ON (0-132) 27.7 11.4 Disease duration (years) 5.6 3.1 Levodopa equivalent 636.7261.2 dose (mg/day) p value .298 .140 .310 .561 .103 <.001* NA NA Notes: MMSE: Mini Mental State Examination; MDS-UPDRS III: Movement Disorders Society – Uniﬁed Parkinson’s Disease Rating Scale, motor part; NA: not-applicable; *difference between OFF and ON state. states and the Levodopa equivalent dose was recorded. Global cognitive function was assessed in all participants. Each trial started with 30s of standing still, followed by 30s of walking around a 26.8m circuit. A fNIRS system and a sensor carpet were used to record PFC (Brodmann areas 9, 10, and 46 in both hemispheres) activation and gait parameters, respectively. Relative concentrations of oxygenated hemoglobin (HbO2) from the left and right PFC were measured. Results: People with PD in the OFF state did not present changes in HbO2 level in the left PFC across walking conditions. In contrast, in the ON state, they presented increased HbO2 level during dual task compared to single task. Regardless of medication state, people with PD presented increased HbO2 level in the right PFC during dual task walking compared to single task. The control group demonstrated increased PFC activity in both hemispheres during dual task compared to single task. People with PD showed increases in both step length and velocity in the ON state compared to OFF state. [table1]; [ﬁgure1]; [table2] Conclusions: With medication, people with PD presented increased (left) PFC activity in the dual task condition, which are in line with the CRUNCH framework1. Dopaminergic medication, which ameliorates the connectivity between striatal and cortical areas2, may increase available resources and compensatory potential1, as well as improving gait parameters. Maybe PFC activation is inﬂuenced by the “posture second” strategy used during dual task walking in PD3. PD limits the activation Figure 1. Normalized oxygenated hemoglobin (HbO2) levels in early and late phases during Single and Dual task for PD OFF, PD ON, and control group. * indicates difference between PD OFF and PD ON and # indicates differences between walking conditions for PD ON. Main effect of condition is expressed by @ (PDOFF vs PD ON), + (PD OFF vs CG), and & (PD ON vs CG). S52 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS of the left PFC during dual task walking and dopaminergic medication facilitates its recruitment. References: 1. Reuter-Lorenz PA, Cappell KA. Curr Dir Psychol Sci. 2008;17(3):177-182. 2. Gilat M, et al. Neuroimage. 2017;152:207-220.3 Nieuwhof F, et al. Pilot Feasibility Stud. 2016;2(1). Neuropharmacology 63 Chlorogenic Acid Protects Dopaminergic Neurons in Parkinsonian Mouse Model Through Suppressing Mitochondrial Dysfunction and Preventing Apoptotic Cascade Saumitra Singh (Varanasi, India), Surya Singh (Varanasi, India) Objective: To investigate the effect of Chlorogenic acid in MPTP induced mitochondrial dysfunction mediated apoptotic death of Dopaminergic neurons in Parkinsonian mouse model. Background: The progressive loss of dopaminergic neurons of substantia nigra results in Parkinson’s disease, marked by disabling motor abnormalities. The exact mechanism underlying PD pathogenesis is yet to be understood; however, it is attributed that mitochondrial malfunction may play a key pathogenic component in PD. Various toxins involved in the characteristic symptoms of PD and genes associated with familial PD induce mitochondrial dysfunction. For that reason, treatments focusing on effective restoring of mitochondrial function may be useful in preventing the progression of PD. Methods: For drug treatments, animals were randomly divided into four groups (eight mice in each group). The ﬁrst group served as control. The Second group was intraperitoneally injected with MPTP (20 mg/kg body weight, 5 doses). The third group received oral treatment with Chlorogenic acid (CGA, 50 mg/kg body weight) in conjunction with MPTP. Whereas, the fourth group animals were only administered with CGA. All the drug treatments were given every day for a period of 24 days. Results: The results indicated that CGA treatment subsequent to MPTP intoxication partially prevented the decline in activity of mitochondrial Complexes-I, and –IV, along with improving the mitochondrial GSH level as well as SOD activity. Furthermore, CGA attenuated the MPP+ induced decrease in ATP synthase activity indicating increased energy available for dopamine biosynthesis. CGA was also reported to improve mitochondrial membrane potential (MMP) in the striatum as compared to the MPTP intoxicated group, indicating restoration of mitochondrial function. Moreover, it was demonstrated that CGA may have reversed the changes in expression of Bcl-2, Bax and caspase-3 in the substantia nigra pars compacta (SNpc) of mice, in addition to signiﬁcantly improving the TH expression in SNpc. Conclusions: Overall, our results suggested that CGA administration reduced MPTP induced mitochondrial dysfunction and apoptotic death of DA neurons, indicating its therapeutic effects in MPTP-induced PD model. References: 1. Luo Y, Hoffer A, Hoffer B, Qi X: Mitochondria: a therapeutic target for Parkinson’s disease? International journal of molecular sciences 2015, 16(9):20704-20730.2. Singh SS, Rai SN, Birla H, Zahra W, Kumar G, Gedda MR, Tiwari N, Patnaik R, Singh RK, Singh SP: Effect of Chlorogenic Acid Supplementation in MPTP-Intoxicated Mouse. Frontiers in pharmacology 2018, 9. Non-Motor Symptoms 59 Non-Motor Symptoms of Parkinson’s Disease: The Role of Physical Activity Assessed by Phone-FITT Questionnaire Joyce Lima (Fortaleza, CA, Brazil), Sâmmara Pinto (Fortaleza, Brazil), Flavia Rolim (Fortaleza / Ceara, Brazil), Andre Gomes (Fortaleza, Brazil), Vivia Mesquita (Fortaleza, Brazil), Antonia Rosivalda Marinho (Fortaleza, Brazil), Fernanda Maia Carvalho (Fortaleza, Brazil) Objective: To identify a possible association between the degree of physical activity and engagement in daily living activities measured by Phone-FITT questionnaire and occurrence of NMS in PD patients. Background: Non-motor symptoms (NMS) such as insomnia, depression, anxiety, apathy, bladder dysfunction, pain, fatigue, cognitive impairment and dementia are extremely frequent and can appear during all stages of Parkinson’s disease (PD), including the premotor stage, leading to severe disability and shortened life expectancy, with burden on quality of life of patients and their caregivers. Physical activity has been known to improve motor and NMS in PD, with a positive impact in disease progression and quality of life. Methods: Cross-sectional study. Clinical and demographic data were obtained of 80 PD patients from the neurology outpatient clinic of the Hospital Geral de Fortaleza. UPDRS-motor score, Hoehn-Yahr stage, Schwab and England daily activity scale and Phone-FITT questionnaire were performed. NMS were evaluated through Non-Motor Symptoms Questionnaire (NMSQuest). Data data were processed and analyzed by SPSS. Pearson Correlation test was performed to assess the association between NMS and physical activity. Results: Of 80 subjects, 65% were male, mean age was 62.35?? 11.71?years-old, and median disease duration was 9,14?years [1–27], with median Hoehn-Yahr Stage 2. At least two NMS was reported by 100% of patients, mostly anxiety, pain, restless legs, nocturia and intense and vivid dreams. Phone-FITT overall score was 26.9, with a predominance of housework activities. We found no statistically signiﬁcant relationship between physical activity and the presence of NMS of PD, yet PhoneFITT proved to be a suitable tool to evaluate the practice of physical activity in this population. Conclusions: Our data show that all patients have presented at least two NMS. Anxiety, pain, restless legs were the most frequent. PhoneFITT proved to be a suitable tool to evaluate the practice of physical activity in PD patients. Even though we found no correlation with occurrence of NMS, we know from several reports that physical activity has a positive impact in disease progression and quality of life. References: A. H. V. Schapira, K. R. Chaudhuri, and P. Jenner, “Non-motor features of Parkinson disease,” Nat. Rev. Neurosci., vol. 18, no. 7, pp. 435–450, 2017. A. W. Amara and A. A. Memon, “Effects of Exercise on Non-motor Symptoms in Parkinson’s Disease,” Clin. Ther., vol. 10, no. 1, pp. 8–15, 2017. M. E. Cusso, K. J. Donald, and T. K. Khoo, “The Impact of Physical Activity on NonMotor Symptoms in Parkinson’s Disease: A Systematic Review,” Front. Med., vol. 3, pp. 1–9, 2016. D. P. Gill, G. R. Jones, G. Y. Zou, and M. Speechley, “The phone-FITT: A brief physical activity interview for older adults,” J. Aging Phys. Act., vol. 16, no. 3, pp. 292–315, 2008. 60 Dyssynergic Defecation is at the Root of Constipation and Other GI Issues in Parkinson’s Disease: Preliminary Results of Comprehensive Neurogastroenterology Evaluation Amol Sharma (Augusta, GA, United States), Yun Yan (Augusta, GA, United States), Xuelian Xiang (Augusta, GA, United States), Shashana Fielder (Miami, United States), Rachel Parr (Augusta, GA, United States), Anam Herekar (Augusta, GA, United States), Julie Kurek (Evans, GA, United States), John Morgan (Augusta, GA, United States), Satish Rao (Augusta, GA, United States) Objective: Our aim was to characterize anorectal motor function, gastric emptying time, small bowel and colonic transit times, and evaluate for the presence of SIBO in each individual subject of a PD cohort. Background: Chronic constipation is a common symptom in Parkinson’s disease (PD), precedes neurological manifestation by over 15 years, and carries a hazard ratio of >3.3 of developing PD. Whether these patients have coexisting and unrecognized dysmotility of the anorectum, colon, and other portions of the GI tract is unclear. Methods: PD subjects were recruited to undergo a comprehensive neurogastroenterology and GI motility (NGM) evaluation including high-resolution anorectal manometry, balloon expulsion test, wireless motility capsule (WMC), and glucose breath testing. Constipation was deﬁned by =3 complete spontaneous bowel movements per week or regular use of a medical therapy for constipation. Dyssynergic defecation was deﬁned by a manometric pattern of dyssynergic defecation and either prolonged balloon expulsion time (> 60 sec) or delayed colonic transit time on WMC. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S53 ABSTRACTS in response to electrical stimulation (200 stimuli) of the anus and rectum with a 4-sensor probe. Using magnetic coils, bilateral transcranial magnetic stimulation (TMS) and translumbosacral anorectal magnetic stimulation (TAMS) was administered to obtain anal and rectal motor evoked potentials (MEPs) in order to assess the efferent pathway. CEP responses were measured for latencies of P1, P2, N1 and N2 waveform responses. Anal and rectal sensory thresholds to electrical stimulation were also captured. All neurophysiological data were compared with previously collected normative data. Results: 16 enrolled PD subjects (F/M=7/9) were compared to 31 healthy subjects (F/M=20/11). 81.3% of PD patients (13/16) had constipation deﬁned by <3 complete spontaneous bowel movements/ week or regular laxative use. The anal and rectal CEP latencies were prolonged, but not signiﬁcantly, in PD when comparted to healthy subjects, possibly due to type II error (table 1). There was diffuse signiﬁcant delays in latencies of bilateral lumbosacro-anorectal MEPs with the exception of right lumborectal MEPs (table 1). There was also only signiﬁcant prolongation of the right cortico-rectal MEP latency, but not others, after TMS in PD subjects. The thresholds for the ﬁrst and pain sensations to the anal and rectal electrical stimulation were signiﬁcantly higher in PD patients (table 2). Conclusions: Our study suggests signiﬁcant efferent neuropathy in the majority of lumbosacro-anorectal pathways and an isolated unilateral cortico-rectal pathway as well as signiﬁcant anorectal hyposensitivity in PD subjects. The afferent anorectal-cortical function seems intact. Together, these neurophysiological abnormalities may help explain neurogastrointestinal dysfunction and symptoms in PD that merit further validation. Acknowledgement: This study is supported by the Parkinson’s Foundation Translational Research Grant (PF-TRG-1730). References: Remes-Troche, Jose M., et al. “A bi-directional assessment of the human brain-anorectal axis.” Neurogastroenterology & Motility 23.3 (2011): 240-e118. Results: In this interim analysis, 28 PD subjects (17 M, 11 F, mean age 66.98.4 years) were enrolled. Sixteen of 28 PD subjects (57.1%) had constipation. Fifteen of 28 PD subjects (53.6%) had dyssynergic defecation. Of the PD subjects with constipation, 10 (66.7%) had dyssynergic defecation, 2 (13.3%) had slow-transit constipation, 1 (6.7%) had normal transit constipation. Two PD subjects did not complete all testing for constipation. Six of 26 (23.1%) PD subjects had delayed gastric emptying on WMC or gastroparesis. Five of these 6 subjects (83.3%) had coexisting dyssynergic defecation. The other PD subject with delayed gastric emptying had delayed small bowel transit time and small intestinal bacterial overgrowth (SIBO) on glucose breath testing. Seven of 23 PD subjects who completed glucose breath testing tested positive for SIBO. Five of these PD subjects (71.4%) had co-existing dyssynergic defecation. Conclusions: Our study demonstrates the majority of PD subjects with or without constipation exhibit dyssynergic or disordered defecation. The majority of other NGM disorders such as gastroparesis and SIBO in PD occur in the presence dyssynergic defecation. These ﬁndings suggest that dyssynergic defecation lies at the root of neurogastrointestinal dysfunction and GI symptoms in PD that merits further validation. Acknowledgement: This study is supported by the Parkinson’s Foundation Translational Research Grant (PF-TRG-1730). 61 Discovering the Roadblocks in Parkinson’s Disease: Preliminary Results of Comprehensive Bidirectional Brain-Gut Investigations Amol Sharma (Augusta, GA, United States), Yun Yan (Augusta, GA, United States), Xuelian Xiang (Augusta, GA, United States), Shashana Fielder (Miami, United States), Rachel Parr (Augusta, GA, United States), Anam Herekar (Augusta, GA, United States), Enoe Jimenez (Augusta, GA, United States), Julie Kurek (Evans, GA, United States), John Morgan (Augusta, GA, United States), Satish Rao (Augusta, GA, United States) Objective: To study anorectal sensory function and bidirectional brain-gut interactions between the pelvic ﬂoor, lumbosacral spine, and cortex in Parkinson’s disease (PD) versus healthy subjects. Background: Chronic constipation is a common symptom in PD that often precedes neurological manifestation by over 15 years, with a hazard ratio of >3.3 of developing PD. Lewy pathology, abnormal neuronal accumulation of distorted alpha-synuclein, may start from the anorectum and spread towards the CNS in PD. Methods: Anorectal cortical evoked potentials (CEP) were obtained 62 The Impact of Three Distinct Exercise Types on Fatigue, Anxiety, and Depression in Parkinson’s Disease Mary Feldman (Centerville, OH, United States), Ashley Paul (Dayton, OH, United States), John Tomeny (Hanover, NH, United States), Angeline Andrew (Hanover, NH, United States), Stephen Lee (Lebanon, NH, United States) Objective: To identify which of three contrasting types of exercise (spinning, yoga, or dance) is most beneﬁcial for prominent non-motor features of Parkinson’s disease (PD): fatigue, anxiety, and depression. Background: There is evidence that exercise improves both motor and non-motor symptoms in Parkinson’s Disease1,2. However, patients often ask which type of exercise is most beneﬁcial for their speciﬁc nonmotor symptom, which has not been well studied. Methods: This is a multicenter, prospective, randomized pilot study, designed with a “Parkinson’s Advocate in Research” (PAIR) as deﬁned by the Parkinson’s Foundation. Twenty-four patients with idiopathic Parkinson’s disease between the ages of 18-75, a Hoehn Yahr score (HYS) <4, MMSE > 23, and a UDPRS < 60 were recruited. Patients had a baseline abnormal score in at least one of the following: Beck Depression Index II (BDI-II), Fatigue Severity Scale (FSS), or the Zung self-rating Anxiety Scale (ZAS). They were randomized to one of three interventions (dance, spin, yoga) and participated in a 1 hour class twice per week for six weeks. Patients completed the BDI-II, FSS, and ZAS at the end of each week and 4 weeks post-study. Results: There was no signiﬁcant difference in baseline demographics among the 3 arms of the study except that the dance groups were all Table 1. Afferent (CEPs), efferent anorectal-cortical and peripheral lumbosacral-anorectal nerve conduction (MEPs) in patients with PD. PD (n=16) HV (n=32) p-value (ms) (ms) CEP-Anal, N2 121.5 + 10.2 109.3 + 3.3 0.61 CEP-Rectal, N2 108.3 + 3.9 104.0 + 3.0 0.26 Left-lumbar anal MEP 4.6 + 0.2 3.4 + 0.1 0.0001* Right-lumbar anal MEP 5.2 + 0.5 3.4 + 0.1 0.003* Left-sacral anal MEP 4.5 + 0.3 3.0 + 0.1 0.0001* Right-sacral anal MEP 4.6 + 0.5 3.1 + 0.1 0.006* Left-lumbar rectal MEP 4.1 + 0.4 2.9 + 0.1 0.003* Right-lumbar rectal MEP 4.2 + 0.6 3.2 + 0.1 0.17 Left-sacral rectal MEP 3.6 + 0.2 2.9 + 0.2 0.01* Right-sacral rectal MEP 4.1 + 0.4 3.0 + 0.1 0.043* *p-value < 0.05 Table 2. Anorectal sensation to electrical stimulation in patients with PD. 1st sensation (mA) pain sensation (mA) Anal stimulation PD (n=10) 27.8 + 6.6 76.2 + 6.5 0.003* Rectal stimulation PD (n=10) HV (n=31) 40.9 + 6.9 19.8 + 2.1 0.003* 0.0001* 85.4 + 5.4 0.0001* P HV (n=31) 9.3 + 1.2 38.5 + 3.9 *p-value < 0.05 S54 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 52.4 + 4.7 P ABSTRACTS male. Statistical analysis of the data shows that in patients who had moderate to severe depression at baseline, the median BDI-II depression score during exercise was 51% lower with spinning, and 28% lower with dance [ﬁgure 1]. Across all exercise groups, the number of patients that selected BDI-II “I do not feel sad” during exercise improved by 26.5% from baseline. At screening 6 people reported suicidal ideation (SI); 5 out of 6 had SI resolve with aerobic exercise. At 4 weeks post-study, repeat BDI-II scores worsened compared to during exercise, but remained lower than baseline scores in dance and spin groups. Conclusions: This pilot study was underpowered and thus statistical signiﬁcance could not be reached; however, important trends were demonstrated. There was marked improvement for subjects who had baseline severe depression with dance and spin, over yoga. Additionally, across all subjects and exercise interventions, there was an improvement in feelings of sadness that approached statistical signiﬁcance. Remarkably, 5 patients no longer reported SI during aerobic exercise. This study raises important points and suggests that aerobic exercise and the social aspects of dancing to music possesses unique pathophysiological properties which can help treat depression in a non-pharmacological way or as a medication adjunct. Additional research is needed. References: 1) Anwar Ahmed, Angela L. Ridgel, Michael J. Phillips, Jerrold L. Vitek, Mark L. Lowe, Mark Hutson, Mary Feldman and Jay L. Alberts. Cortical and motor responses to acute forced exercise in Parkinson’s disease. Parkinsonism and Related Disorders. 2016 2) Abrantes, A, Friedman, J, Brown, et al. Physical Activity and Neuropsychiatric Symptoms of Parkinson Disease. J of Geriatric Psychiatry and Neurology. 2012. 00(0) 1-8. Table 1. Abnormally small letter Size of letters m1 < 3 mm m10 < 3 mm e1 < 1,5 mm e10 < 1,5 mm Handwriting size Width of letter “m” Height of letter “e” Height of letter “e” Variability in the Frequency of Micrograph in Parkinson’s Disease Cynthia Garcia Fernandez (Capital Federal, Argentina), Adriana Ziliotto (Buenos Aires, Argentina), Gabriela Raina (Buenos Aires, Argentina), Cristian Calandra (Buenos Aires, Argentina), Ricardo Maiola (Buenos Aires, Argentina), Nicolas Morera (Buenos Aires, Argentina), Graciela Cersosimo (Buenos Aires, Argentina) Objective: The frequency of micrographia in Parkinson’s disease (PD) is quite variable. A precise and uniform deﬁnition of this graphological phenomenon is lacking. It is usually described as abnormally small letter size or decreasing size of letters. Background: We sought to address: (i) the frequency of micrographia in PD patients and controls according to different deﬁnitions, (ii) whether these manifestations occur early in the course of PD, and (iii) the effect of levodopa treatment on letter size. Methods: All subjects were asked to write a series of 10 repetitions of the sentence in Spanish “respiro el dulce aroma de las ﬂores”. We included cases who spontaneously wrote using cursive HR. We measured the width of the ﬁrst and last letter "m" and the height of the ﬁrst and last letter "e" of the series. Abnormally small letter was considered when the height of the "e" was less than 1.5 mm or the width of the "m" less than 3 mm. It was considered that there was decreasing size when the last version of these letters was smaller than the ﬁrst. The magnitude of such p value ns 0.01 0.02 0.05 PD (n= 55) Decreasing Increasing Unchanged Decreasing Increasing Unchanged n 23 25 7 27 13 15 % 41,8 45,5 12,7 49,1 23,6 27,3 Controls (n= 57) n % 13 22,8 34 59,6 10 17,5 26 45,6 14 24,6 17 29,8 p value 0.04 ns ns ns ns ns Table 3. Magnitude of handwriting decrement Width of letter “m” 99 Controls (n= 57) n % 0 0 0 0 1 1,8 3 5,3 Table 2. Changes in handwriting size Variable Other PD patients (n= 55) n % 2 3,6 6 10,9 7 12,7 9 16,4 Percentage (%) ≥ 10 ≥ 30 ≥ 50 ≥ 10 ≥ 30 ≥ 50 PD (n= 55) n 18 8 1 31 16 5 % 32,7 14,5 1,8 50,8 26,2 8,2 Controls (n= 57) n % 9 15,7 0 0,00 0 0,00 25 43,90 12 21,10 3 5,30 p value 0.03 0.003 ns ns ns ns decrement was expressed as a percentage. Patients were evaluated with the UPDRS-III and the HY scales. Patients with motor ﬂuctuations were evaluated in ON or OFF motor states. Results: Fifty-ﬁve PD patients and 57 controls were evaluated. In PD patients the frequency of micrographia ranged between 3.6 and 50.8%, according to the criteria considered for its deﬁnition. Considering those ﬁndings that were signiﬁcantly different to controls, abnormally small letter was found in 10.9% of the patients vs none of controls. Decreasing size of HR regardless of the percentage of reduction was present in 41.8% of PD patients and 22.8% of controls. A reduction = 10% in the width of the “m” occurred in 32.7 vs. 15.7% of patients and controls respectively (p 0.03); and a decrease = 30% in 14.5% of patients vs none of controls. Letter size was reduced since the ﬁrst year of the illness. HR size was similar in the ON and OFF states. Conclusions: (i) the frequency of micrographia in our series was extremely variable and depends on the criteria used to deﬁne it; (ii) letter size was already affected within the ﬁrst year of the disease; (iii) levodopa did not signiﬁcantly improve HR. References: 1. McLennan J., Nakano K., Tyler H., Schwab R. Micrographia in Parkinson’s Disease. J. neurol. Sci., 1972, 15:141-1522. Inzelberg R., Plotnik M., Harpaz., Flash T. Micrographia, much beyond the writer’s hand. Parkinsonism and Related Disorders xxx (2016) 1-93. Maa H-I., Hwangb W-J, Chang S-H, Wanga T-Y.Progressive micrographia shown in horizontal, but not vertical, writing in Parkinson’s disease. Behavioural Neurology 27 (2013) 169–174 100 Alpha-Synuclein Level in Plasma Extracellular Vesicles in Dementia with Lewy Bodies Konstantin Senkevich (Saint-Petersburg, Russia), Mikhail Nikolaev (Gatchina, Russia), Daria Kulabukhova (Saint-Petersburg, Russia), Alena Kopytova (SaintPetersburg, Russia), Tatiana Usenko (Saint-Petersburg, Russia), Anton Emelyanov MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S55 ABSTRACTS (Saint-petersburg, Russia), Yulia Beltseva (Saint-Petersburg, Russia), Natalia Zalutskaya (Saint-Petersburg, Russia), Sofya Pchelina (Saint-Petersburg, Russia) Objective: To estimate the level of total alpha-synuclein in plasma extracellular vesicles (exosomes) of patients with dementia with Lewy bodies and controls. Background: Extracellular vesicles (e.g., exosomes) are membrane vesicles released from cells into the extracellular space, and they are produced by all nervous tissue cells. Exosomes considered as a transporter for misfolded alpha-synuclein spreading. Methods: Blood was obtained from patients with dementia with Lewy bodies (DLB) (N=14) and controls (N=16). Plasma was collected from 8 ml of peripheral blood by centrifugation at room temperature (4000 Rpm) for 20 minutes. After, plasma was centrifuged at 14 thousand Rpm for 20 minutes to precipitate cell debris. Total alpha-synuclein levels in plasma exosomes were measured by enzyme-linked immunosorbent assay with SensoLyte Anti-alpha-Synuclein Quantitative ELISA Kit (Human) (AnaSpec, USA) and normalized to total protein. To isolate the exosomes from human plasma we used the Exo-Prep (HansaBioMed, Estonia). Lysis was carried out by Total Protein Extraction Kit (Chemicon (Millipore), USA). Data expressed as the median (minimum-maximum). Results: There was no signiﬁcant difference in the level of total alpha-synuclein in DLB patients 29,02 (1,54-105,1) pg/mkg compared to controls 23,4 (6,8-75,3) pg/mkg, p=0,313. We’ve also didn’t ﬁnd any correlation between age of onset, disease duration or cognitive status estimated by MMSE and MoCa scales and total exosomal alpha-synuclein. Conclusions: Our result suggests that total exosomal alpha-synuclein from blood plasma is not a reliable biomarker for DLB. Acknowledgments: The work was supported by RFBR ? 18-315-00387 mol_a. inhibition of COMT can improve levodopa treatment outcomes by decreasing variability in circulating levodopa concentrations. Opicapone is a highly selective once-daily COMT inhibitor under development in the United States as an adjunct to levodopa for PD ﬂuctuations. Methods: Once-daily opicapone 50 mg was administered in the evening on Days 1-14. After the ﬁrst and last opicapone dose, serial blood samples were collected to determine plasma opicapone concentrations and erythrocyte soluble COMT (S-COMT) activity. Participants were randomized to receive levodopa/carbidopa (100/25 mg) every 3 or 4 hours (Q3H or Q4H) on PK sampling days, and their usual levodopa/ carbidopa regimen on other days. On Day 1 (prior to opicapone dosing), Day 2, and Day 15, serial blood samples were collected after the ﬁrst 3 levodopa doses to determine plasma concentrations of levodopa and its 3-OMD metabolite. The effects of opicapone on S-COMT activity, along with its effects on the PK of levodopa and 3-OMD, were assessed. Results are presented as mean values standard deviations. Results: A total of 16 participants were enrolled in the study (men=10, women=6). At steady state (Day 14), opicapone Cmax and AUC0-last were 459252 ng/mL and 2022783 ng/mLhr, respectively. Compared to baseline, on Day 14 COMT activity was inhibited on average by 76.35.4%. After opicapone administration, levodopa total AUC, peak concentration, and trough concentration increased; peak-totrough ﬂuctuation index decreased. Correspondingly, 3-OMD total AUC, peak concentration, and trough concentration decreased (Table 1). Conclusions: Adding once-daily opicapone 50 mg to levodopa resulted in substantial and prolonged COMT inhibition, which decreased systemic exposure to 3-OMD and increased systemic exposure to levodopa. These changes translated into higher trough concentrations and decreased peak-to-trough ﬂuctuations for levodopa. 130 Parkinson’s Disease 129 The Pharmacokinetics and Pharmacodynamics of Once-Daily Opicapone 50 mg in Patients with Parkinson’s Disease on Levodopa/Carbidopa: Results of a Phase 1 Study Gordon Loewen (San Diego, CA, United States), Peter LeWitt (West Bloomﬁeld, MI, United States), C. Warren Olanow (Rye, NY, United States), Karl Kieburtz (Sarasota, FL, United States), Grace Liang (San Diego, CA, United States), Roland Jimenez (San Diego, CA, United States), Kurt Olson (Del Mar, CA, United States), Eiry Roberts (San Diego, CA, United States) Objective: To evaluate the pharmacokinetics (PK) and pharmacodynamics of once-daily opicapone 50 mg as an adjunctive to levodopa/carbidopa in patients with stable Parkinson’s disease (PD). Background: Since levodopa administered with a dopa decarboxylase inhibitor is predominantly metabolized in the periphery by catecholO-methyltransferase (COMT) to 3-O-methyldopa (3-OMD), sustained Risk for Psychiatric Manifestations After Deep Brain Stimulation Surgery in Patients with Parkinson’s Disease Andreea Seritan (San Francisco, CA, United States), Jason Parad (San Francisco, CA, United States), Sarah Wang (San Francisco, CA, United States), Jill Ostrem (San Francisco, CA, United States), Ana-Maria Iosif (Davis, CA, United States) Objective: To explore the association of perioperative risk factors with postoperative psychiatric manifestations in patients with Parkinson’s disease (PD) who had deep brain stimulation (DBS) surgery. Background: DBS therapy is increasingly used in the treatment of PD, with excellent motoric outcomes. Albeit low, there is a risk of psychiatric complications associated with DBS therapy (particularly with subthalamic nucleus target), including depression, impulsivity, mania, and suicidality. Methods: IRB approval was obtained. This was a retrospective chart review of 50 patients with PD treated with DBS who were evaluated by the psychiatrist at the UCSF Movement Disorders and Neuromodulation Center between October 2015 and December 2018. Postoperative Table 1. Pharmacokinetic Parameters for Levodopa and 3-OMD after the Third Carbidopa/Levodopa Dose Parameter, mean (SD) Cmax, ng/mL Total AUC, ng/mLha Ctrough, ng/mL Peak-to-trough ﬂuctuation index, % Levodopa Day 1 Before Opicapone Q3H Q4H (n=7) (n=8) 1412.8 1420.9 (654.2) (678.2) 7339.4 7569.7 (2511.8) (2674.8) 683.0 401.4 (179.0) (192.4) 88.3 173.2 (40.3) (51.9) Day 15 With Opicapone Q3H Q4H (n=7) (n=8) 1954.2 1920.6 (615.0) (684.3) 11713.7 13158.9b (4429.2) (4527.8) 1363.4 842.8 (519.0) (332.1) 58.1 94.3 (23.0) (25.9) 3-OMD Day 1 Before Opicapone Q3H Q4H (n=7) (n=8) 3730.2 3960.8 (1537.1) (1384.2) 27847.8 43593.0 (13702.3) (14917.9) 3619.9 3779.0 (1496.9) (1392.1) 16.5 15.6 (7.1) (3.8) a Day 15 With Opicapone Q3H Q4H (n=7) (n=8) 616.7 671.3 (235.1) (255.5) 4589.7 9551.7b (1840.5) (7236.2) 579.3 604.0 (190.7) (223.1) 19.1 17.0 (12.9) (11.3) Sum of the ﬁrst three doses. Day 15, Q4H (n=9). 3-OMD, 3-O-methyldopa; AUC, area under the concentration-time curve; Cmax, maximum observed plasma concentration; Ctrough, observed trough plasma concentration at the end of the dose interval; Q3H, carbidopa/levodopa dose every 3 hours; Q4H, carbidopa/levodopa dose every 4 hours; SD, standard deviation. b S56 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS psychiatric manifestations explored included: depression, elevated mood, impulse dyscontrol, psychosis, and suicidal ideation or behavior, within one year of surgery. Risk factors examined included patient-related characteristics (age at surgery, gender, age at PD diagnosis, marital status, current or past psychiatric symptoms: depression, mania, impulse dyscontrol, psychosis, substance use, and suicidal ideation or behavior; type of psychiatric treatment received: medications or psychotherapy; family psychiatric history; medical comorbidities; and interpersonal stressors) and DBSrelated characteristics (target, time since surgery, number of programming visits, surgical complications). Multivariate logistic regression will be used to assess the association of risk factors with presence of psychiatric manifestations. Results: Data analysis is underway, in order to identify preoperative risk factors associated with postoperative psychiatric manifestations in patients with PD treated with DBS, within one year of surgery. These risk factors may serve as predictors of psychiatric complications of DBS therapy and should be discussed with patients and families during preoperative counseling. Our ﬁndings will need to be replicated in larger samples of patients with PD from other DBS centers. Conclusions: Predicting the risk of psychiatric manifestations associated with DBS therapy in patients with PD is an important aspect of preoperative counseling and informs postoperative care. An evidencebased approach to this crucial, expanding neuromodulation ﬁeld is proposed. References: Lang A, Houeto JL, Krack P, et al. Deep brain stimulation: Preoperative issues. Mov Disord 2006;21:S171-S196Seritan AL, Ureste P, Duong T, et al. Psychopharmacology for patients with Parkinson’s disease and deep brain stimulation: Lessons learned in an academic center. Curr Psychopharmacol 2019;8:41-54. 131 Withdrawn by Author 132 Male Gender and the Risk of Parkinson’s Disease in an Essential Tremor Population Mihai Cosmin Sandulescu (Danville, PA, United States), Erin Vanenkevort (Danville, United States), Mudit Gupta (Danville, PA, United States), Ramin Zand (Danville, United States) Objective: To evaluate the association between gender and the risk of Parkinson’s disease (PD) in patients with a prior history of essential tremor (ET). Background: Male gender is a risk factor for PD in the general population1. One study suggests that male gender is more frequent in the ET-PD subjects2. Methods: We performed a retrospective cohort study from the Geisinger Health System, which serves a stable rural population from central Pennsylvania. Exclusion criteria included subjects with secondary, vascular or atypical PD, but also drug-induced parkinsonism or tremor. We excluded any diagnosis of PD made on or before the date of ET diagnosis. Neurologists made both diagnoses. The index date, that is the date at which follow up time started was determined based on when ET began to. Comparisons across groups were accomplished using the Kruskal-Wallis test. Cox Proportional Hazard regression model was ﬁt treating time until Parkinson’s as the outcome and censoring follow up time at the date of the last encounter. Results: We identiﬁed a total of 3226 patients with ET, of which 331 developed later PD. The mean duration of follow-up time in the system was 14 years. Female gender has a mean age at PD diagnosis of 71.13 (95% CI 69.47 - 72.78) versus 70.58 (95% CI 68.95 - 72.21). The mean age at ET diagnosis of that population is 67.81 (95% CI 66.15 69.47) for females and 66.99 for males (95% CI 65.33 - 68.65). Male gender has a crude hazard ratio of 1.39 (95% CI 1.12 – 1.72). It reaches 1.61 (95% CI 1.27 - 2.04) with adjustments. We ﬁtted the analysis for age, history of hyperlipidemia, smoking status, appendectomy before PD, COPD or asthma, constipation, depression, anxiety, diabetes or REM behavior disorder, family history of tremor and, or parkinsonism. Conclusions: Male gender is an independent risk factor with 60% increased hazard for PD in our ET population, conﬁrming the prior ﬁndings from the general population1. Female gender seems to be older at ET and PD diagnosis, but the results did not reach statistical signiﬁcance. References: 1. Marras C, Beck JC, Bower JH, et al. Prevalence of Parkinson’s disease across North America. NPJ Parkinsons Dis. 2018;4:21. 2. Minen MT, Louis ED. Emergence of Parkinson’s disease in essential tremor: a study of the clinical correlates in 53 patients. Mov Disord. 2008;23(11):1602-1605. 133 An Outlook: Palliative Care for Patients with Parkinsonism Syndrome Mudassar Arain (Jamshoro, Pakistan) Objective: The main purpose of our study is to provide interventions and highlight the assessment of supportive care needs in Parkinson’s disease and improve the care and management quality of the patients with Parkinsonism syndrome. Background: A clinical syndrome known as parkinsonism is a combination of different complications such as bradykinesia, postural instability, rest tremor, apraxia and rigidity. Most common type of Parkinsonism is Parkinson disease (PD). It is a chronic neurodegenerative disease that requires supportive care and treatments including non pharmacological treatments, such as palliative care for patient, physiotherapy, family education, emotional support, general wellness maintenance, and nutrition. Palliative therapy is ﬁrst step hope and support of patient care treatment. this type of therapy focuses on providing relief from the normal reactions of anxiety, anger, social and psychological problems of patients and their family that begin with diagnosis of neurological disorders. Methods: A cross-sectional study was conducted to ﬁnd out the prospective of PD in resident of different rural and urban areas of Hyderabad, Pakistan. Initially stage, we used a expediency sample. However, as the study progressed, we gathered utmost verity of sampling to make sure the variety in terms of demographic data, Parkinsonism syndrome complications, treatment and management status. During study, total of 40 individual level interviews & 4 group of focus were held to obtain the information of patients, those are not satisﬁed to palliative care & preferences for addressing these needs. Interview domains & sample questions are based on communication, symptoms, Psychological, Quality of life, social, and Advance care planning. Patients were gave their opinions related to nonpharmacologycal treatments and palliative care during interview. We used inductive qualitative data analysis techniques to understand the responses. Results: Thus, almost in all patients described how social embarrassment and the unpredictable nature of symptoms led to restriction of activities and social withdrawal. Speech apraxia and gait issues were most common cause of withdrawal of social activities for patients (81%).after interventions and palliative care support their social activates increase for patients (48%). The concept of self and relationship changes affected patients’ sense of self in 68% cases and Depression is common in these patients. The majority of patients (92%) did not have any idea about palliative care and however, when educated on supportive care and management, patients responded positively. 68% patients believe that Religion and spirituality is a source of treatments and remaining patients consider meditation & prayer both are impotent for treatment. Conclusions: These results concluded the Parkinsonism syndrome and their complications are major burden on the person and also their families. So, palliative care is a supportive treatment for patients of Parkinsonism which treat and develop believe and hope of recovery in patients. This abstract was presented at the 2019 International Congress of Parkinson’s Disease and Movement Disorders in September 2019. 134 Neuroprotective Effect of Quercetin in Combination with Piperine Against Rotenone and Iron Supplement Induced Parkinson’s Disease in Experimental Rats Shamsher Singh (Moga, India) Objective: (1) Quercetin is well tolerated bioﬂavonoid used as antioxidant and anti-inﬂammatory but foremost problem is its low oral MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S57 ABSTRACTS bioavailability and (2) Piperine in combination quercetin as bioenhancer is target as neuroprotective strategy. Background: Rotenone is a pesticide (neurotoxin) which along with iron at low dose causes destruction of dopaminergic neurons, produces Parkinson’s like manifestations both in human and animals. Quercetin possesses good antioxidant, metal ion scavenger and neuroprotective activity but major complication is its poor oral bioavailability. So to overcome this hindrance quercetin along with bioenhancer piperine is used in rotenone and iron supplement induced neurotoxicity in rats. Methods: Rotenone was administered at a dose of 1.5 mg/kg through intraperitoneal route with iron supplement at a dose of 120μg/g in diet from day 1 to day 28. Pre-treatment with quercetin (25, 50 mg/kg, p.o), piperine (2.5 mg/kg, p.o) alone, quercetin (25 mg/kg, p.o) in combination with piperine (2.5 mg/kg) and ropinirole (0.5 mg/kg, i.p) was given for 28 days 1h prior to rotenone and iron supplement administration. All behavioral parameters were assessed on weekly basis. On 29th day, all animals were sacriﬁced and striatum was isolated for biochemical (LPO, Nitrite, GSH, Mitochondrial complex I, IV), neuroinﬂammatory (TNF-a, IL-1ß and IL-6) and neurotransmitters (dopamine, norepinephrine, serotonin, GABA, glutamate) estimation. Results: The present ﬁnding had showed that chronic quercetin treatment for the 28 days signiﬁcantly ameliorated the rotenone and iron supplement induced motor deﬁcit, biochemical, neuroinﬂammatory and neurochemical alterations in rats. Moreover combination of piperine (2.5 mg/kg) with quercetin (25 mg/kg) signiﬁcantly potentiates the protective effect as compared to quercetin alone treated group. Conclusions: It has been concluded that quercetin and piperine had signiﬁcantly prevented the rotenone and iron supplement induced Fig: 1. Effect of quercetin in combination with piperine on TNF-α, IL- 1β and IL-6 in rotenone and iron supplement treated rats. Data is expressed as Mean S.D represented by columns and bars. ap<0.001 vs NC, bp<0.001 vs Rot, cp<0.001 vs QC (25), dp<0.001 vs QC (50), e p<0.001 vs QC (25) and PP (2.5). Statistical analysis performed by one-way ANOVA followed by Tukey’s post hoc test.NC: Normal control, Rot: Rotenone, Fe: Iron, QC: Quercetin, PP: Piperine, Ropi: Ropinirole Fig: 2. Effect of quercetin in combination with piperine on striatal GABA and Glutamate in rotenone and iron supplement treated rats. Data is expressed as Mean S.D represented by columns and bars. ap<0.001 vs NC, bp<0.001 vs Rot, cp<0.001 vs QC (25), dp<0.001 vs QC (50), e p<0.001 vs QC (25) and PP (2.5). Statistical analysis performed by one-way ANOVA followed by Tukey’s post hoc test.NC: Normal control, Rot: Rotenone, Fe: Iron, QC: Quercetin, PP: Piperine, Ropi: Ropinirole S58 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS behavioral, biochemical and neurological alteration through antioxidant, anti-inﬂammatory and metal ion scavenging properties. References: 1) Patel AB, de Graaf RA, Mason GF, Rothman DL, Shulman RG, Behar KL (2005) The contribution of GABA to glutamate/glutamine cycling and energy metabolism in the rat cortex in vivo. Proc Natl Acad Sci U S A. 102:5588–5593. 2) Singh S, Jamwal S, Kumar P (2017) Neuroprotective potential of Quercetin in combination with piperine against 1-methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridineinduced neurotoxicity. Neural regeneration research 12(7):1137-1144. 135 ’Poised for Parkinson’s’: Alexander Technique Group Course improves Posture, Balance and Mobility for People Living With PD Monika Gross (Candler, NC, United States), Mary McInnis (Moscow, ID, United States), Monica Norcia (San Rafael, CA, United States), Ramyaa Ravichandra (Moscow, ID, United States), Malori Basye (Moscow, ID, United States), Allaa Abdelrahman (Moscow, ID, United States), Rajal Cohen (Moscow, ID, United States) Objective: We tested the feasibility and potential of an Alexander technique (AT)-based group course that included care partners to improve posture, balance, functional mobility, independence, and conﬁdence for people living with Parkinson’s disease (PlwPD). Background: We tested the feasibility and potential of an Alexander technique (AT)-based group course that included care partners to improve posture, balance, functional mobility, independence, and conﬁdence for people living with Parkinson’s disease (PlwPD). Methods: Design: Nonrandomized waitlist-controlled trial. Groups met for 90 min, twice/wk, for 8 wks. Participants: 15 PlwPD and 16 care partners completed the course; 8 PlwPD and 7 care partners were in the control group. Intervention: AT-based coursework included functional anatomy and self-management strategies taught by verbal instruction, hands-on guidance, and activities (individual, partnered, and group). AT principles were embedded in everyday acts such as gait, sit-to-stand, and functional ADLs. Care partners were included to encourage retention. Main Outcome Measures: Balance (Brief BESTest), mobility (7 item Physical Performance Test), symptom-management self-report, anonymous course evaluations, posture photos, and video interviews. Results: Average course attendance was 89% for those who completed the course. Retention was higher for PlwPD with a care partner in the class than for those without one. PlwPD in the AT course objectively improved balance, mobility, and upright posture, while the control group worsened or did not improve. Subjectively, AT course participants reported greater ability to manage shufﬂing, freezing, dyskinesia*, upright posture*, rolling over, and anxiety*, while the control group reported decreased balance conﬁdence*. AT course participants reported they had learned practical self-management tools, enjoyed the social interaction of the group setting, and were likely to remember and use what they had learned in their daily lives. *Signiﬁcant ANOVA. Conclusions: AT training shows promise to improve posture, balance, and self-management of Parkinson’s motor symptoms. Group classes for PlwPD and their care partners can provide cost-effective delivery and additional social beneﬁts. Larger randomized studies are needed to statistically verify symptom management improvement, optimize group delivery, and compare to other approaches, including combining AT group classes with private sessions and combining AT with exercise. (Combining AT with exercise was found to be highly effective in patients with back pain [3].) Three and six-month follow-up data are being collected and will be presented, along with subjective data from care partners about symptom management by their care receivers. References: 1. Stallibrass C, Sissons P, Chalmers C (2002). Randomized controlled trial of the Alexander technique for ideopathic Parkinson’s disease. Clinical Rehabilitation, 16(7):695-708. 2. Stallibrass C, Frank C, Wentworth K (2005). Retention of skills learnt in Alexander technique lessons: 28 people with ideopathic Parkinson’s Disease. Journal of Bodywork and Movement Therapies, 9:2, p. 150-157. 3. Little, P., Lewith, G., Webley, F., Evans, M., Beattie, A., Middleton, K., … & Yardley, L. (2008). Randomised controlled trial of Alexander technique lessons, exercise, and massage (ATEAM) for chronic and recurrent back pain. Bmj, 337, a884. 136 Diphasic Pattern of Worsening of Freezing Gait (FOG) Sahyli Perez Parra (Atlanta, GA, United States), Johnathan McKay (Atlanta, GA, United States), Stewart Factor (Atlanta, GA, United States) Objective: To report 2 cases whereby FOG in Parkinson’s disease (PD) worsened during transition from OFF medication state to ON state, a diphasic pattern, during a levodopa challenge. Background: The relationship between FOG and levodopa response is complex. In some cases, FOG is completely levodopa responsive, others are non-responsive and in others, the levodopa causes FOG. Here we present another scenario. Methods: Series of two cases. Results: 2 PD patients with FOG were examined during the practically deﬁned OFF state, then given a levodopa challenge and examined during the transition to ON (15 & 22 minutes post-dose) and in the ON state (45 & 60 minutes post-dose). All exams were videoed. FOG was measured using MDS-UPDRS-III item 11, Freezing of Gait. Case 1 had serum levodopa levels measured. Both patients experienced worsening of FOG during the transition followed by improvement during the full ON state. Case 1 item 11 scores during standard TUG were 1 OFF state, 2 transition and 0 ON. Serum levodopa levels of were 0.06 ng/mg in the OFF state, 14.78 ng/mg in the transition and 18.98 ng/mg in the ON phase. Case 2 was similar. Conclusions: To our knowledge this diphasic pattern of worsening of FOG has not been previously reported. The cause of worsening of FOG in is unknown but may relate to an inhibitory action of subthreshold levels of levodopa as described by Nutt et al 1988, 1990 where they demonstrated worsening of motor symptoms during levodopa infusions. References: 1. Schaafsma JD1, Balash Y, Gurevich T, Bartels AL, Hausdorff JM, Giladi N. Characterization of freezing of gait subtypes and the response of each to levodopa in Parkinson’s disease. Eur J Neurol. 2003 Jul;10(4):391-8. 2. Espay AJ, Fasano A, van Nuenen BF, Payne MM, Snijders AH, Bloem BR. “On” state freezing of gait in Parkinson disease: a paradoxical levodopa-induced complication. Neurology. 2012 Feb 14;78(7):454-7. 3. J.Lucas McKay, Felicia C. Goldstein, Barbara Sommerfeld, Douglas Bernhard, Sahyli Perez Parra, Stewart A. Factor. Levodopa Responsiveness Subtypes of Freezing of Gait: Results Using a Levodopa Challenge. 137 Blepharoclonus in Parkinson’s Disease: A Meaningful Finding? Nicholas Fleming (Miami, FL, United States), Danielle Shpiner (Miami, FL, United States), Corneliu Luca (Miami, FL, United States), Carlos Singer (Fort Lauderdale, FL, United States), Henry Moore (Boca Raton, FL, United States), Jason Margolesky (Miami, FL, United States) Objective: To assess the prevalence and relevance of blepharoclonus (eye lid ﬂuttering) in patients with Parkinson’s disease (PD). If blepharoclonus is determined to be present in early PD stages, or if present in prodromal PD, then it may be a useful clinical diagnostic biomarker. Background: Blepharoclonus elicited with gentle eyelid closure was reported with parkinsonism in congenital hydrocephalus,1 but has not reported in associated with PD. We have observed sustained eye lid ﬂuttering upon gentle eye closure to be a common phenomenon in PD. Methods: We evaluated 30 randomly selected PD patients, including all ages, disease stages, and disease durations. PD diagnosis was based on MDS diagnostic criteria. 2 We recorded patient’s age, sex, disease duration and Hoehn and Yahr (HY) stage. PD medications and potentially tremorgenic medications were recorded. Blepharoclonus was considered present if eyelid ﬂuttering was sustained for greater then 5 seconds after gentle eye closure. For each patient we completed the MDS-UPDRS part 2, REM Sleep Behavior Disorder Questionnaire (RBDQ), and recorded non motor features including anxiety, depression, anosmia, and constipation. Each patient’s eye lid ﬂuttering was videotaped (examples embedded into poster). Results: Of the 30 PD patients included in our study, 26 (86.6%) had sustained blepharoclonus. Our cohort included 12 (40%) women. Mean age was 66.6 years, ranging from 55 to 86 years. Mean disease duration was 5.4 years. HY stages 1-5 are represented; HY1 (n=4; 13.3%), HY2 (n=21; 70%), HY3 (n=3; 10%), HY4 (n=1; 3.3%), and HY5 (n=1; 3.3%), and blepharoclonus was present in patient’s in HY1 and 5. 9 patients (30%) had RBDQ score >5 (suggestive for presence of MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S59 ABSTRACTS RBD); 8 of 9 (88.8%) of these patients had sustained blepharoclonus. Subjective anosmia/hyposmia, constipation, anxiety and depression were present in 18 (60%), 15 (50%), 12 (40%) and 12 (40%), respectively. Conclusions: Blepharoclonus is prevalent in PD, present in 86.6% of our cohort, including in early PD stages, but it is not yet known if it is a potential prodromal symptom. Non-motor symptoms were present with expected prevalence. Future steps for this research include a case-control paradigm to investigate if sustained blepharoclonus is signiﬁcantly more prevalent in PD patients than controls; additionally, electrophysiologic evaluation of the movement utilizing EMG surface electrodes can be employed to further characterize the phenomena. To further investigate the utility of this ﬁnding as a prodromal diagnostic biomarker, its prevalence could be assessed in patients with idiopathic RBD, incidentally detected in sleep studies in patients yet to develop classic PD motor features. References: Gatto M, Micheli F, Pardal MF. Blepharoclonus and parkinsonism associated with aqueductal stenosis. Mov Disord 1990;5: 310-3 Postuma RB, Berg D, Stern M, Poewe W, Olanow CW, et al. MDS Clinical Diagnostic Criteria for Parkinson’s Disease. Movement Disorders 2015; 30: 1591-99 138 CVT-301 Utilization in Clinical Studies William Ondo (Houston, TX, United States), Marie Saint-Hilaire (Boston, MA, United States), Michael Klingler (Ardsley, NY, United States), Christopher Kenney (Ardsley, NY, United States) Objective: To evaluate for abuse-related adverse events (AEs) and treatment utilization of CVT-301, an inhaled levodopa (LD) therapy for intermittent treatment of OFF episodes in Parkinson’s disease (PD). Background: Dopamine dysregulation syndrome (DDS) is characterized by addictive self-administering LD doses beyond those required to control motor symptoms. CVT-301’s pharmacokinetic proﬁle when compared to oral levodopa raises the theoretical potential to cause more DDS. To investigate product overuse or misuse, we analyzed AEs and treatment utilization in 4 CVT-301 clinical trials. Methods: Patients had PD (Hoehn and Yahr 1-3) and were experiencing OFF episodes. There were 2 placebo-controlled studies with up to 3 months of treatment (CVT-301 group n=270) and 2 studies with 12 months of treatment (CVT-301 group n=583); 1 extension study; 1 safety study with an observational cohort (OC). Patients took CVT-301 60mg, 84mg, or placebo =5 times a day for OFF periods. All patients remained on their baseline oral LD medication. The OC remained on baseline medication with no CVT-301 or placebo. Abuse-related AEs (euphoria-related; impaired attention/cognition/mood; and dissociative/psychotic) for the 4 studies were analyzed together with the number of daily doses, and discontinuations were assessed to describe the patient experience. Results: Most common abuse-related AEs in the =3-month CVT301 group and placebo groups respectively were dizziness (2.2% vs 4.5%), hallucinations (1.5% vs 1.3%), and somnolence (0.7% vs 1.3%). In the 12-month group (CVT-301 vs OC) they were dizziness (2.4% vs 0.8%), hallucinations (2.2% vs 1.6%), and euphoric mood (0.3% vs 0%). Intentional oral LD misuse and DDS were recorded in 1 patient (0.2%) in the CVT-301 12-month group. Abuse-related AEs led to 7 patients discontinuing in the CVT-301 groups (euphoric mood, product misuse, hallucination, visual hallucination, dizziness, DDS). 2 patients (0.7%) in the =3-month and 9 patients (1.5%) in the 12-month CVT-301 groups used =8 doses/day (max permitted=5) on at least 1 day, while mean daily use remained ~2.0 doses/day over 12 months. Conclusions: Incidence of abuse-related AEs for CVT-301 treatment was low and similar to placebo and OC. Overutilization and discontinuation for abuse-related AEs remained low. Therefore, CVT-301 does not appear to increase the risk of overuse beyond the known risks associated with oral LD. CVT-301 is not a controlled substance under the US Controlled Substance Act. 139 Assessing Knowledge and Awareness of Parkinson’s Disease Through a Community Farmer’s Market Lynda Nwabuobi (New York, NY, United States), Elizabeth Delaney (New York, NY, United States), Hiral Shah (New York, NY, United States) S60 Objective: To understand and increase knowledge about Parkinson’s disease (PD) in the ethnically diverse neighborhood of Washington Heights/Inwood (WH/IN) in New York City through a thriving community farmer’s market. Background: Ethnic minorities are disproportionately underrepresented in PD specialist clinics and clinical trials. A poor understanding and awareness of PD is a major barrier to seeking timely care, leading to increased morbidity and poorer quality of life. Methods: Through a collaboration with GrowNYC Greenmarket and American Parkinson Disease Association, we established an informational table at the 175th Street farmer’s market in the WH/IN neighborhood. Tabling occurred on a monthly basis for 2-3 hours in the summer of 2019 and was staffed by a movement disorders specialist and a social worker (Spanish-speaking). Educational materials in English and Spanish included pamphlets on early signs of PD, importance of physical activity, treatment approaches and caregiving. We engaged the market’s attendees, provided educational materials, answered questions and collected a demographic survey. Results: Starting July 2019, we have completed 2 tabling efforts. During each event, 30-40 individuals engaged with the staff and we collected a total of 38 surveys. Participants were mostly women (74%), mainly identiﬁed as Hispanic/Latino (82%) or white (16%), and were above the age of 60 (75%; age range 21-81). 14% of participants had never heard of PD. Many described it as the “shaking” disease or “memory” disease. Some felt it was a part of aging and many attributed its cause to stress. Many participants commented that efforts such as our PD table were necessary to improve knowledge about diseases in ethnically diverse communities. Conclusions: We have had excellent engagement of the community with our program at the community farmer’s market. A neighborhood farmer’s market is a cost-effective, feasible and effective community resource to measure and increase knowledge and awareness about Parkinson’s disease in minority communities. It also mirrors the older demographics of PD patients and creates a relaxed environment that allows for engagement of community members. Most importantly, it creates an ease of access to information about PD by placing movement disorders specialists in the community. References: 1. Willis AW, Schootman M, Evanoff BA, Perlmutter JS, Racette BA. Neurologist care in Parkinson disease: a utilization, outcomes, and survival study. Neurology 2011;77(9):851-857 2. Saadi A, Himmelstein DU, Woolhandler S, Mejia NI. Racial disparities in neurologic health care access and utilization in the United States. Neurology 2017;88(24):2268-2275 140 Barriers to Exercise in Patients with Parkinson’s Disease Prarthana Prakash (San Francisco, CA, United States), Carol Schramke (Pittsburgh, PA, United States), Susan Baser (Murrysville, PA, United States), Timothy Leichliter (Pittsburgh, PA, United States), Thomas Scott (Pittsburgh, PA, United States), Kevin Kelly (Pittsburgh, PA, United States) Objective: To explore issues concerning exercise in patients with PD and speciﬁcally to identify potentially modiﬁable barriers to participating in self directed exercise (SDE), physical therapy (PT), and community based exercise programs (CBP). Background: Exercise has been shown to improve physical functioning, grip strength, balance, walking, health-related quality of life and to help people with PD live independently longer. However, there is limited data on the reported barriers to exercise in PD. Methods: Questionnaires were completed by outpatients with PD from 2/2/18 through 4/18/18 at the Allegheny General Hospital Movement Disorder Clinic and from 11/01/18 through 4/1/19 at the Allegheny Health Network Movement Disorders Clinics. This was a Quality improvement initiative approved by IRB.(Survey uploaded) Descriptive data were summarized using means, standard deviations, and frequencies as appropriate. Based on the reported exercise frequency and duration, patients were classiﬁed as sufﬁcient exercisers (SE) and insufﬁcient exercisers (IE). SE was deﬁned as exercising for at least 150 minutes per week per the AHA guidelines. Comparisons between SE and IE groups were tested using t-tests, chi square, and Mann Whitney U, as appropriate. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Results: Survey was completed by 339 patients. Mean age of patients was 69.19 yrs(s.d.= 8.93). Mean age at PD diagnosis was 62.15 yrs(s.d.=11.22).Data on patient mood ratings, PD severity rating, and health ratings are summarized in Fig 1. 86% of patients indicated that exercise had been recommended. 90% of patients indicated that they believed that exercise is helpful. Referral and participation rates for PT/CBP were closely related. The most frequently reported barriers were low energy (35.6%), physical symptoms (33.3%), and fear of falling (29.8%). Barriers were reported in greater frequencies to SDE and CBP when compared to PT. Fear of falling was associated with a lower rating of mood (p<.001) and increased disease severity ratings (p=.03). Conclusions: This study adds to the limited literature and replicates some prior ﬁndings about barriers to exercise, increases our understanding of how barriers may differ depending on type of exercise, and offers insight into potential interventions that may be beneﬁcial to PD patients. Research aimed at determining the efﬁcacy of brief screening for barriers to exercise and the efﬁcacy of programs designed to address these speciﬁc barriers are reasonable next steps. Although we replicated past research ﬁnding relations between mood, fear of falling, and exercise, additional research designed to investigate whether improving mood symptoms results reduced fear of falling may help us to better understand this factor. References: 1. Terry Ellis, Jennifer K Boudreau, Tamara R DeAngelis, Lisa Brown, James T Cavanaugh, Gammon M Earhart, Matthew P Ford, K Bo Foreman, Leland E Dibble. Barriers to Exercise in People With Parkinson Disease. Phys Ther 2013 May;93®5© 628–6362. M. Afshari, Y. Kianirad, D. Bega. Motivators and barriers to participation in exercise in Parkinson’s disease [abstract]. Mov Disord. 2016; 31 (suppl 2). 141 Patient-Reported Motor Responses to Apomorphine Sublingual Film Based on Home Dosing and Response Diaries Robert Hauser (Tampa, FL, United States), Shyamal Mehta (Scottsdale, AZ, United States), Matthew Maulis (Marlborough, MA, United States), Parul Bhargava (Marlborough, MA, United States), Bradford Navia (Marlborough, MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S61 ABSTRACTS MA, United States), David Blum (Upton, MA, United States), Eric Pappert (Marlborough, MA, United States) Objective: To evaluate the efﬁcacy of self-administered apomorphine sublingual ﬁlm (APL-130277; APL) compared with placebo for the treatment of “OFF” episodes in patients with Parkinson’s disease (PD) based on patient-reported home dosing and response diaries from a pivotal Phase 3 controlled study. Background: We have previously reported that APL is effective for the treatment of “OFF” episodes in a controlled in-ofﬁce setting. Methods: Adult patients with PD and =1 “OFF” episode per day while on stable doses of levodopa/adjunctive PD medications were randomized to 12 weeks of double-blind treatment with placebo or their effective dose of APL (10–35 mg) determined during open-label titration. Patients completed a home dosing and response diary on the 2 days prior to their in-ofﬁce visit at weeks 4, 8, and 12. In the diary, patients recorded the time they self-administered study medication during any “OFF” episode they chose to treat and their resulting motor status (FULL “ON”/“OFF”) 30 minutes later. The percentage of instances in S62 which a patient-reported FULL “ON” response was achieved at 30 minutes postdose at week 12 was assessed using a mixed model for repeated measures and was a secondary endpoint. Additional post hoc analyses were assessed descriptively. Results: One hundred nine patients were randomized (APL: n=54; placebo: n=55). Patients self-administered a mean of 2.2 and 2.5 daily doses of APL and placebo, respectively. At week 12, APL treatment was associated with a greater percentage of instances in which patients reported a FULL “ON” response at 30 minutes postdose within the 2 days prior to their in-ofﬁce visit compared with placebo (79% vs 31%; nominal P<0.0001). Across all diary entries, the percentage of instances in which patients achieved a self-reported FULL “ON” response at 30 minutes postdose was 77% with APL and 26% with placebo. Patients self-reported a FULL “ON” response at a similar rate across all periods of the day, ranging from 71% (5 AM to 9 AM) to 86% (10 PM to 5 AM). Conclusions: Based on home dosing and response diaries, the selfadministration of APL may lead to a FULL “ON” response in most patients experiencing “OFF” episodes throughout the day. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS 142 Possible Neuroprotective Propensity of Bioactive Natural Phenolic Acids Combinational Strategy Against Rotenone Induced Parkinsonism in Rodents Ekta Yadav (Prayagraj, India) Objective: The current study was envisaged to explore the synergistic therapeutic effect of phytophenolic acids, i.e., protocatechuic acid (PA) and cholorogenic acid (CGA), combination (PA+CGA) therapy in rotenone induced cognitive, neurotransmitter, mitochondrial as well as oxidative dysfuntion in mouse model of parkinsonism. Background: PA has been found to possess signiﬁcant protective effect on PC12 cells against apoptosis induced by 1-methyl4-phenylpyridinium and hydrogen peroxide as well as rotenone-induced mitochondrial functional damage. Additionally, CGA has anti-parkinson’s potential against 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine in rodents by regulating tyrosine hydroxylase -positive dopaminergic neurons striatal neurotransmitters level. Methods: Mice were randomly grouped in ﬁve groups. (PA+ CGA) combination (10, 20 and 40 mg/kg, p.o.) accompanied with rotenone (1 mg/kg i.p.) injection, saline and rotenone (1 mg/kg i.p.) alone were administered in respective group for 21 days. Rotenone treated group exhibited signiﬁcant impairment in memory and learning behavior, and locomotor activity. Additionally, reduction in enzymatic antioxidant level such as superoxide dismutase (SOD), catalase (CAT) and glutathione peroxide level (GPx), acetylcholinesterase (AChE) and dopamine levels as well as mitochondrial dysfunction via decrease in Complex-II and Complex- III (Succinate Dehydrogenase, MTT (3- (4,5-dimethylthiazol2-yl)-2,5-diphenyl-Htetrazolium bromide, respectively) enzyme activity in mice brain as compared to control group. Results: Results from behavioral test as well as biochemical estimation demonstrated that pretreatment with (PA+CGA) combination signiﬁcantly reversed the rotenone induced damaging effect as compared to rotenone group in a dose dependant manner. Conclusions: Considering the data of current ﬁnding concludes the neuroprotective effect of (PA+CGA) combination towards rotenone induced Parkinson’s in rodents and it may be utilized as a beneﬁcial therapeutic tool for the Parkinson’s disease management. 143 Golgi apparatus. It also acts in maintaining intracellular Ca2+ homeostasis. These functions could potentially be relevant to pathogenesis of gait disorders in PD. These results require replication. 144 Emergence of Speech and Language Symptoms in Stages of Parkinson’s Disease: Study Conducted in a Group of People with Parkinson’s in Venezuela Alejandro Cano Villagrasa (Valencia, Spain), Martha Suárez Torres (Caracas, Venezuela), Beatriz Valles-González (Valencia, Spain) Objective: To know the clinical-epidemiological proﬁle of PwP in its different stages (I, II, III, IV and V), according to the Hoehn and Yahr (HY) scale; and compare the SLS that the PwP present, according to the PDS in which they are. Background: People with PD (PwP) present speech and language symptoms (SLS) such as limitations in vocal, articulatory and deglutory functions, which can vary and intensify depending on the PD scale (PDS), and have an impact on their quality of life (Suárez, VallesGonzález & Cano, 2019). Methods: The sample consisted of 34 patients diagnosed with idiopathic PD (26 men and 8 women) treated in a speech therapy department. Their 34 clinical histories were analyzed and the data corresponding to the ﬁrst comprehensive evaluation of each patient, contained in the medical and speech and language reports, were studied in order to know the epidemiological clinical proﬁle of each person with PD. Subsequently, the different SLS present in each PDS were identiﬁed and compared. The study followed a quasi-experimental cross-sectional design, where the different SLS were analyzed using an ANOVA analysis of a factor, in order to compare the SLS in the different PDS. Results: The PwP were aged between 25 and 86 years. The majority (47%) were in an advanced stage of PD. As for the PDS, they were distributed as follows: I 11.7%; II 17.6%; III 47%; IV 14.7%; V 8.8%. The SLS that were identiﬁed in each PDS were: I hypophony 75%, dysprosodia 50%, dysarthria and dysphagia 0%; II 50% hypophony, 100% dysprosodia, 50% dysarthria, 16.7% dysphagia; III: 75% hypophony, 87.5% dysprosodia, 37.5% dysarthria, 12.5% dysphagia; IV: 100% hypophony, 100% dysprosodia, 40% dysarthria, 60% dysphagia; V: 100% hypophony, dysprosodia, dysarthria and 100% dysphagia. An adequate level Is There a Freezing of gait Gene in Parkinson’s disease (PD)? Zachary Wallen (Birmingham, AL, United States), Erin Hill-Burns (Birmingham, AL, United States), Eric Molho (Albany, NY, United States), Cyrus Zabetian (Seattle, WA, United States), Haydeh Payami (Birmingham, AL, United States), Stewart Factor (Atlanta, GA, United States) Objective: To perform a genome wide association study (GWAS) to identify susceptibility for freezing of gait (FOG) in PD. Background: Freezing of Gait (FOG) is a highly variable feature of PD that is disabling, a major contributor to falling with injury, untreatable with dopaminergic drugs and has a signiﬁcant impact on quality of life. It is thought to be appear and progress independently from other cardinal features of PD. Methods: This study included 672 individuals with PD (mean duration of disease 8.06.2 years). GWAS of FOG UPDRS part II item 14 scores adjusted for age and disease duration were performed using linear regression. FOG score, age, and disease duration were treated as quantitative traits. SNP genotypes were modeled as additive (dosages of the minor allele from 0 to 2 copies). The effect size (ß) corresponds to the change in UPDRS item score for every copy of the minor allele. Pvalues were calculated to assess signiﬁcance of ß. Signiﬁcance was set at P < 5E-8. A total of 8.5 million genotyped and imputed SNPs were analyzed. Results: Our study revealed a genome wide signiﬁcant signal for freezing at P=3E-8, for SNP rs113905089 on chromosome 1, located in an intron of SEC16B gene. Each copy of the rs113905089 minor allele was associated with 0.84 [0.5 – 1.1] increase in UPDRS item 14 score. Conclusions: This is the ﬁrst report of a gene associated speciﬁcally with FOG. The SEC16B gene codes for a scaffold protein involved in selective cargo export from the transitional endoplasmic reticulum to MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S63 ABSTRACTS of signiﬁcance was observed for the hypophony (F=3.898, p=0.033), dysprosodia (F=4.055, p=0.013), dysarthria (F=3.133, p=0.020) and dysphagia (F=5.431, p=0.002). Conclusions: PwP show SLS from the ﬁrst PDS, these symptoms are prone to worsen and become more complex as the PD progresses, such as with the onset of stage III dysphagia, which implies a decrease in the patient’s quality of life and creates the need for a comprehensive speech and language approach since the diagnosis of PD (Miller, Noble, Jones & Burn, 2006). References: Hoehn, M. M., & Yahr, M. D. (1967). Parkinsonism: onset, progression and mortality. Neurology, 17, 427-442. Miller, N., Noble, E., Jones, D., & Burn, D. (2006). Life with communication changes in Parkinson’s disease. Age and Ageing, 35, 235–239. Suárez, M., Valles-González, B., & Cano, A. (2019). Relationship between speech, voice and swallowing disorders and non-motor symptoms in Parkinson’s disease. A study conducted in a group of people with PD in Venezuela. Presented at the Posters Tour Session of 5 World Parkinson´ s Congress. Kyoto, Japan: World Parkinson Coalition. most prevalent from the early stages of PD. Studies have found associations between fatigue and depression, but the results are still unclear. Methods: Eighty-nine PD patients (mean age=69.64 and education years=10.84) underwent a clinical evaluation assessing fatigue and depressive symptoms and QoL with the Fatigue Severity Scale (FSS), Hospital Anxiety and Depression Scale (HADS) and Satisfaction With Life scale, respectively. Rho Spearman test was used to assess the relationship between the three domains. The FSS and HAD scales were adjusted, indicating that the higher the score, the lower the symptoms. Two lineal regression analyses were used to investigate the mediation between fatigue, depression and QoL in PD patients. The nonparametric bootstrapping strategy was employed to calculate effect size of the mediation. Results: Signiﬁcant correlations were found between fatigue, depressive symptoms and QoL in PD, showing that the higher depressive and fatigue symptoms, the lower QoL. The ﬁrst mediation revealed that fatigue was signiﬁcantly associated with QoL (ß =.35; p<.001) and with depressive symptoms (ß =.24; p<.023) in PD. The effect of fatigue on QoL was reduced when depressive symptoms were introduced as a mediating variable (ß =.25; p<.009), explaining 30.77% of the variance [Figure 1]. Boostraping strategy showed that the indirect effect is statistically signiﬁcant. The second mediation analysis showed that the effect of depressive symptoms on QoL was reduced explaining 14.28% of the variance, when fatigue was introduced as a mediating variable [Figure 2]. Conclusions: To our knowledge, this is the ﬁrst study assessing the mediation between depressive symptoms with fatigue symptoms and QoL in PD. Findings suggest that depressive symptoms are directly involved in the relationship between fatigue and QoL in patients with PD. Therefore, the intervention of depressive symptoms could reduce fatigue and improve the QoL in PD patients impacting on their daily lives and functionality. 145 The Impact of Fatigue and Depressive Symptoms in the Quality of life in Parkinson’s Disease Itsasne Sanchez-Luengos (Bilbao, Spain), Olaia Lucas-Jiménez (Bilbao, Spain), Javier Peña (Bilbao, Spain), Natalia Ojeda (Bilbao, Spain), Juan Carlos Gomez (Barakaldo, Spain), María- Angeles Gómez-Beldarrain (Bilbao, Spain), Raquel Vazquez-Picon (Bilbao, Spain), Nerea Foncea-Beti (Bilbao, Spain), Naroa Ibarretxe-Bilbao (Bilbao, Spain) Objective: Little is known about the speciﬁc impact of fatigue and depression on the quality of life (QoL) of Parkinson’s disease (PD) patients. The aim of the study was to analyze the mediating role of depressive symptoms in the relationship between fatigue and QoL. Background: The QoL in PD may be affected by mood disturbances and clinical symptoms, with fatigue and depressive symptoms being the S64 Figure 1: Effect of depressive symptoms on the relationship between fatigue symptoms and the quality of life.* p < .05; ** p < .001B= Non-standardized regression coefﬁcient; SE= Standard error MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Poewe (Innsbruck, Austria), Olivier Rascol (Toulouse, France), Andrew Lees (London, United Kingdom), Joaquim Ferreira (Torres Vedras, Portugal), Kurt Olson (Del Mar, CA, United States), Khodayar Farahmand (San Diego, CA, United States), Jose-Francisco Rocha (S. Mamede Coronado, Portugal), Patrício Soares-da-Silva (Porto, Portugal) Figure 2: Effect of fatigue symptoms on the relationship between depressive symptoms and the quality of life. * p < .05; ** p < .001B= Non-standardized regression coefﬁcient; SE= Standard error 146 Motor Responses to Apomorphine Sublingual Film Compared With Levodopa in Patients With Parkinson’s Disease and “OFF” Episodes Jennifer Hui (Los Angeles, CA, United States), Susan Fox (Toronto, ON, Canada), William Neeson (Mississauga, ON, Canada), Parul Bhargava (Marlborough, MA, United States), Eric Pappert (Marlborough, MA, United States), David Blum (Upton, MA, United States), Bradford Navia (Marlborough, MA, United States) Objective: To compare time to onset, duration, and magnitude of motor improvement following administration of apomorphine sublingual ﬁlm (APL-130277; APL) vs levodopa in patients with Parkinson’s disease (PD) and “OFF” episodes. Background: It has previously been shown that onset of motor improvement occurred faster with subcutaneous apomorphine compared with levodopa, with a similar magnitude of response. Methods: Adult patients with PD and “OFF” episodes receiving levodopa were enrolled. At screening, patients in an “OFF” state were observed for a FULL “ON” response following their usual morning levodopa dose. During open-label titration, patients received increasing doses of APL (10–35 mg) until a FULL “ON” response was achieved without intolerable side effects. Motor responses following open-label APL and levodopa administration were compared by descriptive statistics using the Movement Disorder Society Uniﬁed Parkinson’s Disease Rating Scale (MDS-UPDRS) Part III score assessed predose, and 15, 30, 45, 60, and 90 minutes postdose. Results: One hundred nine patients treated with APL and levodopa were analyzed (levodopa data were missing for 1 patient). Predose MDS-UPDRS Part III scores were comparable at screening and titration (43.5 vs 43.1). At 15 minutes postdose, the magnitude of motor response with APL was ~2-fold higher than with levodopa (–12.6 vs –6.0). Peak response to APL occurred earlier (45 minutes) than with levodopa (90 minutes) across the timepoints measured, and the magnitude of peak responses was comparable (–26.1 vs –27.9). Notably, APL was still associated with a clinically meaningful improvement at 90 minutes postdose (–19.9). Responder rates (deﬁned as a =30% decrease in MDS-UPDRS Part III score from predose) were greater for APL compared with levodopa over the ﬁrst 45 minutes. At 90 minutes postdose, 63% of patients receiving APL were still considered responders. Conclusions: APL was associated with an earlier onset of motor improvement and earlier peak response vs levodopa, while the magnitude of peak response was similar in patients with PD and “OFF” episodes. APL continued to provide a clinically meaningful improvement in MDSUPDRS Part III score at 90 minutes postdose, suggesting that it may offer a durable on-demand treatment for “OFF” episodes. Additional studies are warranted to validate the ﬁndings from this open-label, post hoc analysis. 147 Efﬁcacy and Safety of Once-Daily Opicapone 50 mg in Patients with Parkinson’s Disease and Motor Fluctuations: Pooled Analysis of Two Randomized, Double-Blind, PlaceboControlled Studies Robert Hauser (Tampa, FL, United States), Mark Lew (Los Angeles, CA, United States), Daniel Kremens (Wynnewood, PA, United States), Werner Objective: To evaluate the efﬁcacy and safety of once-daily opicapone (OPC) in adults with Parkinson’s disease (PD) and motor ﬂuctuations. Background: OPC is a highly selective once-daily catechol-Omethyltransferase inhibitor (COMTI) under development in the United States as an adjunct to levodopa for PD ﬂuctuations. Data from 2 doubleblind (DB), placebo (PBO)-controlled, pivotal Phase 3 studies (BIPARK-1 [NCT01568073], BIPARK-2[NCT01227655]) [1,2] were pooled to evaluate the efﬁcacy and safety of OPC 50mg (targeted therapeutic dose) in PD patients with motor ﬂuctuations on a stable levodopa regimen. Methods: BIPARK-1 and BIPARK-2 participants were randomized to DB treatment with OPC or PBO added to their levodopa for 14-15 weeks. Least squares mean (LSM) changes from baseline to Week 14/15 in absolute OFF-time (primary endpoint, both studies) were analyzed using a mixed model for repeated measures in the pooled Full Analysis Set (FAS) and in subgroups based on concurrent PD medication use at baseline (dopamine agonists [DA], monoamine oxidase-B inhibitors [MAOBI]). Treatment-emergent adverse events (TEAEs) and adverse events of special interest (AESIs) were assessed in the pooled safety population. Results: In the pooled FAS (PBO=255; 50mg=262), LSM changes (standard error [SE], hrs) in OFF-time were as follows: PBO, 1.280.17; 50mg, 2.220.17 (P<0.001). In exploratory subgroups, PBO-corrected LSM changes (SE, hrs) for OPC 50mg were as follows: DA (yes, 0.930.26; no, 1.010.44), MAOBI (yes, 1.140.50; no, 0.900.25); all P<0.05 vs PBO. In the pooled safety population (PBO=257; 50mg=265), TEAEs reported in >5% of participants receiving OPC 50mg and at higher incidence than PBO were dyskinesia (20.4% vs 6.2%) and constipation (6.4% vs 1.9%). The most common AESI with OPC 50mg, other than dyskinesia, was increased blood creatine phosphokinase (4.9% vs 1.9% for PBO); all other AESIs occurred in =3% of participants taking OPC 50mg. Conclusions: Adding once-daily OPC 50mg to a levodopa regimen signiﬁcantly reduced OFF-time compared to PBO, with similar improvements observed in patients taking concurrent DAs or MAOBIs. OPC was generally well tolerated in patients with PD and motor ﬂuctuations. TEAEs reported with other COMTIs, such as diarrhea and elevated liver function enzymes, were uncommon and similar to PBO rates. References: 1. Ferreira JJ, Lees A, Rocha JF, et al. Lancet Neurol. 2016;15:154-65. 2. Lees AJ, Ferreira J, Rascol O, et al. JAMA Neurol. 2017;74:197-206. 148 Comparison of Selected Non-Motor Symptoms Between PD Subtypes: Tremor Dominant vs Postural Instability/Gait Difﬁculty Groups Shyamal Mehta (Scottsdale, AZ, United States), Nan Zhang (Scottsdale, United States), Molly Knox (Scottsdale, AZ, United States), Holly Shill (Phoenix, AZ, United States), Erika Driver Dunckley (Scottsdale, AZ, United States), Kathryn Davis (Sun City, United States), Thomas Beach (Sun City, AZ, United States), Charles Adler (Scottsdale, AZ, United States) Objective: To assess if there are any differences in the non-motor symptoms (NMS), such as autonomic symptoms, smell and sleep, between two subtypes of PD: Tremor Dominant (TD) vs Postural Instability/Gait Difﬁculty (PIGD). Background: PD has often been divided into two clinical phenotypes: TD and PIGD. While NMS has been studied in PD based on early vs late disease onset, we have assessed NMS (autonomic symptoms (SCOPA-Aut), smell (UPSIT) and sleep (MSQ and ESS)) based on the above clinical phenotypes in clinico-pathologically conﬁrmed PD patients. Methods: 1471 subjects who had baseline clinical evaluations and had come to autopsy in the Arizona Study of Aging and Neurodegenerative Disorders (AZSAND) were reviewed. Of these, 387 control and MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S65 ABSTRACTS 112 PD subjects were included for analysis. Criteria outlined by Stebbins et al.1 were used to dichotomize the PD patients into TD (n=45) and PIGD (n=57) groups. General linear regression (for continuous outcomes) and logistic regression (for binary outcomes), adjusted for age and gender, were used to compare the outcomes among the three groups. Pairwise comparisons using Bonferroni correction for multiple testing was further carried out if the overall group effect was signiﬁcant at 0.05 level for each outcome. Results: There were no differences in the Total SCOPA-Aut between the TD (20.22) vs PIGD groups (25.03) (p= 0.137) but both groups had higher scores as compared to controls (6.24). An analysis of the sub-sections of SCOPA-Aut showed signiﬁcantly higher scores in PIGD (25.83) vs. TD (17.71) groups (p=0.003) in the SCOPA-Aut GI section but no signiﬁcant difference in the rest of the sub-sections. The Mayo Sleep Questionnaire showed no difference between the 2 PD groups for Epworth Sleepiness Scale (p=0.40) and dream enactment behavior (p=1.0). The mean UPSIT scores were 17.88 (PIGD) vs. 19.68 (TD), showing no difference in smell dysfunction between the two groups and controls had normal scores at 29.27. Both, PIGD and TD groups had statistically lower UPSIT scores as compared to controls (p<0.0001). Conclusions: No differences in smell, excessive daytime sleepiness or dream enactment behavior was found between the TD vs PIGD subtypes of PD. PD subjects in the PIGD group endorsed signiﬁcantly more GI symptoms as compared to the TD group with no differences in other autonomic domains assessed by SCOPA-Aut. While NMS are more frequent in PD than controls, the different clinical phenotypes of PD show little difference in NMS. References: Stebbins GT, Goetz CG, Burn DJ, Jankovic J, Khoo TK, Tilley BC. How to identify tremor dominant and postural instability/gait difﬁculty groups with the movement disorder society uniﬁed Parkinson’s disease rating scale: comparison with the uniﬁed Parkinson’s disease rating scale. Mov Disord. 2013;28(5):668-70. 149 How Parkinson’s Disease Affects Value-Based Decisions Leila Montaser Kouhsari (New York, NY, United States), Akram Bakkour (New York, United States), Raphael Gerraty (New York, NY, United States), Daphna Shohamy (New York, NY, United States) Objective: To determine: 1. Whether Parkinson’s disease (PD) patients show behavioral differences during value-based decisions compared to healthy controls, and 2. whether these differences relate to the dopaminergic state. Background: Deciding between familiar options of similar value is time-consuming. These decisions may depend on accumulating evidence over time. Recent studies show that accumulation of evidence can be via memory input and the coupling strength between hippocampus and striatum correlates with reaction time (RT). PD is a degenerative motor disease causing subtle deﬁcits in decision-making as a result of dopamine depletion of the basal ganglia. However, the mechanism by which dopamine might affect value-based decisions remains unknown. Methods: Behavioral experiments were conducted in individuals with PD on and off dopaminergic medication, as well as age-matched healthy controls. Participants performed a value-based decision task and made choices between pairs of familiar food items. The value of each item was determined in advance using a separate food-rating task. The same participants also performed a control condition where they made perceptual decisions about the color of random dynamic dots with different coherence on each trial. The RT and choice performance in both tasks were measured and ﬁtted with a drift-diffusion model (DDM) when patients were on and off dopaminergic medication. Results: Our results reveal that patients off-medication are faster than healthy controls (HCs) in value-based but not in perceptual decisionmaking while both PD patients and HCs are expected to show similar model ﬁts, accuracies, and RTs in the perceptual task. Furthermore, PD patients’ RTs are longer and not signiﬁcantly different from HCs when on-medication, indicating that their accumulation evidence is comparable to HCs. Conclusions: The accumulation of evidence is less when PD patients are off-medication compared to when they are on-medication indicating S66 a decision process that is less deliberative than HCs but retains similar accuracies. The dopaminergic medication increases the accumulation of evidence. We propose that dopaminergic medication helps the process of accumulation of evidence by strengthening the coupling between striatum and hippocampus. 150 Fabrication, Characterization of PLGA Loaded Nanoformulation of Umbelliferone Exert 6-hydroxydopamine Induced Parkinson’s Disease via Alteration of Genetic Backgrounds Vikas Kumar (Allahabad, India), Firoz Anwar (Jeddah, Saudi Arabia) Objective: In the current study, we prepare and examine the nanoherbaceutical formulation of umbelliferone against 6-hidroxidopamine (6-OHDA) induced PD animal model. Background: Parkinson’s disease (PD) is a common age related neuro-degenerative disease induced via interaction between the various factors including oxidative stress, genetic toxins, mitochondrial deformity and aging. PD, distressing the dopaminergic neuronal, resultant loss of nigrostriatal system, motor dysfunction and impairment of microglial cells. Presently, no effective treatment available for the PD patients but some evidence suggests that the antioxidant rich food may alter the incidence of neurodegenerative disease. Methods: Tap down method was used for the fabrication of PLGA loaded nanoparticle of umbelliferone. Intracerebroventricular administration of 6-OHDA was used for induction the PD and the animal divided into different groups and orally received the PLGA-UF-NPs for 28 days. The enzymatic activities viz., glutathione peroxidase (GPx), superoxide dismutase (SOD), Catalase (CAT), glutathione S-transferase (GST), glutathione (GSH) and reactive oxygen species (ROS), total reactive antioxidant dopamine and its metabolites homovanillic and 3,4-dihydroxyphenylacetic acid, were estimated in striatum. Behavioral parameters including memory, locomotor and depressive were also scrutinized, respectively. Results: The result clearly exhibited that PLGA-UF-NPs treatment was efﬁcient in prevention the memory destruction in depressive like behavior and Morris water maze test. PLGA-UF-NPs altered the GPx (45.5%), SOD (57.6%), CAT (64.5%), GST (64.5%), GSH (58.7%), GR (56.5%), ROS (54.8%) and its metabolite level in striatum. Conclusions: On the basis of result, we can say that PLGA-UF-NPs exhibited the protective effect against 6-OHDA induced neurotoxicity in animal, and suggesting as the alternate therapy for PD treatment. 151 Delay in Diagnosis of Parkinson’s Disease: Who is to Blame? Cynthia Sarabia-Tapia (Mexico City, Mexico), Etienne Resendiz-Henriquez (Mexico City, Mexico), Susana Lopez-Alamillo (Villahermosa, Mexico), Amin Cervantes (Mexico City, Mexico), Mayela Rodriguez Violante (Mexico City, Mexico), Oscar Esquivel-Zapata (Monterrey, Mexico), Yazmin Rios (Ciudad de Mexico, Mexico), Rodolfo Abundes-Corona (Mexico City, Mexico), Omar Cardenas (Ciudad de Mexico, Mexico), Emmanuel Escobar (Monterrey, Mexico), Fanny Herrera-Rodríguez (Mexico City, Mexico) Objective: Identify the most common causes of delay in the diagnosis of the disease. Background: Worldwide, an estimated of 25 cases per 100,000 individuals are diagnosed with Parkinson’s disease (PD) each year; in Mexico it is about 10 cases per 100,000 individuals, with a peak age onset in the seventh decade, mostly affecting males. Some symptoms such as hyposmia, constipation and sleeping disorders can be present even decades before the onset of global bradykinesia, tremor or rigidity, which remain the cardinal symptoms of the disease. The average time from the onset of motor symptoms to the diagnosis of PD is about 12 months; 11 months for patients to recognize their symptoms and ﬁnally seek for medical advisory and around a month for health-care-professionals (HCP) to diagnose PD. Methods: We randomly selected 112 patients with PD for this study between July and August 2019. We performed a semi-structured interview following a questionnaire with 51 variables targeting to obtain a series of sociodemographic data, PD medical history and information regarding their recognition of the onset of symptoms and their journey MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS to seeking for medical advisory. We considered both non-motor and motor-symptoms as part of the onset of PD. Age of onset was deﬁned as the age at which behavioral, cognitive, psychiatric ormotor-symptoms were ﬁrst described. PD diagnosis was established based on the UK Parkinson’s Disease Society Brain Bank Diagnostic Criteria. Results: We interviewed 67 men and 45 women; the average age was 65 years old. About half of them had no social security. 52% had previous knowledge of PD. Almost 90% were able to auto-identify the onset of symptoms, being tremor the most common symptom and the patients’ main reason for seeking medical advisory. Around 27% expected their symptoms to spontaneously resolve before looking for any kind of advisory. An estimated of 18.5 months elapsed between the identiﬁcation of the symptoms and the ﬁrst visit to a HCP, mostly in public health institutions. General medicine practitioners were the most consulted ones and it took an almost 22 months to diagnose PD. The average age was 57.8 years old at the time of the diagnosis. Most of the patients considered this time as “good”, while the ones who considered it as “bad” thought the delay was due to erroneous medical diagnoses. The average time from the identiﬁcation of onset syptoms to diagnosis of PD is 39.3 months. Conclusions: Both patients and HCP play a major role in the delay of diagnosis of PD. It took three times more than the estimated time for our patients to reach the diagnosis. In Mexico, mostly general medicine practitioners are in charge of early diagnosis and prevention of a wide variety of diseases, however almost half of our patients had no access to MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S67 ABSTRACTS any social security. Although most of our patients reported having previous knowledge of PD, the fact that it takes about 6 months more than the estimated time to seek for medical advisory reﬂects the lack of information available for our patients, and while they do not think of this time as a real delay, it takes almost 2 years for HCP to diagnose PD. Promotion of PD should focus on the early recognition of symptoms for both the patients and HCP in order to prevent delayment. References: 1. Plouvier AOA, et al. Time intervals in diagnosing Parkinson’s disease: The patients’ views. Patient Educ Couns (2015).2. Poewe W, et al. Parkinson disease. Nat. Rev. Dis. Primers 3, 17013 (2017).3. Nancy Jane Y, et al. A Q-back propagated time delay neural network for diagnosing severity of gait disturbances in Parkinson’s disease. J Biomed Inform (2016). 4. [Internet]. International Parkinson and Movement Disorder Society. 2019. Available at: https://www. movementdisorders.org/MDS.htm5. [Internet]. Dirección General de Epidemiología. Anuario de Morbilidad 1984 - 2018. 2018. Available at: http:// www.epidemiologia.salud.gob.mx/anuario/2018/incidencia/enfermedad_ grupo_edad_entidad_federativa/170.pdf 6. Wan Y, et al. Determinants of diagnostic latency in Chinese people with Parkinson’s disease. BMC Neurology (2019).7. Breen DP, et al. Determinants of delayed diagnosis in Parkinson’s disease. J Neurol (2013). 152 Anxiety Disorders in Parkinson’s Disease: Validation of a New Scale and Evaluation of Endocannabinoids in a Brazilian Outpatient Clinic Jeane Fonseca Vieira (Ribeir~ ao Preto, Brazil), Nayanne Bosaipo (Ribeir~ ao Preto - SP, Brazil), Camila Marchioni (Ribeir~ ao Preto, Brazil), Maria Nassur (Ribeir~ ao Preto, Brazil), Itiana Menezes (Ribeir~ ao Preto, Brazil), Larissa Sanches Serveli (Ribeir~ ao Preto, Brazil), Camila Pessini Campanini (Ribeir~ ao Preto, Brazil), Angela Pimentel (Ribeirao Preto, Brazil), Manuelina Brito (Ribeirao Preto, Brazil), Vitor Tumas (Sao Paulo, Brazil) Objective: We investigated the association between anxiety disorders in PD with endocannabinoids (eCB) and validated the Parkinson Anxiety Scale (PAS, Leentjens et al. 2014). Background: Neurobiological changes in PD as changes in eCBs related to anxiety may be associated with PD (Pisani et al. 2011). In addition, dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis present in PD could inﬂuence the interactions with the already affected eCB system in a neuropsychiatric condition (Hillard et al. 2018). S68 Methods: Participated 100 volunteers with diagnosed idiopathic PD (non-dementia), for the validation of self-rated version of PAS. Of these sample, 40 were divided into with anxiety (n = 20) and non-anxiety (n = 20) groups. Participants were assessed for PD and psychiatric symptoms and cognitive aspects. For the plasma quantiﬁcation of eCBs (anandamide and 2-AG) fasting morning blood samples were collected. Two salivary cortisol samples (22h; 8h) were analyzed for HPA axis activity evaluation. Results: The scale SR-PAS demonstrated data of good quality and high reliability (Cronbach’s alpha values =0.7). Concerning eCBs, no difference was found between the groups and no association with anxiety disorders. However, most patients have loss of functionality of the circadian rhythm of cortisol. Conclusions: Our results demonstrated a clinical use of SR-PAS. Despite limitations, currently available data measuring circulating eCBs served as a biomarker for aspects of vulnerability to stress-induced sequelae (Hillard et al. 2012). However, no relationship was found between anxiety disorders and plasmatic eCBs in our study. It has been suggested that changes in the circadian rhythm of the HPA axis may have inﬂuenced the eCBs. This dysfunction would further impair PD neurobiology and may have made it more difﬁcult to detect plasma levels of eCBs. References: Hillard et al. Contributions of endocannabinoid signaling to psychiatric disorders in humans: genetic and biochemical evidence. Neuroscience, 204: p. 207–299, 2012. Hillard et al. Endocannabinoid Signaling and the Hypothalamic-Pituitary-Adrenal Axis. Compr Physiol.; 7(1): 1–15, 2018. Leentjens et al. The Parkinson Anxiety Scale (PAS): Development and Validation of a New Anxiety Scale. Mov Disord, v.29, n.8,p. 1035-1043, 2014. Pisani et al. Homeostatic Changes of the Endocannabinoid System in Parkinson’s Disease. Mov Disord, v. 26, n. 2, p. 216-222, 2011. 153 Medical Care Associated with Weekly Physical Therapy Delivered to Patient’s Groups can Maintain the Health-Related Quality of Life over Two Years Regardless of the Increase in the Severity of Parkinson’s Disease Fernanda Miyahara (S~ ao Paulo, Brazil), Erica Neves Guelﬁ (Sao Paulo, Brazil), Erika Okamoto (S~ ao Paulo, Brazil), Pâmela Barbosa (S~ ao Paulo, Brazil), Maria Elisa Piemonte (Sao Paulo, Brazil) Objective: The aim of this study was to investigate the long-term results of a weekly physical therapy program delivery for patient’s group MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS on disease severity, independence in daily living activities and quality of life of people living with PD. Background: Regardless of the best pharmacological intervention, the progression of the neurodegenerative process of Parkinson’s disease (PD), leads to worsening of the motor and non-motor symptoms. The increased severity of symptoms increases the level of deﬁciency and incapacity and consequently, decreases the quality of life of the people living with Parkinson disease. Physical therapy has been considering as a powerful adjuvant for pharmacological treatment to manage the motor symptoms and decrease the level of deﬁciency and incapacity. However, key points for the cost-beneﬁts ratio as the minimal dosage, delivery way for long term results remain uncertain. Methods: Participated this study 54 people with conﬁrmed diagnostic of idiopathic PD, 4 instage 1, 23 in stage 2, 22 in stage 3 of disease progression according to Hoehn and Yahr Classiﬁcation, with 66.72 (SD = 8.9) years of age and 10.16 (SD=4.5) years of education, under levodopa reposition treatment. All participants received usual medical care and weekly physical therapy care delivery for groups with 8 patients with a similar level of deﬁciency. Besides physical therapy, participants were stimulated to make additional physical activities at home. The independence in daily living activities, motor symptoms severity and global disease severity were evaluated by section II, section III and total score of Uniﬁed Parkinson’s Disease Rating Scale (UPDRS) respectively; and Health quality of life was evaluated by Parkinson´s disease questionnaire 39 (PDQ-39). All outcomes were evaluated in 3 annual individual sessions: at beginning of study (E1); at one year after (E2) and at two years after (E3). The results obtained in the three annual evaluations were analyzed by ANOVA for repeated measures. Results: ANOVA showed a signiﬁcant increase in total scores (p=.007, ES=.85), in section II(p=.002; ES=.91) and in section III (p=.002; ES=.75) in UPDRS over two years, conﬁrmed by Tukey posthoc test. However, ANOVA showed no statistically signiﬁcant effect of time for scores in PDQ-39. Conclusions: Medical care associated with physical therapy delivered in weekly group sessionswas able to remain the health quality of life regardless of the worsening in disease severity over two years. The physical therapy delivered in weekly group sessions can be considered a strategy with positive cost-beneﬁt ration to keep the health quality of life of people living with PD. References: Bugalho, P. et al. Non-Motor symptoms in Portuguese Parkinson’s Disease patients: correlation and impact on Quality of Life and Activities of Daily Living. Sci. Rep. vol 6, august 2016. 154 BouNDless: An Active-Controlled Randomized, Double-Blind Double-Dummy Trial of Continuous Levodopa Delivery (ND0612) in Patients with Parkinson’s Disease Experiencing Motor Fluctuations Alberto Espay (Cincinnati, OH, United States), Karl Kieburtz (Rochester, NY, United States), Sheila Oren (Rehovot, Israel), Tami Yardeni (Rehovot, Israel), Liat Adar (Ramat Gan, Israel), Olivia Rosenfeld (Rehovot, Israel), Ryan Case (West Chester, PA, United States), C. Warren Olanow (Rye, NY, United States) Objective: ND0612 is under development as the ﬁrst non-surgical drug-device combination that provides continuous subcutaneous delivery of levodopa/carbidopa (LD/CD) for patients with Parkinson’s disease (PD) experiencing motor ﬂuctuations. Background: Determine the efﬁcacy, safety, and tolerability of continuous subcutaneous ND0612 infusion compared with oral LD/CD in patients with PD experiencing motor ﬂuctuations. Methods: We will enroll a total of 288 PD patients (Hoehn and Yahr =3) on =4 doses/day of LD/CD oral therapy (=400mg of LD), experiencing an average OFF time of at least 2.5 hours daily per ON/OF diary, with a minimum of 2 hours every day, during waking hours. The study comprises 6 periods: Period 1: Screening (1-4 weeks); Period 2: Open-label oral LD/CD optimization (6 weeks); Period 3: Open-label ND0612 optimization (6 weeks); Period 4: Double-blind, doubledummy, active-controlled, maintenance. In this period, patients are randomized to either ND0612 infusion + Dummy IR LD/CD OR Dummy infusion + IR LD/CD (12 weeks); Period 5: Optional openlabel extension (1-year); Period 6: Safety follow-up (12 weeks). Results: The primary endpoint is the change in mean ON time without troublesome dyskinesia from Baseline to end of maintenance (Period 4, Week 12), normalized to 16 waking hours. Secondary outcome measures include changes in: OFF time (key secondary), UPDRS (Parts II and III), Patient’s and Clinician’s Global Impressions of Change, ON time without dyskinesia, PDQ-39 and Parkinson’s Disease Sleep Scale (PDSS) scores. Clinical assessments will be conducted by blindedrater. Safety and tolerability will be assessed via adverse event reporting, including local skin safety assessments, rates of premature discontinuation, and treatment compliance. Conclusions: BouNDless will be the ﬁrst Phase III randomized, active-controlled trial to evaluate the efﬁcacy and safety of continuous subcutaneous ND0612 compared to oral immediate-release LD/CD in patients with PD experiencing motor ﬂuctuations. This abstract was presented at the 2019 International Congress of Parkinson’s Disease and Movement Disorders in September 2019. 155 Is the Parkinson’s KinetiGraph Reﬂective of Clinical Off/On Motor Testing: Single Center Experience Ashwin Malhotra (New York, NY, United States), Samantha Barkan (New York, NY, United States), Andrea Lee (New York, NY, United States), Natalie Hellmers (New York, NY, United States), Harini Sarva (Staten Island, NY, United States), Claire Henchcliffe (New York, NY, United States) Objective: To compare baseline Parkinson’s KinetiGraph (PKG) data with off/on testing in a long-term study. Background: The PKG is a wearable device that provides quantitative remote tracking based upon accelerometry measures of PD motor symptoms (bradykinesia, dyskinesia, and tremor) [1-2]. Methods: We analyzed baseline PKG data and standard measures of motor symptoms, including medication off/on testing, in subjects enrolled into a larger longitudinal observational study (<70 years old, PD duration >5 years, levodopa treatment > 3 years). 37 subjects (25 men, 12 women) with mean PD duration 6.422.94 years (range 3-14 years) since diagnosis were enrolled. Results: Baseline mean PKG dyskinesia score was 2.825.60 (range:0.1-29.2) and mean PKG bradykinesia score was 28.657.64 (range:14.4-44.9). 32 had dyskinesia scores below average (50th percentile score: 4.3), while 33 had bradykinesia scores above average (50th percentile score:18.6). The average off to on improvement after taking standard medication doses after withholding medications for 8-12 hours ranged from -8-100% (average improvement:52.522.8%). 31/37 showed robust levodopa response (response >30%). Average dyskinesia score was skewed to the right (median of 1; interquartile range of .400, 1.00, 2.30), due to two subjects’ having extremely high dyskinesia scores, 29.2 and 19.5. Bradykinesia scores had a normal curve distribution. Conclusions: Despite 31/37 having a robust levodopa response in clinical off/on testing, PKG data suggested suboptimal motor performance based upon higher than anticipated levels of bradykinesia. Thus, the PKG provided additional long-term continuous objective at home data. While off/on testing in the clinic is invaluable in describing levodopa response, it remains a snapshot. This discrepancy may be due to: 1. aggregate collection of bradykinesia data by the PKG; 2. different PD phenotypes. This is a small study sample that included a wide range of PD severity, motor symptoms, and levodopa dosages. Nonetheless, our results suggest that “real life” motor function may be suboptimal in some individuals despite having a robust levodopa response. References: 1. Grifﬁths, R.I., et al., Automated assessment of bradykinesia and dyskinesia in Parkinson’s disease. Journal of Parkinson’s disease, 2012. 2(1): p. 47-55. 2. Pahwa, R., et al., Role of the Personal KinetiGraph in the routine clinical assessment of Parkinson’s disease: recommendations from an expert panel. Expert review of neurotherapeutics, 2018. 18(8): p. 669-680. 156 NILO-PD: A Phase 2A Study of Nilotinib in Patients with Advanced and Early Parkinson’s Disease: Final Study Results Tatyana Simuni (Chicago, IL, United States), Brian Fiske (New York, NY, United States), Kalpana Merchant (Portland, OR, United States), Christopher MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S69 ABSTRACTS Coffey (Iowa City, IA, United States), Helen Matthews (London, United Kingdom), Richard Wyse (London, United Kingdom), Patrik Brundin (Grand Rapids, MI, United States), David Simon (Boston, MA, United States), Michael Schwarzschild (Boston, MA, United States), David Weiner (Austin, TX, United States), Jamie Adams (Rochester, NY, United States), Charles Venuto (Rochester, NY, United States), Laura Trusso (Rochester, NY, United States), Liana Baker (Rochester, NY, United States), Melissa Kostrzebski (Rochester, NY, United States), Tina Ward (Chicago, IL, United States), Gary Rafaloff (Marlboro, NJ, United States) Objective: To assess the safety and tolerability of daily oral administration of nilotinib (150 or 300 mg once daily) compared to placebo in moderate/advanced Parkinson’s disease (PD) participants. Background: Nilotinib is approved for the treatment of chronic myeloid leukemia, but not for PD. Preclinical studies indicate that nilotinib reduces alpha-synuclein pathology and protects dopamine neurons, leading to the hypothesis that it may slow PD progression. This hypothesis gained support from a preliminary, open-label study in moderate to advanced PD patients (Pagan et al., 2016) but requires further support from a randomized, controlled trial. Methods: NILO-PD is a Phase 2A randomized, double-blind, placebo-controlled, parallel group study. The study planned to enroll 75 participants with moderate to advanced PD randomized 1:1:1 to a once daily dose of nilotinib or placebo (150 mg: 300 mg: placebo) for 6 months. The primary outcomes are safety and tolerability of nilotinib while secondary outcomes include its effects on motor symptoms, progression of PD disability (MDS-UPDRS OFF/ON). Exploratory outcomes include serum and CSF PK proﬁle, cognitive function (DRS-2), quality of life and a battery of biomarkers. Results: Enrollment started in November 2017 and was completed in December 2018. The baseline demogaphics of the cohort are as follows: age - 65 (SD=7.5) years, sex - 68% male, and disease duration - 9.9 (SD=4.7) years. The last participant completed the study August 26th 2019. Data lock is scheduled for September 2019 with ﬁnal data analyses to be available in late November 2019. Conclusions: NILO-PD ﬁnal results will be presented at the meeting. This study will provide further information on the safety/tolerability, dose selection, PK and biomarkers proﬁle of nilotinib. In aggregate, these data will help determine if future studies of nilotinib should be conducted in a de novo PD population as a potential symptomatic and/or diseasemodifying therapy for PD. 157 Home Videos Made on Smart Phone Supplements Paper Based Diary for Correct Identiﬁcation of Motor Complications in Parkinson’s Disease Deepa Dash (Delhi, India), Rajesh Singh (Delhi, India), Deepti Vibha (New Delhi, India), Manjari Tripathi (Delhi, India) Objective: We aimed to assess the feasibility and utility of home videos made on smart phone as a supplemental tool to paper based diary in registering the motor states and thus correctly identifying motor complications. Background: Paper based diaries are the most commonly used method to ascertain the motor complication in patients with Parkinson’s disease.The accurate entry for the various motor states in paper based diary entry may be dependent on patient’s education and understanding Home videos may supplement paper based diaries in correctly identifying the motor states and thus the motor complications in patients. Methods: A total of 128 patients with Parkinson disease with motor complications without dementia were enrolled in the study from a Movement disorder clinic of a tertiary hospital in India. We explained the patient and caregivers about the various motor states of the patient (OFF, ON and dyskinesia) and gave them paper- based diary with Hindi translation to enter hourly recording for two consecutive days. We also asked patients to make videos of the various states made every hour for 5 minutes when patient was awake and mark them in the chart. We used a Quality of video scale which scored the video based on the quality of the videos and the content in ascertaining the motor state.Patients marked the timing of video recording made and the corresponding entry made in the PD diary. We then checked the entries made under the various headings in the diary and the motor state of the patient according to S70 the videos .We calculated the proportion of patients who correctly entered each motor state in the paper based diary considering the video as the gold standard. Results: Of the 128 patients, 94 patients followed the instructions and completed the PD diary and recorded home videos for the three motor states of the patient. Mean age of our patients 57.711.5 years and 64 were male. 82(87.2%) patients had less than high school education. A total of 61 patients video scored 6-8 which was graded as of acceptable quality to assess the motor complication,18 patients video were of poor but acceptable quality and 6 patients had poor quality (were not included). Based on the video evaluation, if there were more than 10% errors in the entry in a speciﬁc motor state in a day it was considered as incorrect entry. The percentage of correct entry of motor state based on Video evaluation was 80.7% for OFF,90.9% had correctly entered the ON and 59.1% had correctly entered dyskinesia in the PD charts. Conclusions: In resource strained countries, home videos made on smart phone can supplement paper-based diary in correctly identifying motor complications. Dyskinesia charting in the paper based diary has more errors compared to OFF and ON state. All efforts should be made to correctly identify the type of motor complication, as it helps in correct treatment being instituted. References: Antonini A, Martinez-Martin P, Chaudhuri RK, et al. Wearing-off scales inParkinson’s disease: critique and recommendations. Mov Disord 2011;26:2169–2175.Zanni GR. Patient diaries: charting the course. Consult Pharm 2007;22:472–476, 479–482. 158 Indicators of the Transition Process of the Relative of Elderly with Parkinson’s Disease to the Role of Caregiver Simony Lopes Nunes (Florianópolis, Brazil), Angela Maria Alvarez (Florianópolis-SC, Brazil) Objective: Our study aimed to identify the indicators of the process of transition of family caregivers and the inﬂuence of this in the care for elderly with Parkinson’s disease in the family context. Background: Parkinson’s disease (PD) is a chronic and progressive neurodegenerative disease of universal distribution that affects all ethnic groups and socioeconomic classes. It is considered a multisystem disorder difﬁcult to deﬁne due to the presence of motor symptoms in initial stages, such as gait disturbance and postural instability, that become progressively complicated by autonomic, cognitive and neuropsychiatric dysfunction. This disease represents a major clinical challenge because it is one of most common neurodegenerative diseases, mainly affecting the elderly population, a group whose size is increasing rapidly in the world, besides lacking therapeutic means to inﬂuence dopaminergic loss.Effective intervention from nurses toward family members who are in transitional situations require a better understanding of the main needs of the disease’s trajectory. Therefore, the Theory of Transitions developed by Meleis was selected as the theoretical framework for the present study to provide a comprehensive view of transitions that can serve as useful guidance for designing early interventions for family caregivers of older adults with PD. By deﬁning transitions as "a transition from one stage of life, condition or state to another" and believing that nursing has the purpose to promote the success of these transitions, regardless of the people involved, life, health, circumstances or the context of care, and the theorist deﬁned the response patterns to guide the nursing therapies in the process of transition. Methods: This study is exploratory, descriptive and qualitative conducted with 20 family caregivers of older adults with Parkinson’s disease. With institutional review board approval and participant permission, interviews were conducted in-person and transcribed verbatim. Transcripts were independently reviewed and coded for emerging themes. Data analysis was conducted based on Meleis’ theory of nursing transitions and thematic analysis. Results: The response patterns are responsible for keeping the nursing connected to the process and to the experiences of the individual as it occurs to the transition, and, these responses from individuals throughout the transition are called indicators, which can be indicators of Process and result indicators9. The Process indicators They allow to identify the evolution and success of the transition process. The recognition of this type of indicator is important to verify whether the individual is in the direction of health and well-being, or towards risks and vulnerability, MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS allowing nurses to intervene early in order to facilitate healthy results. The Result indicators They facilitate the identiﬁcation of the end of the transition and enable the evaluation of the evolution of the development of skills, confronting the expected results, that is, they are used to prove whether a transition is healthy or not. The results revealed that, at the passage of exit from transition, family caregivers show integration of the routine of care in their personal life, acceptance of the health state of their relative through positive view of the disease, as well as they use strategies to acquire skills related to care for elderly with Parkinson’s, and they recognize in themselves the identity of family caregivers. Conclusions: Interventions are needed to identify the speciﬁc concerns of family members to strengthen and facilitate the process of transition to exercise the role of caregiver, in face of the various changes in routine that Parkinson’s raises. References: 1. Benavides O, Alburquerque D, Chana-Cuevas P. Evaluación de la sobrecarga en los cuidadores de los pacientes con enfermedad de Parkinson ambulatorios y sus factores de riesgo. Rev. méd. Chile. 2013; 141(3): 320-326. http://dx.doi.org/10.4067/ S0034-98872013000300006. 2. Beitz JM. Parkinson’s disease: a review. Front Biosci (Schol Ed). 2014;1(6): 65-74. http://dx.doi.org/10.2741/ S415. Yarnall A, Archibald N, Burn D. Parkinson’s disease. Medicine. 2012; 40(10): 529-535. http://dx.doi.org/10.1016/j.mpmed. 2012.07.008. 9. Meleis AI, Sawyer LM, Im EO, Hilﬁnger Messias DK, Schumacher K. Experiencing transitions: an emerging middle-range theory. ANS Adv Nurs Sci. 2000; 23(1): 12-28. http://dx.doi. org/10.1097/00012272-200009000-00006. to stages; 7days and 13 days of before motor symptoms appearance. By means of immunohistochemistry, the serotonergic innervation of the Basolateral Amygdala nucleus (BLA) as well as the morphological changes of astroglia within hippocampus (using anti-GFAP marker) were examined at the latest stage (13days) of the disease. Results: Our data show a differential deterioration of short-term working memory without the long-term spatial memory being changed, which was accompanied by a striking change in both density and morphology of astrocytes at the level of the Stratum Lacunosum-Molecular (SLM) of the hippocampus. Conclusions: Our study emphasizes an early alteration of the shortterm working memory without long-term spatial memory damage during the prodromal phase of PD. While astroglia exhibited profound morphological changes within the SLM layer of the hippocampus, leading to suppose a functional disruption of astrocyte-neuron network which may elicit short memory decline in PD. References: Fernandes, V.S., Santos, J.R., Le~ao, A.H.F.F., Medeiros, A.M., Melo, T.G., Izídio, G.S., Cabral, A., Ribeiro, R.A., Abílio, V.C., Ribeiro, A.M., Silva, R.H., 2012. Repeated treatment with a low dose of reserpine as a progressive model of Parkinson’s disease. Behav. Brain Res. 231, 154–163. https://doi.org/10.1016/j.bbr.2012.03.008.Santos, J.R., Cunha, J.A.S., Dierschnabel, A.L., Campêlo, C.L.C., Le~ao, A.H.F.F., Silva, A.F., Engelberth, R.C.G.J., Izídio, G.S., Cavalcante, J.S., Abílio, V.C., Ribeiro, A.M., Silva, R.H., 2013. Cognitive, motor and tyrosine hydroxylase temporal impairment in a model of parkinsonism induced by reserpine. Behav. Brain Res. 253, 68–77. 159 160 Assessment of Memory Function During the Pre-Motor Stage in Rat with Progressive Parkinson’s Disease Onset Induced by Repetitive Reserpine Administration Ahmed Draoui (Marrakesh, Morocco), Omar EL Hiba (Marrakesh, Morocco), Abdelaati El Khiat (Marrakech, Morocco), Abdellatif Abbaoui (Aitourir, Morocco), Radouane El Fari (Marrakech, Morocco), Mohamed Aitihya (Marrakech, Morocco), Halima Gamrani (Marrakech, Morocco) Withdrawn by Author Objective: The purpose of the present investigation is to assess the evolution of two types of memory alteration namely; short working and spatial memories at different stages of the prodromal phase in a rat model of PD, using repetitive reserpine administration at low dose during 13 days. Together with the hippocampal astroglial reaction using immunohistochemistry of GFAP. Background: Non-motor symptoms of Parkinson’s disease (PD) is a particular side of the disease which has been for long underestimated. Taking into account its heavy outcome on patient’s quality of life, studying the non-motor symptoms is of crucial importance. In general, the most commonly used classical neurotoxic animal models such as 6-OHDA and MPTP do not offer the possibility of exploring this important phase especially the chronology of the different patterns onset. Methods: Rats were i.p injected with reserpine (0.2 mg/kg/day) during 13 days. Working memory was assessed by the Novel Object Recognition test, while spatial memory was assessed by Morris Water maze at 161 Sexual Dysfunction: An Understimated Presentation in Women with Parkinson’s Disease Susana Lopez-Alamillo (Villahermosa, Mexico), Carolina Terán (Mexicali, Mexico), Cynthia Sarabia-Tapia (Mexico City, Mexico), Mayela Rodriguez Violante (Mexico City, Mexico), Amin Cervantes (Mexico City, Mexico), Oscar Esquivel-Zapata (Monterrey, Mexico), Yazmin Rios (Ciudad de Mexico, Mexico), Fanny Herrera-Rodríguez (Mexico City, Mexico), Rodolfo AbundesCorona (Mexico City, Mexico), Omar Cardenas (Ciudad de Mexico, Mexico), Emmanuel Escobar-Valdivia (Mexico City, Mexico) Objective: Identify among patients attending the Movement Disorders Unit at the National Institute of Neurology and Neurosurgery, Mexico, if Sexual Dysfunction (SD) in women is more prevalent in patients with Parkinson’s disease (PD) than in general women population. Background: The World Health Organization, refers to sexuality as a central aspect of life and declares that it is a fundamental right of the individual to enjoy and control sexual and reproductive behavior in accordance with social and personal ethics. In clinical practice, management of PD patients tends to be focused on motor symptoms, whereas MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S71 ABSTRACTS sexual function is an issue rarely raised, both by neurologists and patients. There is a lack of research regarding female sexual dysfunction. Methods: We analyzed data and clinical history of female patients with PD and to a control group [table1]. Evaluation of SD was performed based on Female Sexual Dysfunction Index (FSDI) [table2]. In addition, MDS-UPDRS part III, Hoehn and Yahr scale, Assessment of autonomic dysfunction in Parkinson’s disease (SCOPA-AUT), and Hamilton Depression Rating Scale (HAM-D) were applied. Inclusion and exclusion criteria were applied. Results: Mean age in women with PD was (57.29) and most of them were married (71.4%). Mean age of 21 controls was 46.1 years and most of them were married too (66.7%) [table1]. In 47.6 % of patients, sexual dysfunction was presented according to the FSFI. Not statistically signiﬁcance results were obtained among patients with sexual dysfunction and motor symptoms. Conclusions: Sexual dysfunction in women patients with PD was more often found than in general women population. However, further studies shall be done in order to clearly identiﬁed determinants. That’s why we will continue recruiting patients and controls to obtain a sample to provide a complete identiﬁcation of sexual dysfunction determinants. References: Sexual Health [Internet]. World Health Organization. 2019 [cited 19 July 2019]. Available from: https://www.who.int/topics/ sexual_health/es/.2.Varanda S, Ribeiro da Silva J, Costa A, Amorim de Carvalho C, Alves J, Rodrigues M et al. Sexual dysfunction in women with Parkinson’s disease. Movement Disorders. 2016;31 (11):1685-1693.3.Rosen R, Brown C, Heiman J, Leib S. The Female Sexual Function Index (FSFI): A Multidimensional Self-Report Instrument for the Assessment of Female Sexual Function. Journal of Sex & Marital Therapy. 2000;26(2):191-208. 162 Relationship Between Energy Intake of Macro and Micronutrients with Parkinson’s Disease Stages Cynthia Lopez Botello (Monterrey, Mexico), Sergio Castillo-Torres (Monterrey, Mexico), Beatriz Chávez-Luévanos (Monterrey, Mexico), Ingrid Estrada Bellmann (Monterrey, Mexico), Patricia Ancer Rosríguez (Monterrey, Mexico) Objective: Relate the energy intake of macro and micronutrients with the stages of PD in patients. Background: Muscle-targeted nutritional strategies improve rehabilitation [1]. The relationship between nutritional intake composition and disease severity remains largely unexplored [2,3]. Methods: We recruited patients from the outpatient movement disorders clinic, ages 30 to 88, with diagnosis of idiopathic PD. Energy Intake was measured by 24-hour dietary reminder, macro and micronutrients were calculated using Nutrimind Nutritional Software (NNS; Aguascalientes, México). Nutritional status was evaluated with anthropometric measures: Body Mass Index (BMI), Tricipital Skin Fold (TSF), Muscular Arm Circumference (MAC) and Muscular Arm Area (MAA). Disease progression was classiﬁed with Hoehn and Yahr stages (H&Y), in mild (H&Y 1), moderate (H&Y 2-3), and severe (H&Y 4-5). Risk of malnutrition and protein malnutrition was determined by equations and tables elaborated by Frisancho. Chi squared was used to contrast variables. Results: We included 74 patients, 54.7% male with mean age 6111 years; frequent stage was moderate (73.9%). BMI was underweight in 24.6% (n=17). MAC 15.8% (n=9) and MAA 8.8% (n=5) had risk of malnutrition. Total energy intake signiﬁcantly higher in patients over 60 years (p=0.026). Patients with moderate stage had signiﬁcantly higher proportion of inadequate zinc intake (p=0.003), and female patients higher proportion of deﬁcient retinol intake (p=0.030). Most patients with normal protein reserve–according to MAA–had signiﬁcantly higher proportion of calcium deﬁciency (p=0.026) whereas patients with normal parameters had signiﬁcantly higher proportion of excessive intake of retinol (p=0.001) and phosphorus (p=0.046). Conclusions: Despite increased energy intake, patients with PD had several micronutrient deﬁciencies. Zinc is a fundamental mineral for muscle growth but unfortunately, it is not abundant in daily diet of patients. Also it is well known that an excessive consumption of retinol leads to an appetite loss that can cause risk of malnutrition. Therefore, based on the results obtained in this study, new nutritional changes are necessary. The importance of carrying out future studies where we can approach to a better nutritional intervention that can balance the intake of macro and micronutrients to prevent problems related with nutrition is needed. Henceforth the importance of nutrition in PD to improve lifestyle and quality of life. References: 1.Barichella M, Cereda E, Pinelli G, et al. Muscletargeted nutritional support for rehabilitation in patients with parkinsonian syndrome. Neurology. 2019;93(5):e485-e96.2.Du K, Liu M-Y, Zhong X, et al. Decreased circulating Zinc levels in Parkinson’s disease: a meta-analysis study. Scientiﬁc Reports. 2017;7(1):3902.3. Kocot J, Luchowska-Kocot D, Kielczykowska M, et al. Does Vitamin C Inﬂuence Neurodegenerative Diseases and Psychiatric Disorders? Nutrients. 2017;9(7). 163 Effect of an Increase in Dose of Istradefylline, an A2A Receptor Antagonist, in Levodopa (LD)-Treated Patients with Parkinson’s Disease (PD) Nobutaka Hattori (Tokyo, Japan), Takanobu Nomura (Tokyo, Japan), Phyllis Salzman (Wyncote, PA, United States), Hiroki Kitabayashi (Tokyo, Japan), Masatake Ishiuchi (Tokyo, Japan), Keizo Toyama (Tokyo, Japan), Akihisa Mori (Tokyo, Japan) Objective: To observe whether increasing the dose of istradefylline from 20 to 40 mg/day provides more patients with meaningful clinical responses. Background: Istradefylline is an adenosine receptor antagonist indicated as adjunctive treatment to levodopa/carbidopa in adult patients with Parkinson’s disease (PD) experiencing OFF episodes. Istradefylline S72 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS signiﬁcantly improved OFF time in placebo-controlled, ﬁxed-dose studies, with similar mean efﬁcacy responses for 20 and 40 mg/day doses. Methods: Istradefylline was evaluated in PD patients receiving LD and experiencing motor ﬂuctuations. In this open-label extension study, following a Phase 3, randomized, placebo-controlled, double-blind trial of istradefylline, patients (N=308) received a starting once-daily oral dose of istradefylline 20 mg/day. The dose could be increased to 40 mg once daily after 4 weeks if there were no safety concerns, unsatisfactory treatment response, and patient agreement. At Week 8, change in daily OFF time and ON time (using patient-completed 24-hour ON/OFF diaries) and Clinical Global Impression-Global Improvement (CGI-I) were assessed and compared with Week 4. Treatment-emergent adverse events (TEAEs) were recorded throughout. Results: Baseline characteristics were similar between analysis groups (remained on 20 mg or increased to 40 mg). At Week 8, the percentage of patients with =1-hr reduction in daily OFF time was greater among those whose istradefylline dose was increased to 40 mg vs those who remained on 20 mg (Table). Similarly, a greater percentage of patients had =1-hr increase in ON time without troublesome dyskinesia and improved CGI-I scores when the istradefylline dose was increased to 40 mg compared with those who remained on 20 mg (Table). Among TEAEs reported in =5% of patients, those that were =3% more frequent when the dose was increased to 40 mg/day than when maintained at 20 mg/day were nasopharyngitis, dyskinesia, contusion, and constipation. Conclusions: These results demonstrate that for patients whose response to istradefylline 20 mg/day was suboptimal, increasing the dose to 40 mg/day resulted in a greater percentage of patients with improvements in patient-reported OFF time, ON time without troublesome dyskinesia, and physician-rated CGI-I scores. This abstract was presented at the 2019 International Congress of Parkinson’s Disease and Movement Disorders in September 2019. 164 Discussing OFF Periods in Parkinson’s Disease: Expert Panel Feedback Iresha Abeynayake (Ardsley, NY, United States), Risa Torkin (Ardsley, NY, United States), Holly Roberts (Ardsley, NY, United States) Objective: Expert panels of health care providers (HCPs) specializing in movement disorders were held in different regions of the United States to gain insight into clinical experiences with OFF periods in Parkinson’s disease (PD). Background: OFF periods (re-emergence of PD symptoms) are likely to occur at some point in disease progression despite baseline therapies. Challenges in treating OFF periods include identiﬁcation, management, and HCP–patient communication about OFF periods. The burden of OFF periods, particularly of unpredictable OFF periods, and nonmotor symptoms of OFF, such as anxiety, depression, and cognitive issues, can have a profound impact on patients’ lives. Methods: Expert panels consisting of HCPs (physicians and allied health professionals) from movement disorder neurology practices were convened in 6 US geographic regions. The discussion guide focused on aspects of OFF periods including deﬁnition, most common symptoms, awareness and recognition of OFF periods (clinical evaluation, patient awareness, educational gaps), physician/patient communication, treatment decisions to address OFF, burden and impact on daily life, and clinical experience of managing OFF periods. Results: OFF periods were commonly deﬁned by the HCP panelists as return of motor and nonmotor symptoms. HCPs reported that some patients attributed OFF periods to medication, or considered OFF to be part of disease progression. In most regions, there was an unmet need for education on motor and nonmotor (ie, GI dysfunction, anxiety) symptoms of OFF. HCPs reported that OFF periods affected lifestyle and had a negative impact on quality of life including impacting the ability to work, drive, mobility, and other activities of daily living. Some clinics used OFF questionnaires and nonstandardized checklists, and some reported having patient education and support groups to address symptoms and management, but these were not universal across regions. HCPs reported that for management of OFF symptoms, there is no “standard of care” and there is wide variability in the awareness and assessment of OFF symptoms by region. Conclusions: HCP panelists emphasized the need for effective communication between physician, patient, and caregiver, variation in management of OFF periods, and early recognition of OFF, which are important for effective management and treatment. 165 Exploring the Mechanism of Bradykinesia in Parkinson’s Disease Thomas Osterholt (Bethesda, MD, United States), Patrick McGurrin (Bethesda, MD, United States), Felipe Vial Undurraga (Santiago, DC, Chile), Mark Hallett (Bethesda, MD, United States), Debra Ehrlich (Rockville, MD, United States) Objective: The goal of this study was to assess whether bradykinesia observed in Parkinson’s disease (PD) is caused by a slowdown of the typical 8-12Hz frequency of normal movements and whether this frequency can be normalized with dopaminergic therapy. Background: Slow ﬁnger movements in healthy subjects are composed of small movements at a frequency of 8-12 Hz [1]. However in PD, little is known about the frequency characteristics of these movements and if a slowing of this frequency explains bradykinesia. Methods: Six healthy volunteers and three PD patients (UK brain bank criteria) have been enrolled. All subjects used visual feedback to perform continuous, slow adduction-abduction movements with the MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S73 ABSTRACTS index ﬁnger. The frequency of the movement was compared between healthy volunteers and PD patients in both “on” and “off” medication states using a potentiometer. Results: Our preliminary analysis demonstrates that the frequency of slow adduction-abduction movements with the index ﬁnger of healthy volunteers (7.33 Hz) was higher compared to that of PD patients (6 Hz) in the “off” state. Furthermore, PD patients in the “on” state exhibited a movement frequency of 7 Hz compared to 6 Hz in the “off” state. Conclusions: Our preliminary analysis suggests that while PD patients exhibit a movement frequency slower than healthy controls in the “off” state, their movement frequency is closer to that of healthy when in the “on” state.This study is limited by a small sample size. We plan to recruit more participants and expand the analysis to include other experimental conditions such as ballistic movements and maximal force production. Future analysis of EMG and EEG data will be used to explore the underlying mechanisms of this frequency slowing in PD patients. References: 1. Vallbo, A.B. and J. Wessberg, Organization of motor output in slow ﬁnger movements in man. J Physiol, 1993. 469: p. 673-91. 166 Pallidal vs Subthalamic Deep Brain Stimulation (DBS) in Parkinson’s Disease: Our Experience Carla Stefani (Ciudad De Buenos Aires, Argentina), Lucía Ciancaglini (Buenos Aires, Argentina), María Justich (Buenos Aires, Argentina), José Bestoso (Buenos Aires, Argentina), Facundo Silveira (Buenos Aires, Argentina), Carlos Ciraolo (, United States), Diego Bauso (Buenos Aires, Argentina) Objective: The objective of our presentation is to compare the motor, non motor response and quality of life between pallidal and subthalamic DBS in the short term. Background: DBS improves motor function and quality of life in patients with advanced Parkinson’s disease (PD). Subthalamic Nucleus (STN) and Globus Pallidus Internus (GPi) are the main targets. Nowadays, it still remains questionable about which one is the optimal therapy. Methods: We performed a prospective observational cohort study that included patients undergoing DBS surgery between December 2009 and June 2018. We considered demographic variables, The Uniﬁed Parkinson’s Disease Rating Scale (MDS-UPDRS) and The Parkinson’s Disease Questionnaire (PDQ-39), before and 3 months after DBS surgery. Results: 29 patients were included, 72,4% were men. Mean age at the time of surgery was 57 8 years, and mean time of disease was 14 7 years. Fifteen patients (51,7%) underwent pallidal stimulation and 14 subthalamic stimulation. The MDS-UPDRS Ia improved 2,46% in pallidal stimulation, but didn’t modify in subthalamic stimulation (p0,718). The UPDRS-Ib improved 7,7% in pallidal stimulation and 6,4% in subthalamic stimulation (p0,95). UPDRS-II improved 16,83% in pallidal stimulation and 33,72% in subthalamic stimulation (p0,136). UPDRS-III improved 29,95% in pallidal stimulation and 51,83% in subthalamic stimulation (p0.026). UPDRS-IV improved 51,73% in pallidal stimulation and 47,98 in subthalamic stimulation (p0.763). PDQ-39 scale improved 24,6% in pallidal stimulation and 18.41% in subthalamic stimulation (p0.617). Conclusions: Even though there was a signiﬁcant difference between improvement in motor function on patients undergoing subthalamic stimulation, quality of life did not differ between both groups. Motor complications improved similarly in both groups. References: 1. Follett KA, Weaver FM, Stern M, Hur K, Harris CL, Luo P, et al. Pallidal versus subthalamic deep-brain stimulation for Parkinson’s disease. N Engl J Med. 2010;362: 2077–2091. 2. Quality of life predicts outcome of deep brain stimulation in early Parkinson disease. Neurology. 2019;92: 1166. 3. Geraedts VJ, Kuijf ML, van Hilten JJ, Marinus J, Oosterloo M, Contarino MF. Selecting candidates for Deep Brain Stimulation in Parkinson’s disease: the role of patients’ expectations. Parkinsonism Relat Disord. 2019; doi:10.1016/j.parkreldis.2019.07.011 167 Pharmacokinetics (PK) of Inhaled Levodopa Co-Administered with Oral Carbidopa in Subjects with Parkinson’s Disease Under Fed Conditions Beth Saﬁrstein (North Miami Beach, FL, United States), Aaron Ellenbogen (Bingham Farms, MI, United States), Ping Zhao (Ardsley, NY, United States), S74 Herbert Henney (West Grove, PA, United States), Deena Kegler-Ebo (Ardsley, NY, United States), Charles Oh (Ardsley, NY, United Kingdom) Objective: To evaluate PK proﬁles, safety and tolerability of single doses of CVT-301 84 mg, administered with oral carbidopa (CD), and single oral doses of carbidopa/levodopa (CD/LD), under fed conditions, in Parkinson’s disease (PD) subjects. Background: CVT-301 is an LD inhalation powder for treatment of OFF period symptoms in PD subjects on an oral dopa-decarboxylase inhibitor/LD regimen. Methods: In an open-label crossover study, subjects received a single inhaled dose of CVT-301 84mg administered with CD 25mg followed by oral CD/LD (25/100mg) or vice versa in a randomized sequence in 2 dosing periods. 4-5 hours after their typical morning oral CD/LD dose, subjects ate a standardized high-fat, high-protein meal. A predose PK blood sample was obtained after the meal, followed by CVT-301 treatment administered with oral CD 25mg, or oral CD/LD 25mg/100 mg. Blood was sampled at 5, 10, 15, 30, and 45 minutes, and 1, 1.5, 2, 3, and 4 hours postdose to determine plasma LD concentrations. PK parameters were evaluated using a non-compartmental method. Standard safety assessments included treatment-emergent adverse events (TEAEs). Results: 23 subjects (mean age 69.3 years, mean BMI 26.9, mean PD duration 8.2 years) were enrolled: 15 men and 8 women; 17% at Hoehn and Yahr stages 1-1.5, 57% at stage 2, 26% at stages 2.5-3. Mean LD dose was 461mg/day. 1 subject discontinued due to TEAE of cough. Primary PK analyses were based on LD concentrations without adjustment for LD detected in plasma at baseline. PK parameters are shown in [Table 1]. Cmax was lower for CVT-301 than oral CD/LD. C10min, C15min, and C30min values for CVT-301 were higher than those for oral CD/LD. Median Tmax for CVT301 was 15 min vs 120 min for oral CD/LD (P<0.001). The coefﬁcient of variation % for CVT-301 was smaller than with oral CD/LD. Nine (39.1%) and 2 (9.1%) subjects reported TEAEs following CVT-301 and oral CD/LD administration, respectively. Most common TEAE was cough: 7 (30.4%) in the CVT-301 group vs 1 (4.5%) subject administered oral CD/LD. Conclusions: CVT-301 administration to PD subjects under fed conditions was safe and well tolerated. PK parameters showed CVT-301 was more rapidly absorbed and demonstrated lower inter-subject variability than oral CD/LD in the fed condition. 168 Chart Review of the PD Hispanic/Latino Population at the University of Alabama at Birmingham Florencia Scaglia Drusini (Birmingham, AL, United States), Marissa Dean (Birmingham, AL, United States) Objective: To report the characteristics of the Hispanic/Latino Parkinson’s disease (PD) patient population at the University of Alabama at Birmingham (UAB) to assess the accessibility to healthcare that may contribute to disease presentation. Background: Hispanics and Latinos seek medical care from neurologists at a lower rate than non-Hispanic/Latino patients.1 To the best of our knowledge, no studies have compared PD characteristics between the two ethnic groups. Methods: A retrospective chart review was performed on all patients with an ICD10 diagnosis code of G20 that were seen at UAB between 1/1/09 and 1/1/19 and self-identiﬁed with Hispanic/Latino ethnicity. A total of 70 Hispanic/Latino patients were identiﬁed and compared with a random sample of 70 non-Hispanic/Latino patients. Categorical variables were compared with a chi-squared test and interval variables were compared using the Mann-Whitney U test. Statistical analyses were MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS performed using SPSS version 25.0.0.2 (IBM Corp., Armonk, NY). Statistical signiﬁcance was deﬁned as p<0.05. Results: Idiopathic PD was conﬁrmed in 49 patients (30 Hispanic/ Latino, 19 non-Hispanic/Latino), and these patients were included in the analysis. There was no signiﬁcant difference in age, sex, age at diagnosis, time to diagnosis from symptom onset, follow up with a neurologist versus primary care physician (PCP), or cognitive symptoms. However, there was a signiﬁcant difference in race (p<0.0001). Conclusions: As with previous studies, there is a low positive predictive value in identifying idiopathic PD patients with the ICD10 code G20. The difference observed in race was likely secondary to a small sample size. There was no signiﬁcant delay from time of symptom onset to diagnosis in these Hispanic/Latino PD patients, and there was no difference in whether PD care was provided by a neurologist or PCP. These ﬁndings argue against health disparities contributing to access of care. However, given this small sample size, further studies are needed to evaluate for the presence of health disparities in the Hispanic/Latino PD population. References: 1. Saadi A, et al. Racial disparities in neurologic health care access and utilization in the United States. Neurology. 2017;88:2268-2275. 169 Primary Care for People with Parkinson’s Disease in Brazil: A Referral Flowchart Based on Risk of Falls Rafaela Myra (Florianópolis, Brazil), Elaine Gregório (Florianópolis, Brazil), Micheline Da Luz Koerich (Florianopolis, Brazil), Alessandra Swarowsky (Florianópolis, Brazil) Objective: The aim of our study was to develop a referral ﬂowchart based on the risk of falls, on contemporary scientiﬁc evidence, as well as on the need for multi-professional treatment for people with PD in Primary Care in Brazil. Background: Individuals with Parkinson’s disease (PD) need to exercise to have a better quality of life1,2,3. Falls are relevant when choosing the appropriate type of exercise4. In the Brazilian primary care network, ﬂowcharts are used to assist in referral5, but there is no speciﬁc for PD. Methods: We performed a non-systematic literature review on the main databases - PubMed, Scielo, Lilacs, Medline, Cinahal, Pedro - using the keywords Parkinson’s disease AND fall OR falling OR falling. Were included articles in the English language without publication date restriction. Also, we consulted international guidelines for individuals with PD. Results: A fall risk-based ﬂowchart has been created to assist health workers in referral individuals with PD to services available in the primary care network. Fall risk is assessed with the 3-step-fall-prediction tool, it is quick and easy to use6. The professional should refer the individuals with low risk of falls to activities with low follow-up and those with greater risk of falls to specialized neurology services. Individuals with mobility restrictions (wheelchair, or restricted to bed) should also be referred to specialized neurology services. The referral occurs according to what is available on the Brazilian primary care. The images and tables below show the ﬂowchart. Conclusions: This ﬂowchart can help Brazilian healthcare workers to refer individuals with PD according to the risk of falls. This study may mean the beginning of an organized care policy for individuals with PD, paying attention to exercise therapies besides medication. Also, the use of this ﬂowchart may provide safety for the health workers and prevent accidents for people with PD. References: 1. Tomlinson CL. et al. Physiotherapy versus placebo or no intervention in Parkinson’s disease. Cochrane Database Systematic Reviews, (2013) 10(9) doi: 10.1002/14651858.2. Ahlskog JE. Aerobic Exercise: Evidence for a Direct Brain Effect to Slow Parkinson Disease Progression. Mayo Clinic Proceedings, (2018) 93(3):360-3723. Wu PL, Lee M, Huang TT. Effectiveness of physical activity on patients with depression and Parkinson’s disease: A systematic review. PLoS One. (2017) 12(7):e0181515. doi: 10.1371/journal.pone.0181515.4. Canning CG; Paul SS.; Nieuwboer A. Prevention of falls in Parkinson’s disease: a review of fall risk factors and the role of physical interventions. Neurodegen. Dis. Manage. (2014) 4(3), 203–2215. Brasil. Ministério da Saúde. Secretaria de Atenç~ao à Saúde. Departamento de Atenç~ao Básica. Acolhimento à demanda espontânea / Ministério da Saúde. Secretaria de Atenç~ao à Saúde. Departamento de Atenç~ao Básica. – 1. ed.; 1. reimpr. – Brasília: Ministério da Saúde, 2013. 56 p. : il. – (Cadernos de Atenç~ao Básica; n. 28, V. 1)6. Paul SS, Canning CG, Sherrington C, Lord SR, Close JCT, Fung VSC. Three simple steps to accurately predict falls in MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S75 ABSTRACTS people with Parkinson’s disease. Mov Disord. 2013 May;28(5):655-62. doi: 10.1002/mds.254047. van der Marck MA, et al., Consensus-based clinical practice recommendations for the examination and management of falls in patients with Parkinson’s disease, Parkinsonism and Related Disorders (2014), http://dx.doi.org/10.1016/ j.parkreldis.2013.10.030 170 Efﬁcacy of Transcranial Direct Current Stimulation in Patients with Parkinson’s Disease: A Systematic Review and MetaAnalysis Md Azharuddin (New Delhi, India), Pinaki Ghosh (Pune, India), Prem Kapur (New Delhi, India), Manju Sharma (New Delhi, India) Objective: The objective of this study to conduct a meta-analysis to evaluate the efﬁcacy of transcranial direct current stimulation (tDCS) in improving motor, non-motor symptoms and health-related quality of life for PD patients. Background: Parkinson’s disease (PD) is a chronic neurological disease, mostly affects the age group of > 50 years. The loss of dopaminergic neurons, characterized to motor deﬁcits, with the severity of the disability, which poses a therapeutic challenge. Transcranial direct current stimulation (tDCS) has been proposed as a noninvasive brain stimulation approach as an adjunct intervention for PD patients. Methods: A systematic search on MEDLINE and Cochrane Central Register of Controlled Trials was performed with pairing relevant keywords to identify English language articles published between Jan 2010 and August 2019. The eligible studies compared tDCS versus control for improving health-related quality of life, disability, and impairment in patients with PD. A random-effects model was used to calculate the pooled mean difference (MD) with 95% conﬁdence interval (CI). Metaanalysis was performed using RevMan 5.3 software. Results: A total 10 relevant studies met the inclusion criteria with a total of 231 participants. The primary outcome measures, impairment, as measured by the Uniﬁed Parkinson’s Disease Rating Scale (UPDRS) and secondary outcome measures, as gait speed.Results from the pooled meta-analysis showed very low-quality evidence for no effect of tDCS vs control group on UPDRS III score (MD -0.02, 95% CI -1.94 to 1.89; P = 0.98).One study measured the reduction in off and on time (hours) with dyskinesia, but there was no evidence of a signiﬁcant effect (MD 0.10, 95% CI -0.14 to 0.34; P = 0.41 and MD 0.00, 95% CI -0.12 to 0.12; P = 0.98 respectively. However, there was no signiﬁcant difference in gait speed (m/s) in tDCS plus movement therapy compared to control (MD 0.06, 95% CI -0.01 to 0.14; P=0.11). Conclusions: The current meta-analysis found, no signiﬁcant effect in improving health-related quality of life, disability, and impairment in patients with PD between tDCS and control groups. However, further RCTs and real-world studies required to make this ﬁnding more robust. References: 1. Valentino F, Cosentino G, Brighina F, Pozzi NG, Sandrini G, Fierro B, et al. Transcranial direct current stimulation for S76 treatment of freezing of gait: A cross-over study. Movement Disorders. 2014 Jul;29(8):1064-9. 2. Biundo R, Weis L, Fiorenzato E, Gentile G, Giglio M, Schifano R, et al. A Double-blind randomized trial of t-DCS versus sham in Parkinson patients with mild cognitive impairment receiving cognitive training. Brain Stimulation: Basic, Translational, and Clinical Research in Neuromodulation. 2015 Nov 1;8(6):1223-5.3. Tahtis V, Kaski D. Parkinson’s disease treatments: focus on transcranial direct current stimulation (tDCS). Journal of Parkinsonism and Restless Legs Syndrome. 2017;2017(7):55-70 171 Increased Prevalence of Self-Reported Clinically Diagnosed B12 Deﬁciency in LRRK2 and Idiopathic Parkinson’s Disease Sarah Simon (New York, NY, United States), Viktoriya Katsnelson (Brooklyn, NY, United States), Nikita Urval (New York, NY, United States), Leon Meytin (New York, NY, United States), Sonya Elango (New York, United States), Deborah Raymond (New York, United States), Susan Bressman (New York, NY, United States), Rachel Saunders-Pullman (New York, NY, United States), Roberto Ortega (New York, NY, United States) Objective: To determine a possible clinical relationship between B12 deﬁciency, the LRRK2 G2019S mutation and Parkinson’s disease (PD), we evaluated the prevalence of diagnosed B12 deﬁciency in genetic and idiopathic PD, and controls. Background: Vitamin B12 is critical to neurologic health. Lower B12 is associated with worse cognition and gait in PD. In animal and human ﬁbroblast LRRK2 models, B12 (5-deoxyadenosylcobalamin) inhibited LRRK2 kinase and decreased dopaminergic neuronal loss. Methods: Personal medical histories were systematically collected using questionnaires in individuals evaluated as part of a genetic study of movement disorders at Mount Sinai Beth Israel. Subjects completed self administered questionnaires as to whether they were ever diagnosed with and/or been treated for B12 deﬁciency. Responses were limited to: yes, no, unknown; unknown responses were excluded.34 LRRK2 G2019S PD, 64 idiopathic PD (IPD), 47 GBA related PD (GBA PD) and 47 non-PD controls reported status. Subjects with mutations in both LRRK2 G2019S and GBA were also excluded. Validation with clinical record was performed when possible. The odds of B12 deﬁciency was compared between groups using logistic regression, adjusting for age and sex. Results: Age at study visit was not different between LRRK2 PD (699.2 years), idiopathic PD (IPD) (69.18.2), GBA PD (65.310.8), and controls (65.214.0). As expected, women were overrepresented in the control group (42.1%) compared to LRRK2 PD (20.5%), GBA PD (17.1%), and idiopathic PD (20.5%) (p<0.01). 6/34 (17.78%) LRRK2 PD, 1/47 (2.1%) GBA PD, 8/64 (12.5%) IPD and 2/47 (4.3%) of controls reported history of B12 deﬁciency. LRRK2 PD had greater odds of self-reported B12 deﬁciency compared to controls (OR=5.60, 95%CI: 1.02, 30.82; p=0.048) and GBA PD (OR=2.11, 95%CI: -0.07, 4.30; MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS p=0.058). No other group differences in reported B12 deﬁciency were present, including no difference between IPD and controls. Conclusions: Despite limited sample size, higher rates of B12 deﬁciency in the LRRK2 PD group supports a role for B12 in LRRK2 PD. These data are consistent with prior studies suggesting that physiological forms of B12, allosteric inhibitors of kinase hyperactivity, may be beneﬁcial in prevention or treatment of LRRK2 PD. Further investigation is ongoing, including whether lower B12 homocyteine and methylmalonic acids levels differ in LRRK2 PD and/or reported B12 deﬁciency or serum levels may be associated with penetrance of LRRK2. Thus, although additional work is warranted, our ﬁndings highlight the potential of B12 as a therapeutic avenue worthy of exploration. References: Shaffner A, Li X, Gomez-Llorente Y, Yue Z, et al. (2019). Vitamin B12 modulates Parkinson’s disease LRRK2 kinase activity through allosteric regulation and confers neuroprotection. Cell Res., pp. 313-29, doi:10.1038/s41422-019-0153-8. Christine CW, Auinger P, Joslin A, Parkinson Study Group-DATATOP, et al. (2018). Vitamin B12 and Homocysteine Levels Predict Different Outcomes in Early Parkinson’s Disease. Movement Disorders, pp. 762-770, doi: 10.1002/mds.27301 Dietiker C, Kim S, Zhang Y, Christine CW, on behalf of the NINDS NET-PD Investigators. (2019). Characterization of Vitamin B12 Supplementation and Correlation with Clinical Outcomes in a Large Longitudinal Study of Early Parkinson’s Disease. Journal of Movement Disorders. 12(2):91-96, doi: https://doi.org/10.14802/jmd.18049 Division of Hematology, University of Colorado School of Medicine. (2013). Clinical practice. Vitamin B12 deﬁciency. New England Journal of Medicine, 368 (2):149-60, doi: 10.1056/NEJMcp1113996 172 The Quality of Life-Related to Social Stigma is Worse in the Less Developed Region in Comparison More Developed Region in Brazil Cleuton Landre (Macapá, Brazil), Natalia Iosimuta (Macapá, Brazil), Kátia Nóbrega (Macapá, Brazil), Lorane Melim (Macapá, Brazil), Fernanda Miyahara (S~ ao Paulo, Brazil), Maria Elisa Piemonte (Sao Paulo, Brazil) Objective: To compare the QoL related to social Stigma in people living with PD in the highly developed and low develop region of Brazil. Background: The association between motor and non-motor symptoms of Parkinson’s disease leads to a decrease in quality of life the people living with the disease. Besides the intrinsic factor of disease, extrinsic factor like social Stigma, i.e., negative public attitudes, can contribute to a reduction in QoL. Social Stigma arises when a person has a characteristic often considered negative by society. This negative stereotype can lead to suffering from prejudice and discrimination. The social Stigma may relate to the level of the region’s development. Methods: Participated this study 36 people with conﬁrmed diagnosis of PD, mean age of 76.52 (SD=10) years, 11.19 (SD=4.5) years of education, mean scores of 19.72 (SD=4.7) in Montreal Cognitive Assessment and mean scores de 4.82 (SD=3.3) in Geriatric Depression Scale, 19 of them living in Sao Paulo, a city of most developed region of Brazil (Southeast region), and 17 of them living in Macapa, a city of less development region of Brazil (North region). All participants were evaluated in an individual section by Uniﬁed Parkinson’s disease rating scale (UPDRS) and 39-item Parkinson’s Disease Questionnaire (PDQ-39), a validated and widely used scale for assessing health-related quality of life in PD. This scale has eight domains, among of the Stigma. The Stigma domain consists of the following questions: "Due to Parkinson’s disease, how many times during the past month have you: 1) Felt you had to hide the disease from other people? 2) Avoided situations that involved eating or drinking in public? 3) Felt embarrassed in public? 4) Felt concerned about other people’s reactions to you?" Each question is scored from zero (never) to four (always). The scores range from 0 to 100, with higher scores indicating poorer quality of life. The results of total scores and the sub scores in each domain were analyzed by one-way ANOVA considered as the main factor the city (Sao Paulo and Macapa). Results: The results showed that there was no signiﬁcant difference between the groups in age, gender, years of education, cognition capacity (according to MoCA) and depressive symptoms (according to GDS), level of independence in daily living activity (according to UPDRS - section II) and severity of motor symptoms (according to UPDRS section III). However, there was a signiﬁcant difference in scores of stigma domain in PDQ-39 (F1, 22=7.9, p=.009, ES= .8), conﬁrmed by Tukey post-hoc test that showed the signiﬁcant higher score for people living with PD in Macapa in comparison Sao Paulo (p=.01), regardless of the difference statistic signiﬁcantly in the total scores or in the other domains of PDQ-39. Conclusions: The quality of life-related to social Stigma is worse in the region less developed in Brazil, despite the severity of motor symptoms, depression, age, gender education and cognitive capacity. The public policy to reduce the social Stigma is fundamental considering that hen these negative public attitudes are believed by the person with an unfavorable condition, "self-stigma" occurs, which is usually associated with decreased self-esteem and self-efﬁcacy and reduced quality of life. 173 Clinical Correlations of Parkinson’s Disease and Vascular Parkinsonism: Retrospective Review from Uzbekistan Azamatjon Umarov (Tashkent, Uzbekistan), Nodirjon Bakhodirov (Tashkent, Uzbekistan), Dilnoza Tursunbaeva (Tashkent, Uzbekistan) Objective: We aimed to study evidence for or against the role of narrowing lesions of the main brain arteries in progressing of parkinsonism, and to identify clinical signs that suggest a vascular origin. Methods: We provide a Retrospective review in history materials of 231 patients with Parkinson’s disease (PD) and Vascular Parkinsonism (VP), 46 and 185 respectively; for the period from 2014 to 2018y. We divided the patients to 2 groups (VP and PD), and compared the clinical features. Results: The both groups were differentiated in terms of evidence of cerebrovascular disease (P<.001 to P<.00001). Patients with VP were older, more likely to present with gait difﬁculty rather than tremor, and less likely to respond to the use of levodopa compared with patients with PD (P<.00001). Patients with VP were also signiﬁcantly more likely to have predominant lower body involvement, postural instability, a history of falling, dementia, corticospinal ﬁndings, incontinence (P<.00001), and pseudobulbar effect (P<.05). Conclusions: These differences in clinical features suggest a different pathogenesis of parkinsonism in these 2 patient groups. The strong evidence of cerebrovascular disease in the VP group and the differences in clinical features support the concept of VP as a distinct clinical entity. We conclude that compared with PD, patients with parkinsonism associated with vascular disease are more likely to present with gait difﬁculty and postural instability rather than tremor, have a history of stroke and risk factors for stroke, and fail to respond to levodopa therapy. This abstract was presented at the XXIII World Congress on Parkinson’s Disease and Related Disorders in August 2018. 174 Timing of Diabetes Diagnosis and its Inﬂuence on the Age of Parkinson’s Disease Onset Oscar Esquivel-Zapata (Monterrey, Mexico), Emmanuel Escobar-Valdivia (, Mexico), Jose David Garcia Romero (Monterrey, Mexico), Angel Alcocer Salas (Ciudad de Mexico, Mexico), Amin Cervantes (Mexico City, Mexico), Mayela Rodriguez Violante (Mexico City, Mexico), Cynthia Sarabia-Tapia (Mexico City, Mexico), Susana Lopez-Alamillo (Villahermosa, Mexico), Yazmin Rios (Ciudad de Mexico, Mexico), Omar Cardenas (Ciudad de Mexico, Mexico), Rodolfo Abundes-Corona (Mexico City, Mexico), Fanny Herrera (San Pedro Sula, Honduras) Objective: To identify if Diabetes Mellitus (DM) in patients with Parkinson’s disease (PD) has an effect in the age of onset, PD duration, motor and non-motor symptoms and quality of life. Background: Several common degeneration pathways between PD and DM have been described. (1) It has been shown that patients with DM are associated with a worse motor symptoms and faster disease progression. (2) Methods: Cross-sectional, observational study. Patients with PD and DM were analyzed. We randomly selected 2:1 patient from the REMEPARK (Mexican’s Parkinson’s Disease Registry) to conform the PD without DM group. We subdivided the PD and DM group into MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S77 ABSTRACTS 2 groups, according to the time of diagnosis of DM, DM prior to the onset of PD, and DM after the onset of the PD. We included patients with H&Y lower than 3. Kolmogorov-Smirnov test was used to test normality. We compared groups using a X2 or Kruskal-Wallis test for categorical or numerical variables. We performed the Bonferroni multiple comparison test to calculate the p values for each variable. Results: 114 patients with PD and DM were include, and 697 patients with PD without DM, 228 were randomly selected to accomplish 2:1. We found that the DM prior PD group had an older age of onset (64.5, 15-94) than the DM post PD group (53.5, 26-81) (p<0.05) and the PD without DM group (54, 17-82) (p<0.05). We found that the DM post PD group had a longer time with PD (9, 3-28) than the DM prior PD group (5, 0-16) (p<0.05) and the PD without DM group (6,0-33) (p<0.05). [Table 1] Conclusions: We found that patients with PD that developed DM prior the onset of PD tend to have a later age of onset compared to patients with DM after the onset of PD and with PD without DM, which can suggest that DM can be a factor that delay the onset of the disease. A subsequent follow-up is planned, to evaluate the progression of the disease, and to identify if in a more advanced H&Y there is a difference in the progression of the disease, so we can be able to demonstrate with greater certainty whether the development of DM prior the onset of PD could be consider as a factor that delays the onset of the PD. References: 1. Aviles-Olmos I, Limousin P, Lees A, Foltynie T. Parkinson’s disease, insulin resistance and novel agents of neuroprotection. Brain. 2013;136(2):374–84. 2. Pagano G, Polychronis S, Wilson H, Giordano B, Ferrara N, Niccolini F, et al. Diabetes mellitus and Parkinson disease. Neurology. 2018;10.1212/ WNL.0000000000005475. Methods: Participated this study 49 people with conﬁrmed diagnosis of PD, mean age of 66.91 (SD=9.6) years, 10.55 (SD=4.5) years of education, mean scores of 24 (SD=5) in Montreal Cognitive Assessment and mean scores de 5.88 (SD=3.4) in Geriatric Depression Scale, 19 of them living in Sao Paulo, the most urbanized city of Brazil, included in the Southeast region group (SEG), and 17 and 15 of them living in Macapa and Manaus respectively, cities of low level of urbanization in Brazil, included in the North region group (NOG). All participants were evaluated in an individual section by Uniﬁed Parkinson’s disease rating scale (UPDRS) to assess the independence in daily living activities and severity of motor symptoms, FTSTS to assess lower-extremity strength, 6-min walk distance test (6WT) to assess functional mobility, associated to Borg scale (BS) to assess the of Perceived Exertion. The results obtained with each test was analyzed by one-way ANOVA considered as the main factor the group (SEG and NOG). Results: The results showed that there was no signiﬁcant difference between the groups in age, gender, years of education, cognition capacity (according to MoCA) and depressive symptoms (according to GDS), level of independence in daily living activity (according to UPDRS section II) and severity of motor symptoms (according to UPDRS section III) and the maximal distance walked in the 6WT. However, there was a signiﬁcant difference in the total time in the FTSTS (F 1,45=6.5, p=.01, ES= .8), conﬁrmed by Tukey post-hoc test that showed the signiﬁcant higher time for the SEG in comparison to NOG (p=.01). Additionally, there was a signiﬁcant difference in the BS at the beginning of 6WT (F 1,45=9.4, p=.00, ES= .9), conﬁrmed by Tukey post-hoc test that showed that the level of perceived exertion was signiﬁcantly higher in SEG than NOG (p=.01). Conclusions: The decrease in the lower-extremity strength is higher in people living in the more urbanized regions, despite the severity of motor symptoms, level of independence in daily living activity, depression, age, gender education and cognitive capacity. Although the decreased lower-extremity strength was not associated with functional mobility, it may affect the perceived exertion before the physical activity, leading to decreased physical activity, establishing a vicious circle. Thus, should be included exercises to increase the lower-extremity strength among the goals of the physiotherapeutic interventions. 176 Neuroleptic-like Malignant Syndrome in a Patient with Deep Brain Stimulation for Parkinson Disease for Battery Depletion: Case Report Juliana Bejarano (Bogota, Colombia), MIguel Ayala (Bogtoa, Colombia), Claudia Moreno Lopez (Bogota, Colombia), Xiomara Garcia (Bogotá, Colombia) 175 The Lower-Extremity Strength and Perceived Exertion are Less Impaired in People Living with Parkinson’s Disease in the Amazon Region than in More Urbanized Areas of Brazil Renato Freire (Manaus, Brazil), Cleuton Landre (Macapá, Brazil), Rafael Azamor (Manaus, Brazil), Natalia Iosimuta (Macapá, Brazil), Rafaella Rodrigues (Manaus, Brazil), Kátia Nóbrega (Macapá, Brazil), Rayanara Dos Santos (Manaus, Brazil), Lorane Melim (Macapá, Brazil), Fernanda Miyahara (S~ ao Paulo, Brazil), Maria Elisa Piemonte (Sao Paulo, Brazil) Objective: To compare the Five Times Sit-to-Stand Test (FTSTS) performance in people with Parkinson’s disease living in regions with different level of urbanization in Brazil. Background: Parkinson’s disease causes several primary motor and non-motor alterations that lead to decreased mobility. The reduction of mobility causes secondary alterations as a reduction in the lowerextremity strength, i.e., the reduced force-generating capacity of muscles, especially at the hip, which may contribute to the ability to ascend from a chair. Sit-to-Stand Test is commonly used to assess lower-extremity strength. The level of urbanization may be an additional factor to contribute to mobility reduction. S78 Background: Neuroleptic malignant syndrome (NMS) is an infrequent yet life threatening condition that was initially described by Delay and colleagues in 1960, shortly after the introduction of antipsychotic medications. There is neuroleptic like malignant syndrome also called parkinsonism hyperpyrexia syndrome and is a severe clinical condition often fatal due to the sudden suspension of antiparkinsonian drugs, sudden depletion battery of the deep brain stimulation (DBS) or during the postoperative period of the DBS. It is characterized by altered state of consciousness, fever, rhabdomyolysis, rigidity and autonomic dysfunction, among other clinical features. Results: We describe a clinical case of a 68-year-old male patient with a history of Parkinson’s disease diagnosed at age 55, who went through multiple pharmacological therapies until requiring DBS management for the last 8 years and presented a signiﬁcant improvement in his functionality and quality of life. The patient present a sudden stiffness of the 4 limbs, general tremor, impaired state of consciousness and fever, so he enters the emergency department 24 hours after the onset of symptoms, where document elevation of renal function, marked elevation of total CPK, considering that it has a Neuroleptic-like Malignant Syndrome leading to renal failure, with prolonged stay in the ICU with subsequent use of alternative feeding route, most of the symptoms resolved after the placement of the new battery to DBS however ultimately generating marked deterioration with respect to its state of base with a total dependency. Conclusions: In general, the most frequent cause of neuroleptic like malignant syndrome is attributed to the abrupt suspension of medications MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS and on other occasions due to abrupt battery loss of DBS, this syndrome can be fatal or leave irreversible sequelae in the patient, acute management for the Syndrome could improve the patient’s life expectancy and reduce the possible sequelae. Therefore, it should be noted that this pathology must be taken into account in this type of patients and never leave the battery lost. References: Sauer, T., Wolf, M., Blahak, C., Capelle, H., & Krauss, J. (2017). Neuroleptic-like Malignant Syndrome After Battery Depletion in a Patient with Deep Brain Stimulation for Secondary Parkinsonism. Movement Disorders Clinical Practice, 4(4), 629-631. Urasaki E et al. Neuroleptic malignant syndrome (parkinsonism–hyperpyrexia syndrome) after deep brain stimulation of the subthalamic nucleus. J Clin Neurosci (2013). Themistocleous et al.: Malignant neuroleptic syndrome following deep brain stimulation surgery: a case report. Journal of Medical Case Reports 2011 5:255. 177 Impact of Saﬁnamide on Non-Motor Experiences of Daily Living: Interim Findings from a Prospective, Observational Study Stuart Isaacson (Boca Raton, FL, United States), Thomas Clinch (Louisville, KY, United States), Eric Farbman (Henderson, NV, United States), Rajesh Pahwa (Kansas City, KS, United States) Objective: In this interim analysis, we investigated the effect of saﬁnamide treatment on non-motor and motor experiences of daily living as assessed using the MDS-UPDRS (Parts I and II). Background: Saﬁnamide is an alpha-aminoamide with both dopaminergic and non-dopaminergic (glutamate inhibition) mechanisms of action. While the efﬁcacy of saﬁnamide in improving ON time has been established, its effectiveness on non-motor symptoms is less clear. Methods: This is an open label study in PD patients (30-80 years) with motor ﬂuctuations treated with saﬁnamide for 2 months, and with an optional 4-month extension (NCT03944785). The decision to prescribe saﬁnamide is made in accordance with the US package insert, and independently from, the decision to enroll the patient in this study. Patients can either be naïve to previous MAO-B inhibitor treatment or switched from adjunct therapy with a dopamine agonist or previous MAO-B inhibitor. The MDS-UPDRS is assessed at baseline and Day 60. Results: As of August 2019, 91 patients have a baseline and postbaseline assessment. Overall, 70% of patients are male and the mean SD age is 67 8 years. MeanSD time since diagnosis is 5.5 4.2 years and mean levodopa dose is 585mg. As early as Day 60, MDS-UPDRS Part I and Part II subscores were reduced from baseline [Table1]. Analysis of nM-EDL subitems at day 60 indicated that all 13 non-motor items were either improved or maintained. Key drivers for overall non-motor improvement were sleep and pain and other sensations (mean SD change from baseline to day 60: -0.5 1.2 & -0.4 1.1, respectively). Conclusions: Interim analysis of this prospective open label study indicate an effect of saﬁnamide treatment on non-motor and motor experiences of daily living, especially sleep and pain and other sensations. 178 Niacin Reduces Inﬂammatory Cytokine Load in Parkinson’s Disease Chandramohan Wakade (Augusta, GA, United States) Objective: We evaluated pro- and anti-inﬂammatory cytokines in CSF and plasma of Parkinson’s disease (PD) patients at baseline and after niacin intervention. Background: PD is a neurodegenerative disorder characterized by progressive loss of dopaminergic neurons. Sustained release of proinﬂammatory molecules by microglia is responsible for the progression of neurodegeneration of PD. Results from our previous clinical trial suggests that supplementation of niacin changes the pro-inﬂammatory phenotype to anti-inﬂammatory phenotype by altering the production of cytokines and chemokines. Methods: 25 individuals diagnosed with idiopathic PD were randomized to receive either placebo or niacin (250 mg daily for six months). Subjects were assessed for motor and non-motor symptoms along with cytokine proﬁles in CSF and plasma before and after the supplementation. Results: Following niacin intervention, patients recorded lower UPDRS scores compared to the scores at baseline. Due to small sample size we did not detect any coorealtion with UPDRS III score and the cytokine proﬁle. Of the nine cytokine molecules analyzed, IL-2 (0.12 vs 0.082 pg/ml at baseline) and IL-1 (0.068 vs 0.015 pg/ml) were signiﬁcantly increased in the CSF, whereas levels of IFN? and IL-8 showed a downward trend in CSF. The IFN? in plasma showed a downward trend as well. Il-10 and IL-6 did not show signiﬁcant changes either in CSF or in plasma. IL-2 levels were signiﬁcantly lower in both CSF and plasma compared to baseline. IL-13 showed an upward trend both in CSF and plasma compared to baseline. Conclusions: Overall niacin reduced the levels of pro-inﬂammatory cytokines in PD subjects. Niacin has also decreased IL-8 in both CSF and plasma of PD subjects which is pro-inﬂammatory and is probably involved in macrophage recruitment crossing blood-brain-barrier. Collectively these results suggest that niacin intervention is helpful in PD in reducing the pro-inﬂammatory load. References: J Neuroimmunol. 2018 Jul 15;320:76-79. doi: 10.1016/ j.jneuroim.2018.05.002. Epub 2018 May 4. Niacin modulates macrophage polarization in Parkinson’s disease. Wakade C, Giri B, Malik A, Khodadadi H, Morgan JC, Chong RK, Baban B 179 Does Dopamine Precursor Interfere in Action Prediction Function in Parkinson Disease? Anaelli Nogueira-Campos (Juiz de Fora, Brazil), Amanda Chagas (Juiz de Fora, Brazil), Isabella Faria (Juiz de Fora, Brazil), Ruben Ernesto Navarrete (Juiz de Fora - MG, Brazil), Thiago Vale (Juiz de Fora, Brazil), Ghislain Saunier (Belém, Brazil) Objective: This study aimed to investigate the role of basal ganglia in the predictive function of the motor system and the inﬂuence of the dopamine intake in this process. Background: Action execution and observation imply in a predictive control, which ensures the anticipation of sensorimotor upcoming events. The basal ganglia seems to be part of this circuitry, however, experimental evidence is still scarce. Methods: Twelve individuals with Parkinson’s disease (PD) (5810.3 years old) in the ON medication phase and eight healthy controls (54.511 years old) were recruited. Biological and non-biological videos depicted hand actions were presented. Participants should reconstruct the observed actions in chronologically or in reverse order based on four frames taken from the same videos. Biological videos began with the actor’s hand in the resting position. The hands moved to the object and ended when touched it. Consequently, the expected and natural action step after interaction with the object was the return of the hand to the resting position. The accuracy, corresponding to the rate of correct trials was recorded. Results: Repeated-measures ANOVA revealed neither signiﬁcant difference in accuracy for PD ([F(1,11)=2.92, p=0.12; n2p=0.21; ß=0.35]) and for control group ([F(1,7)=0.59, p=0.47; n2p=0.08; ß=0.1])), nor between them. In the PD group, correlation analysis showed that the mean accuracy per subject negatively correlated with the Hoehn & Yahr scores (P<0.05; r =-0.68) and the Beck depression scale score (P<0.05; r=- 0.66). We found no signiﬁcant correlations between accuracy per subject with UPDRS III (P>0.05; r=-0.41) and Mini Mental (P>0.05; r=0.48). In the control group signiﬁcant correlations were found with Mini Mental (P<0.05; r=0.85) but no with Beck score (P>0.05; r=0.46). Conclusions: There was no modulation of action prediction function in both groups. The present results may be related to the dopamine precursor’s intake, which play an important role in the maintenance of MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S79 ABSTRACTS dopaminergic circuits. Also, the negative correlation between Hoehn & Yahr scale score and accuracy may reﬂect that the impairment in the performance of tasks is associated to the motor repertoire. These ﬁndings may contribute to develop an action observation therapy based on the improvement of the predictive function. Finally, the recruitment of the same patients at OFF time of the medication could show whether dopamine replacement is indeed capable of normalizing that function. References: ALEGRE, M.; RODRÍGUEZ-OROZ, M.C.; VALENCIA, M.; PÉREZ-ALCAZAR, M.; GURIDI, J.; IRIARTE, J.; OBESO, J. A.; ARTIEDA, J. Changes in subthalamic activity during movement observation in Parkinson’s disease: Is the mirror system mirrored in the basal ganglia? Clinical Neurophysiology, v. 121, p. 414–425, 2010. BECK A.T.; WARD, C.H.; MENDELSON, M.; et al. An inventory for measuring depression. Arch Gen Psychiatry, v. 4, p. 561-571, 1961. DONNARUMMA, F.; COSTANTINI, M.; AMBROSINI, E.; FRISTON, K.; PEZZULO, G. Action perception as hypothesis testing. Cortex, v. 89, p. 45-60, 2017. FOLSTEIN, M. F.; FOLSTEIN, S. E.; MCHUGH, P. R. ‘Mini-mental state’: a practical method for grading the cognitive state of patients for the clinician. Journal of Psychiatric Research, v. 12, n. 3, p.189-198, 1975. FRISTON, K. J. Learning and inference in the brain. Neural Network, v. 16, p. 1325–1352, 2003. GOETZ, C.G; FAHN, S.; MARTINEZ-MARTIN, P.; et al. Movement Disorder Society sponsored revision of the Uniﬁed Parkinson’s Disease Rating Scale (MDS-UPDRS): process, format, and clinimetric testing plan. Mov Disord, v. 22, p. 41–47, 2007.HOEHN, M.M.; YAHR, M.D. Parkinsonism: onset, progression and mortality. Neurology, v.17, p.427–442, 1967. 180 Teraupetic Conduct and Response in Patients with Impulse Control Disorder, in Parkinson’s Disease Fanny Herrera (San Pedro Sula, Honduras), Amin Cervantes (Mexico City, Mexico), Arturo Abundes-Corona (Mexico City, Mexico), Emmanuel EscobarValdivia (, Mexico), Omar Cardenas (Ciudad de Mexico, Mexico), Angel Alcocer Salas (Ciudad de Mexico, Mexico), Susana Lopez-Alamillo (Villahermosa, Mexico), Yazmin Rios (Ciudad de Mexico, Mexico), Cynthia Sarabia-Tapia (Mexico City, Mexico), Oscar Esquivel-Zapata (Monterrey, Mexico), Mayela Rodriguez Violante (Mexico City, Mexico) Objective: To determine the choice of therapeutic strategies and its success rate in the management of impulse control disorders (ICD) in a sample of patients with Parkinson’s disease (PD). Background: Common strategies for the treatment of ICD include: dopamine agonist (DA) dose reduction, DA switch, DA release formulation switch or DA withdrawal. Nevertheless, frequency of choice and success rate of these strategies have been scarcely reported.1Some experts propose the use of neuroleptics in the management of refractory ICDs, no clear evidence indicates that the addition of these or other psychotropic medications without concomitant reduction of anti-parkinsonian medication is effective in resolving ICDs.2 Methods: A longitudinal retrospective study was carried out. Patients with PD evaluated with the QUIP-RS at least in two separated visits (V0, basal and V1, 6-month follow-up). Published cut-off values for individual ICDs were used. Data on antiparkinsonian drugs and LEDDs were collected. Differences on treatment, dosage, QUIP-RS score and the presence/absence of ICD between visits were analyzed. Results: A total of 217 (54% male) patients were included. The mean age was 67 12 years. Table 1 summarizes the comparison of the main variables between the initial and follow-up visit. At V0 a total of 46 had an ICD. The most common stratey was decrease the DA dosage (8.7%). Table 2 compares the QUIP-RS and drug treatment in the patients with ICD at visit 0 and 1. Ten patients without ICD at visit 0 developed ICD on follow-up. Conclusions: The prevalence of ICD was 21%. A reduction of 79% on ICD was found between visits. References: 1.Ramirez A et al. Treatment of impulse control disorders in Parkinson’s disease: Practical considerations and future directions. Expert Review of Neurotherapeutics. 2016. ISSN: 1473-7175.2.Samuel M et al. Management of Impulse Control Disorders in Parkinson’s Disease: Controversies and Future Approaches. Movement Disorders, 2015. Table 1. Comparison between the initial and follow-up visit DOPAMINE AGONIST (%) ICD (n, %) LEDD TOTAL (mg) LEDD-DA (mg) QUIP-RS total GAMBLING COMPULSIVE SHOPPING HYPERSEXUALITY COMPULSIVE EATING HOBBYISM PUNDING UPDRS III NMS TOTAL INITIAL VISIT Frequency 138 (63.5%) 46 (21%) Median 650.0 150.0 Mean 4.74 0.08 0.67 0.44 1.34 1.26 0.64 28.60 46.63 Rank 0.0-2100 0.0-600.0 SD 8.580 0.422 2.147 1.779 3.285 2.995 2.134 13.879 40.092 FOLLOW-UP VISIT Frequency 149 (68.6%) 21 (9.6%) Median 780.0 150.0 Mean 2.36 .04 0.47 0.32 0.71 0.32 0.33 26.92 40.77 Value p Rank 0.0-2500 0.0- 650.0 SD 6.133 0.523 2.145 1.995 2.612 1.660 1.531 13.770 44.440 0.117 0.000 Value p 0.000 0.065 0.000 0.182 0.288 0.514 0.009 0.000 0.033 0.043 0.090 Table 2. Treatment strategy in the ICD group ICD (n, %) ON DOPAMINE AGONIST ON PRAMIPEXOLE IR ON PRAMIPEXOLE ER ON ROTIGOTINE LEDD TOTAL LEDD-DA QUIP-RS TOTAL S80 INITIAL VISIT Frequency 46 (100%) 37 (80.4%) 17 (36.9%) 11 (23.9%) 9 (19.5%) Median 925.0 300.0 Mean 18.07 Rank 0.0-2100.0 0.0- 600.0 SD 9.876 FOLLOW-UP Frequency 10 (21%) 33 (71.7%) 19 (41.3%) 9 (19.5%) 5 (10.8%) Median 962.0 232.5 Mean 4.52 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 Value p Rank 0.0- 2500.0 0.0-650.0 SD 7.515 0.000 0.289 0.687 0.687 0.125 Value p 0.591 0.093 Value p 0.000 ABSTRACTS 181 Cursive Versus Printed Letter for Diagnosis of Progressive Micrographia in Parkinson’s Disease Cristian Calandra (Buenos Aires, Argentina), Cynthia Garcia Fernandez (Capital Federal, Argentina), Adriana Ziliotto (Buenos Aires, Argentina), Gabriela Raina (Buenos Aires, Argentina), Ricardo Maiola (Buenos Aires, Argentina), Nicolas Morera (Buenos Aires, Argentina), Maria Cersosimo (Buenos Aires, Argentina) Objective: We performed a prospective study to determine if cursive or printed in lower or upper-case letters during writing inﬂuences the occurrence of progressive micrographia in PD patients. Table 1. Size: cursive lower-case vs printed upper-case. Cursive lower-case Letter PD patients (n= 55) Meassured Median Min e1 (mm) 3 1 e10 (mm) 2,5 0 m1 (mm) 6 2,5 m10 (mm) 6,5 1,5 Max 5,5 6 12,5 22,5 IQR 2 1 2,5 3,5 Controls (n= 57) Median Min 3,5 1,5 3 0 7,5 5 8 5,5 Max 7,5 6,5 12,5 13 IQR 1,25 1 2 2 Printed upper-case Letter PD patients (n= 35) Meassured Median Min E1 (mm) 3,5 2 E10 (mm) 3 1 M1 (mm) 3,5 2,5 M10 (mm) 4 1,5 Max 7,5 7 8,5 8,5 IQR 2 1,5 2,5 3 Controls (n= 23) Median Min 3,5 2,5 3,5 2 4 2,5 4,5 2,5 Max 25 5 7 7 IQR 1 1,5 1,5 2 p value 0.01 0.006 0.000 0.000 p value ns ns ns ns HR= handwriting; PD= Parkinson’s disease; ns= not signiﬁcant; Min= minimum; Max= maximum; IQR= interquartile range; mm= millimeters; cm= centimeters; e1= height of ﬁrst cursive letter e; e10= height of last cursive letter e; m1= width ﬁrst cursive letter m; m10= width of last cursive letter m; E1= height of ﬁrst capital letter e; E10= height of last capital letter e; M1= width of ﬁrst capital letter m; M10= width of last capital letter m Table 2. Gradual reduction in size: cursive lower-case Cursive lower-case Variable Width of letter “m” Height of letter “e” Percentage of decrement ≥5 ≥ 10 ≥ 20 ≥ 30 ≥ 40 ≥ 50 ≥ 60 ≥5 ≥ 10 ≥ 20 ≥ 30 ≥ 40 ≥ 50 ≥ 60 PD (n= 55) n 23 18 12 8 6 1 0 32 31 19 16 11 5 4 % 41,8 32,7 21,8 14,5 10,9 1,8 0 52,5 50,8 31,1 26,2 18 8,2 6,6 Controls (n= 57) n 13 9 3 0 0 0 0 26 25 16 12 7 3 2 % 22,8 15,7 5,3 0,00 0,00 0,00 0 45,60 43,90 28,10 21,10 12,30 5,30 3,50 p value 0.04 0.03 0.01 0.003 0.01 ns ns ns ns ns ns ns ns PD (n= 35) n 14 14 12 6 4 2 1 21 21 18 12 7 4 2 % 40 40 34,3 17,1 11,4 5,7 2,9 60 60 51,4 34,3 20 11,4 5,7 Controls (n= 23) n 6 6 5 2 1 0 0 11 11 10 5 3 2 1 % 26,1 26,0 21,7 8,7 4,3 0 0 47,8 47,8 43,5 21,7 13 8,7 4,3 p value ns ns ns ns ns ns ns ns ns ns ns ns ns ns Tabla 3. : Gradual reduction in size: printed upper-case Printed upper-case Variable Widht of letter “M” Height of letter “E” Percentage of decrement ≥5 ≥ 10 ≥ 20 ≥ 30 ≥ 40 ≥ 50 ≥ 60 ≥5 ≥ 10 ≥ 20 ≥ 30 ≥ 40 ≥ 50 ≥ 60 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S81 ABSTRACTS Background: Progressive micrographia consists of a gradual reduction in the size of the letters during writing and is a clinical feature of Parkinson’s disease (PD). The letters used in writing might be cursive or printed and lower or upper-case. Methods: PD patients and control subjects were asked to write a same phrase ten times. Based on their own preference, they could write in cursive or printed letter. The width and height of the ﬁrst and last letter “m” and “e” were measured. Progressive micrographia was considered when the size of the last “m” and “e” was smaller than the ﬁrst one. Reductions in size between 5 and 60 % were calculated. The UPDRSIII and HY were administrated to the PD patients. Results: Ninety PD patients and 80 healthy controls were included. Sixty-one percent of PD patients used cursive lower-case and 38.9% printed upper-case. In the control group, 71.3% and 28.7% used cursive lower-case and printed upper-case, respectively. PD patients that wrote with cursive lower-case had a signiﬁcantly smaller size of the letter. There was no statistically signiﬁcant difference in the size in both groups that wrote with printed upper-case. The gradual reduction in the size of writing was observed only among patients that used cursive lower-case. This feature was more evident in the PD group. Patients and controls that used printed upper-case did not show signiﬁcant differences in progressive micrographia frequency. Conclusions: Cursive lower-case HW was sensitive to demonstrate progressive micrographia in PD patients. On the other hand, printed upper-case HW did not show any differences between patients and controls in any measurement. References: Inzelberg R, Plotnik M, Harpaz NK, Flash T. Micrographia, much beyond the writer’s hand. Parkinsonism Relat Disord. 2016 May;26:1-9. doi: 10.1016/j.parkreldis.2016.03.003. Epub 2016 Mar 11. Wu T et al. Neural correlates underlying micrographia in Parkinson’s disease. Brain 2016 Jan;139(Pt 1):144-60. doi: 10.1093/ brain/awv319. Epub 2015 Nov 2 Zham P, Raghav S, Kempster P, Poosapadi Arjunan S, Wong K, Nagao KJ, Kumar DK. A Kinematic Study of Progressive Micrographia in Parkinson’s Disease. Front Neurol. 2019 Apr 24;10:403. doi: 10.3389/fneur.2019.00403. eCollection 2019. Thomas M, Lenka A, Kumar Pal P. Handwriting Analysis in Parkinson’s Disease: Current Status and Future Directions. Mov Disord Clin Pract. 2017 Nov 1;4(6):806-818. doi: 10.1002/mdc3.12552. 182 Research Participation in Parkinson Disease Daniel Garbin Di Luca (Miami Beach, FL, United States), Chantale Branson (Atlanta, GA, United States), Angie Sanchez (Boston, MA, United States), Meaghan Holzworth (Miami, FL, United States), Corneliu Luca (Miami, FL, United States), Allison Willis (Philadelphia, PA, United States) Objective: (1) Presently, to build a network of individuals interested in sociodemographic differences in PD risk/ outcomes, (2) Ultimately, this network will conduct research using diverse PD cohorts to detect meaningful differences across demographic subgroups. Background: Women, minorities and adults ages70+ at diagnosis are underrepresented in PD research trials, and disproportionately experience negative consequences of non-guideline adherent care. Methods: The collaborative network will be built via one-on one mentoring opportunities supported by professional and academic neurology associations and Parkinson disease foundations, led by an academic PD clinician researcher with experience in distance mentoring and expertise in clinical PD epidemiology. Collaborator interest was gauged at a 2019 meeting of academic PD researchers and stakeholders. As an initial task, members of the newly formed coalition were charged with enrolling PD patients to participate in a survey study focused on understanding the drivers of research participation. After three months, initial barriers to participation and activation were discussed by members of the coalition and descriptive analysis of early enrolling participants was performed. Study data were collected and managed using REDCap electronic data capture tools hosted at the University of Pennsylvania. Results: Two of the four candidate network sites were able to activate within three months and completed data entry for 183 participants. Primary barriers to activation and patient enrollment were institutional regulatory processes, not expressed or perceived patient interest. Of the early participants, 68.4% were female, 25.7% did not have a college S82 degree; 4.1% were Hispanic; 10% were minority race. Average time since physician diagnosis of PD was 6.57 (SD 5.35) years, and average time since ﬁrst noticed PD symptom was 9.4 (SD 6.01) years. Of note, 35.3% stated they had never been asked to participate in PD research before, and 62.4% were interested in opportunities to participate in PD biomarker, genetic and pharmacogenetic research. Conclusions: Understanding and addressing the reasons for underrepresentation of key demographic groups in PD research is urgently needed to complete our understanding of disease processes and treatment response and improve clinical outcomes. Multi-center networks consisting of invested stakeholders from academia and private foundations may be needed to assemble population representative PD cohorts, although individual institutional barriers will need to be addressed to standardize recruitment times and achieve shared recruitment goals. Understanding and addressing the reasons for underrepresentation of key demographic groups in PD research is urgently needed to complete our understanding of disease processes and treatment response and improve clinical outcomes. Multi-center networks consisting of invested stakeholders from academia and private foundations may be needed to assemble population representative PD cohorts, although individual institutional barriers will need to be addressed to standardize recruitment times and achieve shared recruitment goals. References: 1. Dahodwala N, Shah K, He Y, et al. Sex disparities in access to caregiving in Parkinson disease. Neurology 2018;90:e48-e54. 2. Fullard ME, Thibault DP, Todaro V, et al. Sex disparities in health and health care utilization after Parkinson diagnosis: Rethinking PD associated disability. Parkinsonism Relat Disord 2018;48:45-50. 3. Willis AW, Schootman M, Evanoff BA, Perlmutter JS, Racette BA. Neurologist care in Parkinson disease: a utilization, outcomes, and survival study. Neurology 2011;77:851-857. 4. Willis AW, Schootman M, Kung N, Wang XY, Perlmutter JS, Racette BA. Disparities in deep brain stimulation surgery among insured elders with Parkinson disease. Neurology 2014;82:163-171. 5. Willis AW, Schootman M, Tran R, et al. Neurologist-associated reduction in PDrelated hospitalizations and health care expenditures. Neurology 2012;79:1774-1780. 6. PA Harris, R Taylor, R Thielke, J Payne, N Gonzalez, JG. Conde, Research electronic data capture (REDCap) – A metadata-driven methodology and workﬂow process for providing translational research informatics support, J Biomed Inform. 2009 Apr;42 (2):377-81.7. U.S. Census. www.cesus.gov 183 Metabolomic Analysis Identiﬁes Caffeine and its Metabolites as Plasma Markers of Resistance to Parkinson’s Disease Among LRRK2 Mutation Carriers in the LRRK2 Cohort Consortium (LCC) Grace Crotty (Cambridge, MA, United States), Junhua Wang (San Francisco, CA, United States), Manuel Montalban (San Francisco, CA, United States), Sonnet Davis (San Francisco, CA, United States), Jamal Alkabsh (San Francisco, CA, United States), Rachit Bakshi (Cambridge, MA, United States), Xiqun Chen (Boston, MA, United States), Alberto Ascherio (Boston, MA, United States), Eric Macklin (Boston, MA, United States), Romeo Maciuca (San Francisco, CA, United States), Giuseppe Astarita (San Francisco, CA, United States), Sarah Huntwork-Rodriguez (San Francisco, CA, United States), Michael Schwarzschild (Boston, MA, United States) Objective: To identify novel metabolic pathways at the intersection of LRRK2 gene biology and PD; and to discover potential markers of resistance to developing PD among LRRK2 mutation carriers (LRRK2+). Background: LRRK2 gene mutations have incomplete penetrance for PD suggesting that genetic and/or environmental modiﬁers determine whether or at what age PD will develop. Metabolomic analysis provides a powerful approach for identifying such modiﬁers. Methods: Plasma from 376 LCC subjects, who were LRRK2+/PD + (n=117), LRRK2+/PD- (n=120), LRRK2-/PD+ (n=70) and LRRK2-/PD- (n=69), was assessed by HPLC-ECD/UV and LC-MS. We quantiﬁed 307 analytes, focusing on purines and potentially related markers of lysosomal function or PD risk. For each analyte, we assessed differences among the four groups, speciﬁcally the presence of a LRRK2-PD status interaction, using ANCOVA models adjusted by age MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS and sex, with Benjamini-Hochberg p-value corrections to control the false discovery rate. Data and biospecimens used in the analyses presented in this abstract were obtained from the MJFF-sponsored LRRK2 Cohort Consortium (LCC). For up-to-date information on the study, visit www. michaeljfox.org/lcc. Results: Caffeine concentration (mean; adjusted for age and sex) was lower in PD subjects vs. unaffected controls (p<0.001), more so among LRRK2+ carriers (75%) than among LRRK2- subjects (32%), with signiﬁcant LRRK2-PD status interaction (p=0.006). Similarly, adjusted concentrations of caffeine metabolites (paraxanthine, theophylline, 1-methylxanthine) and a marker of coffee consumption (trigonelline) were lower in PD subjects vs. unaffected controls (p=0.001), more so among LRRK2+ carriers (by 61-66% for each) than among LRRK2subjects (by 16-23%), with signiﬁcant LRRK2-PD status interaction (p=0.01 for each). Conclusions: Metabolomic analysis of the LCC identiﬁed caffeine, its demethylated metabolites, and trigonelline as markers of reduced PD penetrance of pathogenic LRRK2 mutations, with stronger associations among LRRK2+ carriers than non-carriers. As these analytes are known both as correlates of coffee consumption and as neuroprotectants in animal models of PD, their links to LRRK2+ PD may reﬂect a pathophysiological predisposition to PD among LRRK2+ caffeine avoiders or direct neuroprotective effects of therapeutic relevance to LRRK2 mutation carriers. The LRRK2 Cohort Consortium is coordinated and funded by The Michael J. Fox Foundation for Parkinson’s Research. subsequently developed pronounced generalized myoclonus, worsened by intention and exacerbated by startle. “Generalized myoclonus” in adults with 22q11 has also been reported, although not associated with PD. References: •Butcher NJ, Kiehl TR, Hazrati LN, et al. Association between earlyonset Parkinson disease and 22q11.2 deletion syndrome: identiﬁcation of a novel genetic form of Parkinson disease and its clinical implications. JAMA Neurol 2013;70:1359–1366. •Mok KY, Sheerin U, Simon-Sanchez J, et al. Deletions at 22q11.2 in idiopathic Parkinson’s disease: a combined analysis of genome-wide association data. Lancet Neurol 2016;15:585–596. •Boot E, Butcher NJ, Udow S, et al. Typical features of Parkinson’s disease and diagnostic challenges with microdeletion 22q11.2. Neurology 2018;90:e2059–e2067. •Baralle D, Trump D, FfrenchConstant C, Dick DJ. Myoclonic movement disorder associated with microdeletion of chromosome 22q11. J Neurol Neurosurg Psychiatry. 2002;73:600–601. •Boot E, Butcher NJ, van Amelsvoort TA, Lang AE, Marras C, et al. Movement disorders and other motor abnormalities in adults with 22q11.2 deletion syndrome. Am J Med Genet A. 2015;167A:639–645. •Buckley E, Siddique A, McNeill A. Hyposmia, symptoms of rapid eye movement sleep behavior disorder, and parkinsonian motor signs suggest prodromal neurodegeneration in 22q11 deletion syndrome. Neuroreport 2017;28:677–681. •Strehlow V, Swinkels MEM, Thomas RH, et al. Generalized Epilepsy and Myoclonic Seizures in 22q11.2 Deletion Syndrome. Mol Syndromol 2016; 7: 239–46. 185 Parkinsonism (Secondary and Parkinsonism-Plus) 184 Myoclonus and Parkinsonism in 22q11.2 Syndrome - Expanding the Phenotype Leon Meytin (New York, NY, United States), Rachel Saunders-Pullman (New York, NY, United States), Matthew Swan (Brooklyn, NY, United States) Objective: To describe an association of generalized myoclonus with 22q11 deletion syndrome (DiGeorge syndrome) in the setting of adult onset Parkinsonism. Background: 22q11.2 deletion syndrome (DS) encompasses facial dysmorphia, mental retardation, seizures, mood disorders, endocrine & cardiac abnormalities, and myoclonus or Parkinsonism. It has not been reported with both myoclonus and Parkinsonism in adults. Methods: 50 year old man with childhood onset epilepsy and intellectual disability presented with 3 years of progressive gait dysfunction, tremor, and myoclonus, more rapidly progressing over the prior 6 months. Exam showed postural and action-induced myoclonus of the both arms superimposed with an irregular jerky postural tremor, bilateral rigidity and bradykinesia, and a slow gait with postural instability. The myoclonic jerks were irregular, large amplitude and asynchronous but no stimulus sensitivity was noted. There were also dysmorphic facial features, left facial weakness and a left wrist drop. EEGs were normal aside from mild cerebral slowing. A chromosomal microarray demonstrated a 2.54 MB deletion at chromosome 22q11.21. Results: Our patient’s Parkinsonism and myoclonus improved signiﬁcantly with treatment (levodopa 600 mg/day and levetiracetam 2000mg/ day, respectively). He did not have some of the classic features of 22q11 syndrome such as congenital heart defects or endocrine abnormalities, although the facial dysmorphism and cranial neuropathy characteristic of 22q11DS were present. The patient went undiagnosed for 50 years until being seen by a movement disorder specialist, highlighting the importance of performing chromosomal microarray in adult patients with Parkinsonism and myoclonus who have dysmorphic features or endocrine abnormalities. Conclusions: We believe this is the ﬁrst reported case of adult-onset Parkinsonism in 22q11 DS with generalized myoclonus. Myoclonus in 22q11DS has been infrequently reported in pediatric patients with Parkinsonism, adult patients with Parkinsonism on clozapine, and adult patients without Parkinsonism. Epileptic myoclonus with EEG correlate is also is a well-reported symptom in 22q11 syndrome, but not associated with Parkinsonism speciﬁcally. Myoclonus associated with Parkinsonism was ﬁrst described in a child who developed tremor at age 5 and Parkinsonism Due Dilated Virchow Robin Space Maria Segamarchi (Buenos Aires, Argentina), Sergio Rodriguez Quiroga (Buenos Aires, Argentina), Tomoko Arakaki (CABA, Argentina), Nélida Garretto (Buenos Aires, Argentina) Objective: To describe a case of atypical parkinsonism associated with dilated Virchow Robin Space (VRS). We also brieﬂy review the literature and comment on its possible neurophysiological mechanisms. Background: VRSs are perivascular compartments surrounding small vessels within the central nervous system. Typical VRSs are asymptomatic, but occasionally, they could cause speciﬁc clinical manifestations depending on location and the degree of tissue compression Methods: Report of a 66-year-old woman who developed unilateral left parkinsonism due to the presence of a dilated VRS in the right basal ganglia. Results: The patient presented to our movement disorders center with a 3-years history of left-hand progressive tremor. She was previously diagnosed with Parkinson Disease and received treatment with dopamine agonists for 6 months, but she decided to stop it due to lack of response. At clinical examination, there was left mild rigidity and bradykinesia in upper and lower limbs. Moderate resting tremor and mild postural and action tremor in the left hand was present. Postural stability and gait were MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S83 ABSTRACTS normal. UPDRS part III score of 9 points: left-sided items = 9/rightsided items = 0.Brain MRI showed a large VRS (diameter of 14 mm) in the right basal ganglia at the level of the lenticulostriate vessels. The transcranial sonography was normal. The Levo-Dopa test was negative (no changes in UPDRS III score). Conclusions: We believe, that in this case, parkinsonism could be associated with the mass effect of a signiﬁcantly enlarged VRS. Features in support of this hypothesis include the location of the isolated dilated VRS, its signiﬁcant size, the unilateral parkinsonian symptoms and the lack of L-Dopa response. Different hypotheses have been put forward to explain the mechanism underlying dilated VRS, including disturbance of cerebrospinal ﬂuid drainage, focal brain atrophy, and leaking of interstitial ﬂuid to the pial space. VRS compression would cause a dysfunction downstream from the presynaptic nigrostriatal dopaminergic system causing parkinsonian clinical features.This case highlights that a dilated VRS could be a cause of unilateral parkinsonism. eISSN 2093-4939. http://dx.doi.org/10.14802/jmd.15013 -Tiago A. Mestre, MD, Melissa J. Armstrong, MD, Richard Walsh, MD, Amaal Al Dakheel, MD, Elena Moro, MD, A. Jon Stoessl, MD, Anthony E. Lang, MD. Can Isolated Enlarged Virchow-Robin Spaces Inﬂuence the Clinical Manifestations of Parkinson’s Disease? – Movement disorders clinical practice. doi:10.1002/mdc3.12009 -Ali Shoeibi, MD, Irene Litvan, MD. Levodopa-Responsive Parkinsonism Associated with Giant Virchow-Robin Spaces: A Case Report. Movement disorders clinical practice. doi:10.1002/mdc3.12484 186 Parkinsonism Secondary to Mercury Poisoning in a Colombian Population Juan Vargas Jaramillo (Bogota, Colombia) Objective: To describe the clinical and paraclinical characteristics of patients over 18 years of age who consulted the Neurology service of the University Hospital with diagnosis of mercury poisoning and parkinsonism in the period of 2015-2017. Background: Mercury is a heavy metal and its compounds are recognized as potentially hazardous materials. Industrial growth, and especially ﬂuorescent lamps, has increased the exposure and the risk of mercury poisoning. Through environmental studies and case-control studies, demonstrated that mercury is related to the presence of parkinsonism. Methods: Descriptive observational study of patients diagnosed with mercury poisoning and secondary parkinsonism, in a Neurology clinic of the University Hospital between 2015-2016. Sociodemographic, clinical and laboratory, collected in each patient. Results: A total of 7 individuals are reported in whom an intoxication is evidenced by mercury secondary to occupational exposure and that after chelation and management of acute symptoms present parkinsonism. Of the individuals studied, 100% were male and belonged to a company that produces ﬂuorescent lamps; the age range was 38-50 years. In these patients, the range of mercury levels at admission was 93-761 μg / l with controls after chelation in the ofﬁce neurology lower than 20 μg / l in blood; In addition, intoxication by other heavy metals was ruled out in all patients. 100% of the patients consulted for symptoms due to tremor at rest and bradykinesia, in addition to stiffness, with a positive levodopa test and a score on the MDS-UPDRS-III scale of 5-18. Conclusions: The results of this study suggest that there is a possible association of occupational exposure to mercury (with acute intoxication) and subsequent parkinsonism, however, larger and more methodologically robust studies are required to determine this association. This study is the ﬁrst in the country to report an empirical association of mercury poisoning and parkinsonism. References: Agency for Toxic Substances and Disease Registry (ATSDR). Evaluating Mercury Exposure: Information for Health Care Providers. 2011. pp. 1–2.2. Gasca-Alvarez A. Exposición ambiental a mercurio en minas de oro: medición del impacto en la salud en Guainia, Colombia. Rev. Salud Pública (Bogotá); 2000.3. Osorio García SD, Y, Perez CasHernández Flores LJ, Sarmiento R, González Alvarez tiblanco DM, Barbosa Devia MZ, et al. Prevalencia de mercurio y plomo en población general de Bogotá 2012/2013. Revista de Salud Pública. 2015 Mar 6;16(4):621–8. 187 Withdrawn by Author 188 References: -López Fernández M, et al. Espacios de VirchowRobin: ¿una causa de parkinsonismo? Neurología. 2015. http://dx.doi. org/10.1016/j.nrl.2015.01.002 -Jamal Omidi S, Noorollahi Moghadam H, Ghorbani A, Fatehi F. Giant Virchow-Robin Spaces as an Incidental ﬁnding in a Patient with Parkin- sonism. Arch Iran Med. 2014; 17(8): 587 – 588. -Myung Sik Lee, Cheol Hyung Lyoo, Tae Sub Chung. Parkinsonism and Dementia Associated with Giant VirchowRobin Spaces / J Mov Disord 2015;8(2):106-107 pISSN 2005-940X / S84 Early-Onset Parkinsonism: Clinical Characterization of a Serie of 27 Chilean Patients Daniela Munoz (Santiago, Chile), Monica Troncoso (Santiago, Chile), Valentina Naranjo (Santiago, Chile), Maria Jose Hidalgo (Santiago, Chile), Isadora Ruiz (Santiago, Chile), Veronica Saez (Santiago, Chile), Susana Lara (Santiago, Chile), Paola Santander (Santiago, Chile), Javiera Tello (Santiago, Chile), Andres Barrios (Santiago, Chile) Objective: The aim of this study is to characterize child population with early-onset parkinsonism. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Background: Parkinsonism corresponds to an entity widely described in adults, but little recognized in the child population. It is deﬁned by the presence of 2 or more cardinal signs: rest tremor, bradykinesia, rigidity or loss of postural reﬂexes. Primary and secondary causes are described, secondary causes are the most common in pediatric patients. Methods: Retrospective descriptive study, analysis of clinical records and revision of videos of pediatric patients with parkinsonism. Patients with drugs induced parkinsonism were excluded. Results: 27 patients evaluated, 12 males and 15 females. Average starting age parkinsonism: 10 years. Parkinsonian symptoms: bradykinesia/akinesia: 24/27, rigidity: 16/27, rest tremor: 4/27. Associated symptoms: 17/27 dystonia, 15/27 intellectual disability, 12/27 pyramidal signs, 11/27 epilepsy, 8/27 cerebellar signs, 5/27 myoclonus, Other: choreoathetosis, stereotypies. Etiologies: Primary 2/27 (1 PARK7, 1 PARK8), Secondary 24/27 (5 PKAN, 3 Wilson’s Disease, 3 Huntington’s Disease, 3 Neuronal Ceroid Lipofuscinosis, 2 Infantile neuroaxonal dystrophy, 1 Pitt Hopkins Syndrome, 1 GLUT-1 Deﬁcit, 1 Phenylketonuria, 1 Machado-Joseph disease, 1 SPG 11, 1 MELAS, 1 Fragile X syndrome, Metachromatic leukodystrophy). 1/27 with unknown cause. Conclusions: In our serie, early-onset parkinsonism manifests mainly with bradykinesia/akinesia and rigidity. Rest tremor is rare. The most common associated symptom was dystonia. Childhood parkinsonism can be a manifestation of different pathologies, its most frequent causes are secondary. References: Di Fonzo A, Dekker MC, Montagna P, Baruzzi A, Yonova EH, Correia Guedes L, et al. FBXO7 mutations cause autosomal recessive, early-onset parkinsonian-pyramidal syndrome. Neurology. 2009 Jan 20;72(3):240-5. Lucking CB, Durr A, Bonifati V, Vaughan J, De Michele G, Gasser T, et al. Association between early- onset Parkinson’s disease and mutations in the parkin gene. The New England journal of medicine. [Research Support, Non-U.S. Gov’t]. 2000 May 25;342(21):1560-7. Chung EJ, Ki CS, Lee WY, Kim IS, Kim JY. Clinical features and gene analysis in Korean patients with early-onset Parkinson disease. Archives of neurology. 2006 Aug;63(8):1170-4. Pediatric Movement Disorders 197 Movement Disorders in Patients with Childhood Autism Taras Voloshyn (Truskavets, Ukraine) Objective: To assess level of movement disorder pathology in patients with childhood autism. Background: Movement disorders are often accompanying childhood autism. Underestimating its role in a disease clinical manifestations leads to lack of treatment efﬁcacy. Methods: 375 patients with childhood autism (F84.0) examined. Mean age 7y2m (SD= 2.2). Anamnestic data analysis, neurological examination, Box and Blocks test, hand dynamometry were used. Results: There was a delay in development of all gross motor functions (sitting in average at 8m4days, standing at 10m12days, walking at 14months7days). Hand power characteristics corresponded normative data but manipulative functions were worse in comparison to normative rates in peers. Conclusions: There is a need to code and treat motor disorders in patients with childhood autism. Improvement in motor activity may lead to better integration into social activities and interaction. 198 Delayed Diagnosis of Patients with Neurodegeneration with Brain Iron Accumulation Travis Larsh (Seven Hills, OH, United States), Mohammed Aldosari (Cleveland, OH, United States), Hubert Fernandez (Cleveland, OH, United States) Objective: We aimed to investigate the time from symptom onset to the diagnosis of Neurodegeneration with brain iron accumulation (NBIA) disorders, which has not been reported previously. Background: Given the rarity of NBIA and the heterogeneity of its presentation, diagnostic uncertainties and delays may be more common than realized. However, recent studies have shown promising results in regards to potential therapeutics for patients with NBIA, and disease duration may play a role in how patients respond to therapy 1,2. Methods: A retrospective chart review of patients seen at Cleveland Clinic between January 2000 and April 2019 with diagnostic codes associated with any NBIA disorder was performed. In addition to basic demographics, details related to diagnosis, including presenting symptom, time from symptom onset to diagnosis (deﬁned as genetic testing with a known mutation causing NBIA or clinical diagnosis by a neurologist as probable NBIA in idiopathic cases), symptom onset and ﬁrst contact with a health care professional (and discipline of provider)/ referral to a neurologist/referral to a movement disorders specialist were collected. Results: Five patients with probable or deﬁnite NBIA were identiﬁed (table 1): 2 with probable PKAN; 2with deﬁnite BPAN; and, 1with probable neuroferritonopathy. Average age at initial symptom was 12 years old (range: 0.8 to 28.7). The time from symptom onset to initial evaluation ranged from the same day as symptom onset to one year after. Initial evaluating providers included two pediatricians, one family medicine physician, one emergency medicine physician, and one otolaryngologist. All patients were evaluated by a neurologist, with an average time from onset to evaluation of 1.4 years. Two patients saw a movement disorders neurologist, with an average time from onset to evaluation of 1.5 years. The average time from the onset to diagnosis was 8.7 years. Conclusions: Patients were symptomatic for 8.7 years on average prior to diagnosis, and none were diagnosed within the ﬁrst year. Four of the ﬁve patients originally presented to community providers, suggesting that education and communication with community providers may be critical for early diagnosis of patients with NBIA. Further studies aimed at expediting identiﬁcation of patients with these rare disorders should be pursued. References: 1. Klopstock T, Tricta F, Neumayr L, et al. Safety and efﬁcacy of deferiprone for pantothenate kinase-associated neurodegeneration: a randomised, double-blind, controlled trial and an open-label extension study. Lancet Neurol. 2019;18(7):631-642. doi: https://doi.org/10.1016/S1474-4422(19)30142-52. Sharma LK, Subramanian C, Yun M-K, et al. A therapeutic approach to pantothenate kinase associated neurodegeneration. Nat Commun. 2018;9(1):4399. doi:10.1038/s41467-018-06703-2 199 Clinical and Laboratory - Neurovisual Heterogeneity of Tic Disorders Zuleykha Zalyalova (Moscow, Russia), Sabina Munasipova (Kazan, Russia) Objective: To study the clinical and laboratory neuroimaging features of tic disorders. Background: Tic hyperkinesis, which is one of the most common and maladaptive diseases of childhood, has been actively studied in recent years. At the same time, scientiﬁc information about the causes, developmental mechanisms, and therapies contain conﬂicting opinions.The MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S85 ABSTRACTS ambiguous ideas about the nature of tics, the existence of difﬁculties in the clinical, laboratory, neuroimaging differential diagnosis of tics, and the difﬁculties of therapy served as arguments for us to conduct this study. Methods: The main group consisted of 111 patients. Two control groups consisted of 56 people. The patients were divided into 2 groups: “PANS-pediatric-onset neuropsychiatric syndrome” – “pediatric acute neuropsychiatric syndrome” and “non-PANS”.Research methods: 1) neurological examination; 2) laboratory clinical and immunological studies; 3) MRI of the brain with morphometry of the basal ganglia, thalamus, corpus callosum, cingulate gyrus. Results: 1. Based on clinical and anamnestic analysis, taking into account reversibility, persons with Tourette’s syndrome (33%) prevailed, less commonly with chronic simple motor tics (31%), with chronic simple and complex motor or vocal tics (17%), chronic simple motor and vocal tics (15%), with transient tics (4%). Tic severity signiﬁcantly prevailed in patients with Tourette’s syndrome. Regardless of the phenomenology of tics, a family history was traced in all subgroups. Concomitant chronic infections of the upper respiratory tract didn’t affect the clinical manifestations of tics.2. Obsessive-compulsive disorders were found in 54.1% of patients with tics. The direct dependence of the severity of chronic simple and complex motor or vocal tics (r = 0.61, p=0.01) and Tourette’s syndrome (r = 0.54, p=0.01) on the severity of OCD was determined. Tic hyperkinesis was associated with ADHD with a predominance of hyperactivity in 81% of cases. A reliable direct dependence of the severity of tics on the severity of ADHD was revealed in terms of inattention, hyperactivity, and impulsivity.3. A correlation was established between phenotypic manifestations and MRI morphometric indicators:- in patients with tics, the height of the anterior third of the cingulate gyrus is less than in the control groups (p <0.05); the severity of motor tics depends on the height of the middle third (r = 0.285, p=0.025), and vocal tics on the height of the back third of the cingulate gyrus (r = 0.331, p <0.05); the dorsoventral size of the caudate nucleus of the right hemisphere in patients with tics is greater than in the control groups (p <0.05).- the height of the middle third of the cingulate gyrus in patients with OCD is greater than in patients with tics without OCD (p = 0.02); the severity of OCD correlates with the knee length of the corpus callosum (r = 0.334, p=0.05); the size of the thalamus in the left hemisphere is increased (p = 0.02); the volume of the pale ball in the right hemisphere is reduced (p <0.05); the shape of the shell in the left hemisphere is ﬂattened and the shape of the shell in the right hemisphere is elongated (p <0.05);- the severity of ADHD depends on the distance from the frontal pole of the brain to the knee of the corpus callosum.4. The epidemiological and phenomenological features of tic hyperkinesis and the severity of behavioral disorders in patients with "PANS" were established. PANS syndrome was 2.5 times more common in males. The presence of a chronic focus of infection was not a predictor of the development of PANS syndrome. The PANS group was characterized by Tourette’s syndrome (39%), mild to severe OCD, ADHD with a predominance of hyperactivity.5. Morphometrically, the PANS group was characterized by an increase in the size of the corpus callosum (an increase in its length, knee, roller, width of the corpus callosum in a vertical section, p <0.05), a decrease in the size of the thalamus in the left hemisphere (p <0.05), the ﬂattened form of a pale ball in both hemispheres, the rounded shape of the shell in the left hemisphere.6. Clinical and laboratory analysis of patients with tics did not reveal a correlation with the clinical manifestations of ASL-O titers, antinuclear antibodies, IgA, CD4 + lymphocytes, and CEC values, while a reduced level of JgM, an increased level of CD8 + lymphocytes and carriage of ß- hemolytic streptococcus group A are determined development of autoimmune neuropsychiatric disorders in patients with PANS. Conclusions: Tic is the cause of pancerebral dysfunction, as a result of which they have a complex psychomotor “nature.” Impaired Blymphocyte differentiation, lack of humoral response, special antigens that can cause an inadequate immune response, accompanied by a powerful release of cytokines, can be the basis for the launch of autoimmune disorders with neuronal tissue damage and as a result of the development of tic hyperkinesis. References: 1. Munasipova S.E. Tic hyperkinesis: clinical, laboratory-neurovisual heterogeneity. Abstract. dis. Cand. honey. S86 sciences. - Moscow, 2015 .-- 24 p.2. 182.Swedo S.E. From research subgroup to clinical syndrome: Modifying the PANDAS criteria to PANS (pediatric acute-onset neuropsychiatric syndrome) / S.E. Swedo, J.F. Leckman, N.R. Rose // Pediatr Therapeut. – 2012. - Vol. 2. – ?. 2-8. Phenomenology and Clinical Assessment of Movement Disorders 204 Tinnitus Secondary to Stapedial Muscle Spam in Patients with Hemifacial Spasm: Clinical Characterization and Rresponse to Botulinum Toxin Treatment Gabriela Raina (Buenos Aires, Argentina), Nicolas Morera (Buenos Aires, Argentina), Silvia Folgar (Buenos Aires, Argentina), Cristian Calandra (Buenos Aires, Argentina), Ricardo Maiola (Buenos Aires, Argentina), Cynthia Garcia Fernandez (Capital Federal, Argentina), Maria Cersosimo (Buenos Aires, Argentina) Objective: (i) To assess the frequency of tinnitus in patients with HFS, (ii) to describe the clinical features of it and, (iii) to explore the utility of botulinum toxin (BTX) injections in the preauricular region to abolish tinnitus. Background: Tinnitus is the perception of sounds in the absence of external stimuli. It has been described that patients with post-paralytic hemifacial spasm (HFS) may complain of tinnitus, which is thought to result from the abnormal contraction of the stapedius muscle (SM) innervated by cranial nerve VII. Different treatments such as carbamazepine or masking devices have failed to improve tinnitus and tenotomy of SM may produce hyperacusis. Methods: Patients with idiopathic and post-paralytic HFS were included in a retro-prospective study in order to identify cases with tinnitus secondary to SM spasm (TSMS). In those cases presenting TSMS we injected 2 points of 5 BTX units in the preauricular region (in front of the tragus), in addition to the usual inﬁltration for their HFS. Results: Among 283 patients with HFS we found 33 (11.66%) cases with TSMS (25 females; age 66.5710; disease duration 12,927.15 years). Only 4 patients presented post-paralytic HFS. TSMS and HFS started simultaneously in 9 cases (27.2%) while in 24 (72.7%) TSMS occurred 9.3 7.5 years after the onset of HFS. TSMS was described like a ”plop”, “pop” or “pap” in 93.10% (n=27), like a “buzz” in 9.09 % (n=3) and “like water falling” in 6 % (n=2). In all cases TSMS was described as irregular and intermittent. It worsened at bedtime in 42.4% of cases, with stress in 9 %, and in 6 % TSMS it was coincident with facial contractions that were severe and sustained. A transient relief of TSMS with alleviating maneuvers was present in 30.3% of the patients. Inﬁltrations with BTX resulted in a complete remission of TSMS in 27 patients (84.3%), partial improvement in 4 (12.5%) and no response in 1 (3.1%). TSMS reappeared by the end of the beneﬁcial effect of BTX. A case with post-paralytic HFS had a spontaneous remission of TSMS. Conclusions: (i) TSMS is an under reported and under diagnosed manifestation of HFS that reﬂects the involvement of the SM, (ii) It may accompany not only post-paralytic but also idiopathic HFS, (iii) BTX injections in the preauricular region resulted in a marked improvement of TSMS. References: 1) Watanabe I, Kumagami H, Tsuda Y. Tinnitus due to abnormal contraction of stapedial muscle. An abnormal phenomenon in the course of facial nerve paralysis and its audiological signiﬁcance. ORL J Otorhinolaryngol Relat Spec. 1974;36(4):217-26. 2) Ellenstein A, Yusuf N, Hallett M. Middle Ear Myoclonus: Two Informative Cases and a Systematic Discussion of Myogenic Tinnitus. Tremor Other Hyperkinet Mov (N Y). 2013 Apr 15;3. pii: tre03-103-3713-1. 3) Liu HB1, Fan JP, Lin SZ, Zhao SW, Lin Z. Botox transient treatment of tinnitus due to stapedius myoclonus: Case report. Clin Neurol Neurosurg. 2011 Jan;113(1):57-8.4) Park SN1, Bae SC, Lee GH, Song JN, Park KH, Jeon EJ, Park YS, Yeo SW. Clinical characteristics and therapeutic response of objective tinnitus due to middle ear myoclonus: a large case series. Laryngoscope. 2013 Oct;123(10):2516-20. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Psychiatric Manifestations 201 Examining Real World Treatment Pathways and Outcomes in Parkinson’s Disease Psychosis: Early Findings From the INSYTE Observational Study Stuart Isaacson (Boca Raton, FL, United States), Jeffrey Cummings (Los Angeles, CA, United States), Susan Fox (Toronto, ON, Canada), Jeffrey Trotter (Morrisville, NC, United States), Niccole Larsen (Bedford, MA, United States), Jesse LoVerme (Prior Lake, MN, United States), Andrew Shim (San Diego, CA, United States), Jennifer Goldman (Chicago, IL, United States) Objective: The INSYTE study provides information on the management of Parkinson’s disease psychosis (PDP) in practice settings, including use of antipsychotics (AP) and their impact on clinical, economic, and humanistic outcomes over up to 3 years. Background: Various clinical treatment strategies for PDP and their impact have not been evaluated. Providers and policymakers may beneﬁt from such contemporaneous “real world” ﬁndings on treatment modalities and their outcomes. Methods: INSYTE recently completed enrollment of new patients, with 764 patients enrolled at 77 US sites. Study patient follow-up is ongoing. Data are compiled at the baseline (BL) visit and from standardof-care follow up visits over 3 years. PDP treatment pathways are deﬁned from 3 BL cohorts reﬂecting (1) no AP medication, (2) use of pimavanserin (PIM), or (3) other AP treatment. Information about APs used is collected at each follow-up visit: history, duration, dose, adjustment, and rationale for adjustment of treatment. Outcomes assessments (clinical, quality of life, disease burden) by the physician, patient, and caregiver are collected. AP medication and outcomes data are analyzed for patients completing a BL and 1 follow up visit (FU1). Results: For 404 patients with BL and FU1 visits (mean 120.7 days from BL), 56.8% used no AP medications, 26.0% used PIM, and 13.6% used other APs at BL. The No Medication group was noted to be less severe in key BL disease parameters. Considering primary PDP treatments at BL and FU1 (including no treatment), 26 distinct treatment maintenance and switching strategies have been observed. 12.6% of patients had AP medication adjustments between BL and FU1 visits, most frequently from the non-PIM group. Adjustments of APs occurred in many forms: introduction of a single AP (64.7%%), introduction of multiple APs (5.9%), switching to another AP (3.9%), decreasing the number of APs (5.9%), and discontinuation (19.6%). Conclusions: Multiple, divergent AP treatment strategies for PDP exist in actual practice. No identiﬁable BL characteristics correlated with the broad range of AP treatment pathways. The numerous distinct AP treatment pathways utilized (n=26) reﬂect “real world” observations about treatment practice and provide an opportunity for education and additional dialogue among providers and between patients and providers. The greater use of APs outside of PIM and clozapine indicate that more AP types are utilized in PD, despite the MDS evidence-based recommendations. These data will be updated at the time of presentation with the anticipated study results reﬂecting the diversity of treatment regimens and their relative impact on outcomes. 202 Chart Review Examining Outcomes in Patients Using Pimavanserin Salima Brillman (Menlo Park, CA, United States) Objective: The objective of the current case review was to examine the clinical outcomes in patients using pimavanserin for hallucinations and delusions. Background: Hallucinations and delusions remain a burdensome and undertreated non-motor symptom of Parkinson’s disease (PD). Treatment approaches are now established, but clinicaloutcomes regarding quality of life are scant. Methods: Chart review of six patients at the Parkinson’s Disease and Movement DisordersCenter of Silicon Valley between September 2017 and December 2018 experiencinghallucinations and/or delusions. Patient ages ranged between 68 and 89 years; two werefemale, four male; two with diagnoses of dementia with Lewy bodies and four with PD. Outcome measures included the Clinical Global Impressions (CGI) scale for hallucinations anddelusions severity, and the Parkinson’s Disease Questionnaire-8 (PDQ-8). Results: There was an overall improvement in CGI for hallucinations and delusions severity, andPDQ-8 from prior to the initiation of pimavanserin to three months following initiation. Conclusions: The implications for treating this debilitating nonmotor symptom are vastincluding patient safety, maintaining relationships and independence, and avoiding nursinghome placement and hospitalizations. Further exploration of outcomes in the use ofpimavanserin in this population is warranted. 203 Medication-Induced QTc Prolongation in Parkinson’s DiseaseRelated Psychosis: A Literature Review Amelia Carwin (Arlington, VA, United States), Jacob Carolan (Washington, DC, United States), Steven Nakano (Silver Spring, MD, United States), Shahnaz Miri (Washington, DC, United States), M. Waseem Anjum (Germantown, MD, United States), Yasar Torres-Yaghi (Washington, DC, United States), Charbel Moussa (Washington, DC, United States), Fernando Pagan (McLean, VA, United States) Objective: Our objective is to assess the relative QTc prolongation induced by medications used to treat Parkinson’s Disease (PD)-related psychosis. Background: PD-related psychosis is multifactorial, and as such, medications used to treat hallucinations and delusions in this population have several therapeutic targets. It is treated with pimavanserin, olanzapine, clozapine, quetiapine, rivastigmine, donepezil, galantamine, and memantine. Methods: Pubmed searches for “Pimavanserin QTc,” “Olanzapine QTc,” “Clozapine QTc,” “Quetiapine QTc,” “Rivastigmine QTc,” “Donepezil QTc,” “Galantamine QTc,” and “Memantine QTc” were performed and relevant information was extracted, with a focus on more recent articles published in the last 10 years. Results: According to the Nuplazid (pimavanserin) Prescribing Information, pimavanserin prolongs the QT interval. (1) Data are mixed with respect to olanzapine, clozapine, and quetiapine effect on QT interval, with some studies demonstrating prolongation, some demonstrating no signiﬁcant effect. (2-13) Rivastigmine was shown, in patients with Alzheimer’s disease, to not affect QTc intervals. (14, 15) QTc prolongation has been identiﬁed in case reports of donepezil. (16, 17) A retrospective study, however, demonstrated donepezil to not be associated with signiﬁcant changes relative to the baseline QTc interval. (18) Case reports describe possible galantamine- and memantine-induced QTc prolongation. (19-22) Conclusions: A speciﬁc study on QTc prolongation in PD patients treated with antipsychotics is warranted. While PD patients exhibiting psychotic symptoms are treated with medications targeting multiple neurotransmitters, it is possible that PD patients exhibit different QTc prolongation proﬁles following antipsychotic use as compared to other patient populations. Neuroimaging evidence from a 1997 paper demonstrated cardiac sympathetic denervation in PD, which has been conﬁrmed by many studies since, (23) and additional studies are needed to determine whether these changes could affect QTc intervals in PD patients treated with antipsychotics. References: 1. Nuplazid Prescribing Information. https://www. nuplazid.com/pdf/NUPLAZID-Prescribing-Information-PI-v8-Sep-2018. pdf. (PDF). San Diego, California: Acadia Pharmaceuticals, Inc.; 2018. 2. Aronow WS, Shamliyan TA. Effects of atypical antipsychotic drugs on QT interval in patients with mental disorders. Ann Transl Med. 2018;6 (8):147. doi:10.21037/atm.2018.03.17 3. Czekalla J, Beasley CM Jr, Deliva MA, Berg PH, Grundy S (2001). Analysis of the QTc interval during olanzapine treatment of patients with schizophrenia and related psychosis. J Clin Psychiatry 62:191–198. 4. Grande I, Pons A, Baeza I, Torras A, Bernardo M. QTc prolongation: is clozapine safe? Study of 82 cases before and after clozapine treatment. Hum Psychopharmacol. 2011;26(6):397-403. doi:10.1002/hup.1221. Epub 2011 Aug 9. 5. Ozeki Y, Fujii K, Kurimoto N, Yamada N, Okawa M, Aoki T, Takahashi J, Ishida N, Horie M, Kunugi H. QTc prolongation and antipsychotic medications in a sample of 1017 patients with schizophrenia. Prog Neuropsychopharmacol Biol Psychiatry. 2010 Mar 17;34(2):401-5. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S87 ABSTRACTS 6. Harrigan EP, Miceli JJ, Anziano R, Watsky E, Reeves KR, Cutler NR, Sramek J, Shiovitz T, Middle M. A randomized evaluation of the effects of six antipsychotic agents on QTc, in the absence and presence of metabolic inhibition. J Clin Psychopharmacol. 2004 Feb;24(1):62-9.7. Olsen RE, Kroken RA, Bjorhovde S, Aanesen K, Jorgensen HA, Loberg EM, Johnsen E. Inﬂuence of different second generation antipsychotics on the QTc interval: A pragmatic study. World J Psychiatry. 2016 Dec 22;6 (4):442-448. 8. Lin CH, Chen MC, Wang SY, Ling CY (2004). Predictive factors for QTc prolongation in schizophrenic patients taking antipsychotics. J Formos Med Assoc 103:437–441. 9. Kang UG, Kwon JF, Ahn YM, Chung SJ, Ha JH, Koo YJ, Kim YS. Electrocardiographic abnormalities in patients treated with clozapine. J Clin Psychiatry. 2000 Jun;61 (6):441-6. 10. Harrigan EP, Miceli JJ, Anziano R, Watsky E, Reeves KR, Cutler NR, et al. (2004). A randomized evaluation of the effects of six antipsychotic agents on QTc, in the absence and presence of metabolic inhibition. J Clin Psychopharmacol 24:62–69. 11. Lee S, Morris A, Kim S, Li F, Baumgartner L. Impact of Quetiapine Therapy on QTc Prolongation in Critically Ill Patients. Ann Pharmacother. 2019 Jul;53 (7):705-710.12. Kim A, Lim KS, Lee H, Chung H, Yoon SH, Yu KS, Cho JY, Jang IJ, Chung JY. A thorough QT study to evaluate the QTc prolongation potential of two neuropsychiatric drugs, quetiapine and escitalopram, in healthy volunteers. Int Clin Psychopharmacol. 2016 Jul;31(4):210-7.13. Dube KM, DeGrado J, Hohlfelder B, Szumita PM. Evaluation of the Effects of Quetiapine on QTc Prolongation in Critically Ill Patients. J Pharm Pract. 2018 Jun;31(3):292-7. 14. Morganroth J, Graham S, Hartman R, Anand R. Electrocardiographic effects of rivastigmine. J Clin Pharmacol. 2002 May;42(5):558-68.15. Riepe MW. High-dose cholinergic therapy with rivastigmine patch does not prolong QTc time in patients with Alzheimer’s disease. J Clin Psychiatry. 2014 Mar;75(3):288. 16. Vogel SM, Mican LM, Smith TL. Donepezil-induced QTc prolongation: A case report. Ment Health Clin. 2019 May 10;9(3):128-132.17. Pourmand A, Shay C, Redha W, Aalam A, Mazer-Amirshahi M. Cholinergic symptoms and QTc prolongation following donepezil overdose. Am J Emerg Med. 2017 Sep;35 (9):1386.18. Isik AT, Yildiz GB, Bozoglu E, Yay A, Aydemir E. Cardiac safety of donepezil in elderly patients with Alzheimer disease. Intern Med. 2012;51(6)575-8. 19. Nelson MW, Buchanan RW. Galantamine-induced QTc prolongation. J Clin Psychiatry. 2006 Jan;67(1):166-7. 20. Vigneault P1, Bourgault S, Kaddar N, Caillier B, Pilote S, Patoine D, Simard C, Drolet B. Galantamine (Reminyl) delays cardiac ventricular repolarization and prolongs the QT interval by blocking the HERG current. Eur J Pharmacol. 2012 Apr 15;681(1-3):68-74. 21. Takehara H, Suzuki Y, Someya T. QT prolongation associated with memantine in Alzheimer’s disease. Psychiatry Clin Neurosci. 2015 Apr;69(4):239-40.22. Kajitani K, Yanagimoto K, Monji A, Maruyama T. Memantine Exacerbates Corrected QT Interval Prolongation in Alzheimer’s Disease: A Case Report from an Unintentional Rechallenge. J Am Geriatr Soc. 2016 Jan;64(1):232-3. 23. Goldstein, D.S., et al., 1997. Sympathetic cardioneuropathy in dysautonomias. N. Engl. J. Med. 336, 696–702. Quality of Life/Caregiver Burden in Movement Disorders caregivers differed in the amount of information desired at the time of diagnosis, while many wanted information regarding progression. Most patients and caregivers sought information from various sources including the internet. The meaning of diagnosis varied among participants with some identifying positive aspects of the diagnosis in terms of ﬁnding meaning and changes to relationships while others could not identify any positive aspects. Conclusions: Patients and caregivers may differ in the amount of information they require at the time of diagnosis and throughout the course of illness. Clinicians should be aware that patients and caregivers seek out information elsewhere in the absence of information from their clinician. Some patients and caregivers may ﬁnd positive changes in their lives and relationships despite the dire prognosis while others may not ﬁnd positive meaning from a diagnosis of MSA. Unanswered questions include how to determine the medical information required by individual patients, whether patients and caregivers should receive information separately to align with differing information styles, and at the time of diagnosis (rather than in retrospect), how much information is appropriate to share with patients. 123 The Impact of Parkinson’s Disease on Quality of Life and Cognition in Older Adults Livia Caroline Chaves Dos Santos (Mogi Das Cruzes, Brazil) Objective: To analyze the inﬂuence of PD on cognition and quality of life. Background: In addition to the functional problematic of biological aging, it is during this period that most of the chronic diseases arise. Among these, one of the most important is Parkinson’s disease (PD), due to the gravity and the high prevalence for adults older than 60 years. Methods: This was a cross-sectional study involving 40 older adults of both genders, divided into two groups: Parkinson’s group and Control group (without PD). Quality of life was assessed using the WHOQOLOLD and WHOQOL-BREF instruments and cognition by MoCA assessment. Results: The quality of life questionnaires showed worse scores for the Parkinson’s group when making comparisons via the WHOQOLBREF questionnaire in relation to the domains: physical, psychological and environmental. There was no difference in the cognition examinations. The table 1 showed quality of life and cognition scores. Conclusions: PD negatively affects quality of life in physical, psychological and environmental aspects. The clinical contributions refer to the speciﬁc aspects that can be incorporated into the rehabilitation of older adults with PD. References: 1. Teixeira TG, Batista AC. Treinamento físico para idosos vulneráveis: uma revis~ao sobre as estratégias de intervenç~ao. Rio Table 1. Test results for the hypothesis of equality of the means in quality of life for the groups PD Group 122 Two Sides to a Story: Perspectives on Information of Patients and Caregivers Living with Multiple System Atrophy (MSA) Beth Langford (Edmonton, AB, Canada), Janis Miyasaki (Edmonton, AB, Canada) Objective: To investigate the differing information preferences of patients and caregivers living with MSA as well as to examine the meaning patients and caregivers attach to the diagnosis of MSA. Background: Qualitative research regarding quality of life in MSA and concordance or discordance between patients and caregivers is rarely documented in the literature. Methods: We are conducting a mixed methods study of patients living with MSA and their family caregivers recruiting from a tertiary centre and Parkinson Association of Alberta. Qualitative interviews of patients and caregivers are conducted and analyzed using a thematic approach. Results: Four patients and four caregivers have been interviewed to date. Two emerging themes are presented: the desire for information and the meaning of the diagnosis for patients and caregivers. Patients and S88 Control P-value Group Mean (SD) 3.15(0.66) P<0.001* 1.85 (0.70) 0.001* 8.68(2.09) 0.307 2.69(0.57) P<0.001* WHOOL-BREF Mean (SD) Physical 3.81(0.41) Psychological 5.18(1.02) Social relations 7.81(3.15) Environment 3.19(0.54) WHOQOL-OLD Sensory abilities 3.89(0.78) 3.51(1.06) Autonomy 3.72(0.54) 3.58(0.53) Past, present and 3.64(0.53) 3.58(0.71) future activities Social participation 3.73(0.50) 3.40(0.93) Death and dying 3.48(1.08) 3.50(0.68) Intimacy 3.63(0.75) 3.70(0.83) COGNITION MoCA Assessment 23.27(3.45) 23.31(4.94) 0.189 0.409 0.759 0.147 0.968 0.796 0.974 Parkinson’s Disease (PD); Standard deviation (SD); World Health Organization instrument (WHOQOL-OLD and WHOQOL-BREF); Montreal Cognitive Assessment (MoCA); Student t *P≤0.05 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Claro, 2009. 2. Castro MF, Sanchez EGM, Felippe LA, Christofolett G. The Role of Physiotherapy Postural Control in the Elderly. Revista Movimenta. Rio Grande do Sul, 2012. 3. Chaimowicz F. A saúde dos idosos brasileiros às vésperas do século XXI: problemas, projeções e alternativas. Revista Saúde Pública. 1997;31(2):184-200. 4. Rajput AH, Rajput EF. Octogenarian parkinsonism Clinicopathological observations. Parkinsonism Relat Disord. 2017; 37:50-57. 124 Impact of Mindfulness Training on Quality of Life, Deppression, Anxiety, Stress and Cognitive Function in Patients with Parkinson’s Disease Fatemeh Seifar (Tabriz, Iran, Islamic Republic of), Hormoz Ayromlou (Tabriz, Iran, Islamic Republic of), Safa Najmi (Tabriz, Iran), Neda Ghaemian (Tabriz, United States), Sheida Shaaﬁ (Tabriz, Iran, Islamic Republic of), Roghayyeh Asadi (Tabriz, Iran, Islamic Republic of) Objective: The aim of this study was to evaluate the possible effect of mindfulness-based stress reduction training (MBSR) on non-motor symptoms of patients with Parkinson’s disease (PD). Background: PD is the second common neurodegenerative disorder afﬂicting a large number of elder populations. It is mainly considered a movement disorder; however, the non-motor symptoms have a great impact on the patients’ quality of life. Depression, anxiety and stress are the most common non-motor symptoms. Methods: This study was conducted at outpatient neurology clinic of Imam Reza and Razi University- Hospital-Tabriz, Iran. Participants were 40 patients aged 67.956.8 years (56-80) with deﬁnite diagnosis of PD who were receiving dopaminergic drugs for at least one year. Participants were randomly categorized in two experiment and control group with 20 patients in each. The experiment included 8-week MBSR training each for 2h with a 15-minute break between ﬁrst and second hour. The assessments included patients’ quality of life using PDQ-39 questionnaire, Depression, anxiety, stress by DASS-21 questionnaire and cognitive function based on mini- mental state exam (MMSE) were conducted at baseline and one month after the experiment. The results were compared between two study groups and within each group using t-test analysis. Results: The assessment revealed a lower mean score in all PDQ-39 items in experiment group compared to control subjects, however the difference was only signiﬁcant for social support (34.139.7 vs. 26.197.7 for control and experiment group, respectively. P=0.007). The mean PDSI score of patients in experiment group was 31.886.5 after one month compared to baseline score of 33.936.2 (p<0.001). The mean depression score of the participants in experiment group was signiﬁcantly lower than the controls (p=0.04). Within group analysis demonstrated a signiﬁcant decrease in the experiments’ mean depression score from 15.202.4 to 14.002.5 (p=0.001), mean anxiety score from 14.302.7 to 13.452.3 (p=0.001) and in stress score from 24.43.7 to 22.93.1 (p< 0.001) in one month. The mean MMSE scores were identical before and after the experiment in each study group. Conclusions: In our study, mindfulness training improved the overall quality of life in PD patients. Overall PDSI decreased modestly in the experiment group by 5.12% after the experiment. It had a short-term positive impact on depression, anxiety and stress but not on the cognitive function of the PD patients. However, long-term follow up on a large scaled population is required to evaluate the impact of mindfulness-based stress reduction on each item. 125 Educational Text-Messaging for Parkinson’s Disease and Caregivers to Promote Disease Speciﬁc Knowledge Shahnaz Miri (Washington, DC, United States), Adil Alaoui (Washington, DC, United States), Yasar Torres-Yaghi (Washington, DC, United States), Amelia Carwin (Arlington, VA, United States), Steven Nakano (Silver Spring, MD, United States), M. Waseem Anjum (Germantown, MD, United States), Fernando Pagan (McLean, VA, United States) Objective: This study aimed to develop an automated text messaging system to support Parkinson’s disease (PD)-speciﬁc education for patients and caregivers. Background: PD is the second most common neurodegenerative disorder with an estimated combined (direct and indirect) cost of $52 billion per year in the United States. Limited patients’ knowledge and difﬁculty in understanding the complexities of the disease lead to sub-optimal outcomes in PD patients. Methods: We have developed a text-messaging platform powered by artiﬁcial intelligence in two sequential phases. First, we performed stakeholders’ needs assessment by interviewing patients and reviewing literature. Consequently, a bank of educational text messages was developed and validated by two movement disorders specialists and four neurologists. A preliminary pilot feasibility study was conducted. Results: The content of the automated-text messaging platform was validated by a total of six neurologists and movement disorder specialists for improving PD-speciﬁc knowledge in patients and caregivers. Preliminary results demonstrate the feasibility of the intervention among patients and caregivers. Conclusions: Improving PD-speciﬁc knowledge through text messaging is a feasible and scalable intervention to promote self-management in PD and to improve care support. 126 ‘Partnering with Poise’: Alexander Technique Group Classes Are a Promising Self-care Intervention to Counter Loss-of-Self for Care Partners of People Living With Neurodegenerative Disease Monika Gross (Candler, NC, United States), Ramyaa Ravichandra (Moscow, ID, United States), Belinda Mello (New York, NY, United States), Allaa Abdelrahman (Moscow, ID, United States), Jordan Becker (Providence, RI, United States), Sawyer Smith (Providence, RI, United States), Rajal Cohen (Moscow, ID, United States) Objective: Studies show that 1:1 Alexander technique sessions can reduce musculoskeletal pain and anxiety [1-3]. We tested an Alexander group course to improve quality of life and increase agency for care partners of people living with Parkinson’s disease. Background: Caregivers often experience role engulfment and loss of self [4]. Alexander technique is an embodied cognitive approach that aims to transform disruptive reactions to stress into adaptive responses, increasing conﬁdence, calm, and self-awareness. Methods: We delivered our course to 71 participants in 10 cities in southeastern USA. Groups met for 90 min/week x 10 weeks. The selfmanagement approach was taught by verbal instruction, hands-on guidance, and interactive (partner and group) activities. Importantly, every activity was preceded by intentional strategies to interrupt automatic reactions and afﬁrm the centrality of the self. These strategies were simple enough to be remembered and used independently outside of class. Outcomes assessed before and after the intervention included executive function (Stroop and Digit Span), balance (Mini-BEST), posture photos, and self-report measures. We also collected anonymous post-course evaluations and post-course video interviews. Results: Ten subjects dropped out of the class. Attendance for the remaining participants was 85%. On a 0-10 scale, the mean rating was 9.5 for “enjoyed the interaction with other participants,” 9.2 for “encountered new ideas,” 8.4 for “learned skills to take care of myself emotionally,” and 8.3 for “likely to use the new skills in my daily life.” Executive function improved (p<.05), and increases in self-reported mindfulness correlated strongly with increased self-efﬁcacy and reduced fear and fatigue (p<.00005). During assessments and interviews, improvement in overall poise is often visible. Numerous participants reported increased physical conﬁdence and emotional well-being despite their care receivers’ declining health and increasing needs. Conclusions: Alexander technique shows promise as a long-term self-management approach to reduce loss-of-self in care partners of people with serious neurodegenerative disease, with potential beneﬁts increasing over time. The educational nature of the approach appeared to lead to continued improvement and enthusiasm from participants long after the course had ended. Group classes have the potential to provide cost-effective delivery with additional social beneﬁts. Future studies could include additional follow-up opportunities, such as drop-in classes and peer-to-peer training. References: 1.Little P, Lewith G, Webley F, Evans M, Beattie A, Middleton K, Barnett J, Ballard K, Oxford F, Smith P, Yardley MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S89 ABSTRACTS L. Randomised controlled trial of Alexander technique lessons, exercise, and massage (ATEAM) for chronic and recurrent back pain. Bmj. 2008 Aug 19;337:a884. 2. MacPherson H, Tilbrook H, Richmond S, Woodman J, Ballard K, Atkin K, Bland M, Eldred J, Essex H, Hewitt C, Hopton A. Alexander technique lessons or acupuncture sessions for persons with chronic neck pain: a randomized trial. Annals of internal medicine. 2015 Nov 3;163(9):653-62. 3. Kenny DT. Music performance anxiety: Origins, phenomenology, assessment and treatment. Context: Journal of music research. 2006;31:51.4. Skaff MM, Pearlin LI. Caregiving: Role engulfment and the loss of self. The Gerontologist. 1992 Oct 1;32(5):656-64. Rare Genetic and Metabolic Diseases 127 Valosin-Containing Protein Mutation in a Patient with Ataxia and Parkinsonism Alana Kirby (Chicago, IL, United States), Virginia Kimonis (Irvine, CA, United States), Aikaterini Kompoliti (Chicago, IL, United States) Objective: To discuss an unusual case of progressive ataxia and parkinsonism due to a mutation in the valosin-containing protein (VCP) gene. Background: VCP mutations cause multi-system proteinopathy with a range of observed phenotypes including movement disorders, typically parkinsonism [1]. A phenotype of spinocerebellar degeneration has been mentioned only brieﬂy in the literature [2]. Methods: Case report and review of the literature. Results: A 36-year old woman presented with gradually progressive dysarthria, ataxia and neuropathy for seven years. Over the next ten years she developed mild dysphagia, urinary frequency and parkinsonism. Balance improved with amantadine and rest tremor initially responded to Carbidopa/Levodopa. Medical history was noncontributory. Prior to becoming disabled she worked in hospital administration. She was adopted and has two sons who are healthy teenagers. Workup was notable for: MRI brain which was unrevealing; DaTscan at age 41 showed presynaptic dopaminergic impairment bilaterally; SCA panel showed borderline SCA2 repeats (31 and 32); and whole exome sequencing showed a known pathologic mutation in the VCP gene (heterozygous p.R159C c.475 C>T). Conclusions: VCP is a member of the AAA+ ATP-ase family and is involved in many cellular functions including protein degradation. Autopsies of affected patients show abnormal deposition of proteins: TDP-43 depositions are common in brain and muscle; and alphasynuclein deposits have been observed in a subset of patients, some but not all of whom have parkinsonism [2]. Protein deposits in SCAs have been shown to contain VCP [3]. Given the known role of VCP in neurodegenerative conditions, this patient’s movement disorder is likely due to her pathologic VCP mutation. This report highlights a novel genetic cause of chronic progressive ataxia and parkinsonism which should be considered in the workup of patients with negative SCA testing. References: 1. Plewa J. et al., A cross-sectional analysis of clinical evaluation in 35 individuals with mutations of the valosin-containing protein gene Neuromuscul Disord. 2018; 28: 778–7862. Spina S. et al., Phenotypic variability in three families with valosin-containing protein mutation. Europ J Neurol 2013; 20: 251–258.3. Chung CG et al., Mechanisms of protein toxicity in neurodegenerative diseases. Cell and Molec Life Sci. 2018; 75: 3159-3180 128 Adult-Onset POLR3 Leukodystrophy: A Case Report Roshni Patel (Chicago, IL, United States), Natalie Witek (Chicago, IL, United States), Mitra Afshari (Chicago, IL, United States) Objective: To describe a case of rapidly progressive weakness, dystonia, dysphagia, cognitive decline and MRI white matter changes in a young man with POLR3A gene mutation. Background: POLR3-related leukodystrophy is characterized clinically by a varying combination of progressive neurologic dysfunction S90 (including cerebellar, extrapyramidal, pyramidal and cognitive dysfunction), abnormal dentition, endocrine abnormalities with or without grown hormone deﬁciency (most commonly, hypogonadotropic hypogonadism), and ocular problems. MRI brain with diffuse hypomyelinating leukodystrophy with relative preservation of myelination in the speciﬁc brain structures (e.g. ventrolateral thalamus, dentate nuclei, and optic radiations), and atrophy of the cerebellum and corpus collosum are highly suggestive of the diagnosis. Age on onset is typically in early childhood; we report an adult-onset case. Methods: Case report. Results: A 22 year-old previously healthy man of Nepalese decent presented with 2.5 years of progressive neurologic symptoms. Initial symptoms were cognitive difﬁculties and repetitive motor tic involving the left shoulder. He progressively developed spastic limb weakness (left greater than right), dystonia, ataxia, dysarthria, pseudobulbar affect, aphasia and dysphagia requiring feeding tube placement. Approximately 6-12 months after symptom onset, he was wheelchair-bound. On exam, he also had short stature, poor dentition with oral ulcers and normal fundoscopic exam. Cerebrospinal ﬂuid analysis revealed oligoclonal bands and elevated IgG index. Serial MRIs of the brain over 18 months showed evolution of non-enhancing T2 hyperintensities involving subcortical and periventricular white matter and corpus collosum, affecting right greater than left side (Figure 1). There was no consanguinity or family history of similar symptoms. A genetic panel returned with variants in gene POLR3A, c.638G>A and c.1070C>T. We suspect these gene changes were pathologic since the clinical phenotype and MRI ﬁndings are consistent with previously reported cases of POLR3A gene mutation, though our patient had later age of onset. Whole exome sequencing and endocrine evaluation is underway. Conclusions: POLR3-related leukodystrophy can have a wide phenotypic spectrum including neurologic dysfunction and endocrine, ocular, and dental abnormalities. This diagnosis should be considered in children and young adults with suggestive clinical and imaging ﬁndings. References: 1. Bernard G, Vanderver A. POLR3-Related Leukodystrophy.; 1993. http://www.ncbi.nlm.nih.gov/pubmed/22855961. Accessed September 30, 2019.2. Wolf NI, Vanderver A, Van Spaendonk RML, et al. Clinical spectrum of 4H leukodystrophy caused by POLR3A and POLR3B mutations. Neurology. 2014;83(21):1898-1905. 3. Daoud H, Tétreault M, Gibson W, et al. Mutations in POLR3A and POLR3B are a major cause of hypomyelinating leukodystrophies with or without dental abnormalities and/or hypogonadotropic hypogonadism. J Med Genet. 2013;50(3):194-197. Rating Scales 205 Item Response Theory Analysis of the MDS-UPDRS Part III Michelle Tosin (Santos, Brazil), Christopher Goetz (Chicago, IL, United States), Sheng Luo (Durham, NC, United States), Glenn Stebbins (Chicago, IL, United States) Objective: To investigate the multidimensional measurements of MDS-UPDRS Part III using the Item Response Theory (IRT) approach. Background: The International Parkinson and Movement Disorder Society revision of the Uniﬁed Parkinson’s Disease Rating Scale (MDSUPDRS) has become the most widely used scale for measuring parkinsonian symptoms in clinical and research practice (1). As part of the continuous development of a rating scale, periodic review of its clinimetric properties is necessary.Examination of clinimetric properties of the MDSUPDRS have employed Classical Test Theory (CTT) approaches to psychometric evaluation focused on the relationship of individual items to the entire scale (2). CTT results have demonstrated acceptable levels of internal consistency, inter-rater reliability, factor structure, sensitivity to change, minimal clinical important change, and differential item functioning (3). However, there has been limited investigation of the MDSUPDRS focusing on the measurement characteristics of the items in relation to the individual trait being measured by the scale, as proposed by Item Response Theory (IRT) (4). To address this limitation, we sought to measure the individual item characteristics of the MDS-UPDRS Part MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS III (motor examination) to the latent model of Parkinsonian severity in a large sample of Parkinson’s disease (PD) patients. Methods: The data from the MDS-UPDRS translation program were used to analyze the 33 MDS-UPDRS Part III items (6,298 records with complete scores).A graded-response IRT analysis was conducted using the mirt RStudio (5) and quantiﬁed the strength of relationship between measured item and the parkinsonian motor symptoms (discrimination) and calculated the threshold of each item response to the global domain accordantly to each level of item severity: from 0 to 1 (from normal to slight), from 1 to 2 (from slight to mild), from 2 to 3 (from mild to moderate), from 3 to 4 (from moderate to severe). Item Characteristic Curves (ICC) were used to plot the item scaling level. Results: Whereas high discrimination values were achieved for items other than tremor, low discrimination and poor thresholds occurred for postural, kinetic and resting tremor (Table 1). Segregating the tremor items from the remaining Part III items resulted in markedly improved discrimination and threshold values for overall Part III (Table 2). Conclusions: These results suggest that the items representing the various elements of Parkinsonian tremor may need to be remodeled, tested and analyzed separately from the other MDS-UPDRS Part III items and may be measuring signs related to other mechanisms than those underlying the rest of the Part III items. References: 1. Goetz CG, Tilley BC, Shaftman SR, Stebbins GT, Fahn S, Martinez-Martin P, et al. Movement Disorder Society-sponsored revision of the Uniﬁed Parkinson’s Disease Rating Scale (MDSUPDRS): scale presentation and clinimetric testing results. Mov Disord [Internet]. 2008;23(15):2129–70. 2. DeVellis RF. Classical Test Theory. Med Care [Internet]. 2006 Nov;44(Suppl 3):S50–9. 3. Goetz CG, Stebbins GT, Tilley BC. Calibration of uniﬁed Parkinson’s disease rating scale scores to Movement Disorder Society-uniﬁed Parkinson’s disease rating scale scores. Mov Disord [Internet]. 2012 Sep 1;27(10):1239–424. Kean J, Reilly J. Item response theory. Lang Test. 1985;2(2):197.5. Chalmers RP. mirt: A Multidimensional Item Response Theory Package for the R Environment. J Stat Softw [Internet]. 2012;48(6). MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S91 ABSTRACTS 206 Brazilian Portuguese Version of the Frenchay Dysarthria Assessment (FDA-2): Preliminary Results in Parkinson’s Disease Ana Teresa Britto (Belo Horizonte, Brazil), Tiago Attoni (Lagoa Santa, Brazil), Victor Quintas (Belo Horizonte, Brazil), Larissa Baracho (Contagem, Brazil), Hugo de Resende (, Portugal), Francisco Cardoso (Belo Horizonte, Brazil), Rui Rothe-Neves (Belo Horizonte, Brazil) Objective: The goal of this study is to cross-culturally adapt and evaluate the reliability and applicability of the Frenchay dysarthria assessment (FDA-2) for Brazilian Portuguese-speaking Parkinson’s disease patients. Background: Previous studies have suggested that the Frenchay dysarthria assessment (FDA-2) is a reliable, standardized protocol for dysarthria evaluation in Parkinson’s disease patients. FDA-2 is a 5-point classiﬁcatory scale with which the Speech Therapist (ST) assesses seven different aspects of oromotor movements and speech performance: reﬂexes, respiration, lips, palate, laryngeal, tongue, and intelligibility. So, the FDA-2 combines observation of oral structures and non-verbal oral functions, assessment of speech characteristics, and measurement of intelligibility. The critics notwithstanding, one ﬁnds FDA-2 versions in French, Swedish, German and European Portuguese. S92 Methods: We translated FDA-2 for research purposes according to cross-cultural adaptation standards. Thirty patients (10 female) diagnosed with idiopathic Parkinson’s disease and hypokinetic dysarthria were evaluated with FDA-2 by an ST before (OFF condition) and after (ON condition) intake of dopamine mimetic medication. A certiﬁed neurologist administered the MDS-UPDRS – Part III and established the disease stage (Hoehn & Yahr scale). The neurologist rated dysarthria severity on item 3.1 in the MDS-UPDRS. FDA scores both in OFF condition, W = 0.93, p = .04, and in ON condition, W = 0.82, p < .01, were signiﬁcantly non-normal. For reliability scores, we calculated Cronbach’s a (alpha) for all items and each subscale, except for the twoitems ‘Respiration’ subscale for which we calculated Spearman’s correlation coefﬁcient. For comparison purposes, we reliability was estimated only in the ON condition. Results: On average, male and female participants did not score differently on FDA either in OFF condition (W = 99.5, p = 0.98, r = –.004) or in ON condition (W = 119, p = 0.41, r = –.15). The same holds for UPDRS (OFF: t(18.66) = 0.97, p = .34, r = 0.22; ON: t (20.21) = –0.30, p = .76, r = 0.07). As male and female participants did not differ as to the other sample characteristics either (Table 1), we considered all participants in a single group. Six out of seven subscales of the FDA-2 had high-reliability scores, Cronbach’s a around .7 or higher MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Table 1 – Sample characteristics (Mean SD) Males (N = 20) 57.95 11.39 13.65 3.38 11.3 5.26 Age (in years) School years Years from Diagnosis Females (N = 10) 56.1 7.38 11.2 3.55 11.2 4.1 Average (N = 30) 57.33 10.14 12.83 3.57 11.27 4.83 Differencea t = –0.53, p = .6 t = –1.81, p = .09 t = –0.06, p = .95 a.Welch Two Sample t-test Table 2 – Reliability scores Section Reﬂexes Respiration Lips Palate Laryngeal Tongue Intelligibility N. items 3 2 5 3 4 6 3 Median 12 8 18.75 12 14 20.5 12 Mean 11.68 7.67 18.1 11.8 14.18 20.08 11.5 SD 1.26 0.56 2.18 0.47 2.1 3.46 1.07 Range (0–12) (0–8) (0–20) (0–12) (0–16) (0–24) (0–12) Alpha coefﬁcient 0.69 0.425a (p = .02) 0.84 0.5 0.8 0.87 0.77 a.This value represents Spearman’s Correlation Table 3 – Effect of Medication FDA-2 Total score (M SD) Possible Maximum = 104 MDS-UPDRS III (M SD) Possible Maximum = 132 Item 3.1 Hoehn and Yahr stagec Off 91.67.75 49.7713.03 1.400.89 2 On 94.789.14 36.7316.15 1.130.90 2 Difference Va = 359.5, p < .01 t(29)b = 6.4, p < .001 Va = 55, p = .03 Va = 231, p < .001 a.Wilcoxon rank sum test b.Paired t-test (degrees of freedom) c.Median (Table 2). The Palate subscale was an exception (a = .5) as all but one patient performed as ‘normal task/movement/sound’ in the ﬁrst item (‘Fluids’). Nonetheless, the total a score for all FDA-2 items was very high (.93). A comparison between the conditions showed a signiﬁcant effect of medication on both FDA-2 and UPDRS scores (Table 3). Conclusions: Our data showed that the Brazilian Portuguese version of FDA-2 is reliable and able to tap into the effects of medication on oromotor movements and speech performance as evaluated by an ST. However, the Palate subscale was unreliable. This result may reﬂect the fact that in our sample dysarthria is not severe: in 19 out of 23 FDA2 items, the median = 4 (the highest possible score). As is known, low variation in score range impacts Cronbach’s alpha negatively. So, our results are considered as provisional and, therefore, we postponed conﬁrmatory factor analysis. References: Auzou, P., Ozsancak, C., Jan, M., Leonardon, S., Menard, J. F., Gaillard, M. J., … Hannequin, D. (1998). Clinical assessment of dysarthria: Presentation and validation of a method. Revue neurologique, 154(6–7), 523–530. Cardoso, R., Guimar~aes, I., Santos, H., Loureiro, R., Domingos, J., de Abreu, D., … Ferreira, J. (2017). Frenchay dysarthria assessment (FDA-2) in Parkinson’s disease: Crosscultural adaptation and psychometric properties of the European Portuguese version. Journal of neurology, 264(1), 21–31. Enderby, P. (1980). Frenchay dysarthria assessment. British Journal of Disorders of Communication, 15(3), 165–173. Enderby, P. M., & Palmer, R. (2008). FDA-2: Frenchay dysarthria assessment: examiner’s manual. Pro-ed.Field, A., Miles, J., & Field, Z. (2012). Discovering statistics using R. Sage publications. Goetz, C. G., Tilley, B. C., Shaftman, S. R., Stebbins, G. T., Fahn, S., Martinez-Martin, P., … Dodel, R. (2008). Movement Disorder Society-sponsored revision of the Uniﬁed Parkinson’s Disease Rating Scale (MDS-UPDRS): Scale presentation and clinimetric testing results. Movement disorders: ofﬁcial journal of the Movement Disorder Society, 23(15), 2129–2170. Knuijt, S., Kalf, J. G., van Engelen, B. G., de Swart, B. J., & Geurts, A. C. (2017). The Radboud dysarthria assessment: Development and clinimetric evaluation. Folia Phoniatrica et Logopaedica, 69(4), 143–153. Pinto, S., Chan, A., Guimar~aes, I., RotheNeves, R., & Sadat, J. (2017). A cross-linguistic perspective to the study of dysarthria in Parkinson’s disease. Journal of Phonetics, 64, 156–167. Ziegler, W., Staiger, A., Schölderle, T., & Vogel, M. (2017). Gauging the auditory dimensions of dysarthric impairment: Reliability and construct validity of the Bogenhausen Dysarthria Scales (BoDyS). Journal of Speech, Language, and Hearing Research, 60(6), 1516–1534. Sleep Disorders and Restless Legs Syndrome 200 Comparison of Polisomnographic Features in Different Parkinsonisms Omar Cardenas (Ciudad de Mexico, Mexico), Mayela Rodriguez Violante (Mexico City, Mexico), Amin Cervantes (Mexico City, Mexico), Vanessa Alatriste (Mexico City, Mexico), Rodolfo Abundes-Corona (Mexico City, Mexico), Fanny Herrera (San Pedro Sula, Honduras), Emmanuel Escobar (Monterrey, Mexico), Oscar Esquivel-Zapata (Monterrey, Mexico), Susana Lopez-Alamillo (Villahermosa, Mexico), Angel Alcocer Salas (Ciudad de Mexico, Mexico), Cynthia Sarabia-Tapia (Mexico City, Mexico), Yazmin Rios (Ciudad de Mexico, Mexico), Fernando Arvizu (Mexico City, Mexico) Objective: To identify if there is a difference in the prevalence of RBD, PLM and OSAS between patients with Parkinson’s disease (PD), Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) and to describe the duration of each sleep stage. Background: There is a recognized association of RBD and neurodegenerative diseases; but periodic limb movements (PLM) and obstructive sleep apnea syndrome (OSAS) may coexist with these disorders. Evidence suggest that the structure of the sleep phases is also different in this patients. Methods: Cross-sectional, observational study. We included patients with the diagnosis of PD, MSA or PSP that had a polysomnographic study for any mean, from January 2015 to july 2018. The diagnosis of RBD, PLM and OSAS was conﬁrmed based on the practice guidelines of the American Academy of Sleep Medicine (AASM). We performed the Kolmogorov-Smirnov test to test normality. We compared groups using a X2 or Kruskal-Wallis test for categorical or numerical variables. A p value of <0.05 was used. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S93 ABSTRACTS Sex Male(%) Age Median (Range) Fases del Sueño% Stage 1 Median (Range) Stage 2 Median (Range) Stage 3 Median (Range) REM Median (Range) *X2 **K-W OSAS N/A (%) No (%) Low (%) Mid (%) Severe (%) PLM N/A (%) No (%) PLM (%) RBD N/A (%) No (%) RBD (%) Subclinic (%) REM without atonia (%) *X2 PD MSA PSP 106 (55.2) 3 (30) 6 (60) 61 (17-86) 65 (45-70) 60.5 (49-69) 9.1% (0-71.3) 9.75% (0-20.4) 14.8% (9.5-79.2) 59.75% (6.6-98.4) 48.95% (0-67.2) 31% (14.4-57.8) 14% (0-91.4) 22.7% (8.7-45.1) 13.1% (0-40.1) 13.42% (0-36.9) 11.2% (0-23.7) 9.7 (0-27.3) 0.64** 0.013** <0.001** 0.067** 0.74** PE MSA PSP 6 (3.1) 86 (44.8) 48 (25) 20 (10.4) 32 (16.7) 1 (10) 3 (30) 3 (30) 1 (10) 2 (20) 6 (60) 0 1 (10) 0 3 (30) 6 (3.1) 128 (66.7) 58 (30.2) 1 (10) 3 (30) 6 (60) 5 (50) 2 (20) 3 (20) 5 (2.6) 117 (60.9) 56 (29.2) 7 (3.6) 7 (3.6) 0 2 (20) 6 (60) 0 2 (20) 4 (40) 3 (30) 2 (20) 0 1 (10) P <0.001* <0.001* <0.001* Results: We included 192 patients with PD, 10 with MSA and 10 with PSP. We found that patients with MSA (60%) were diagnosed more frequently with PLM than patients with PD (30.2%) (p<0.05). There was no signiﬁcant difference with RBD and OSAS. Patients with PSP spend more time in stage 1 (14.8%, 9.5-79.2) than patients with PD (9.1%, 0-71.3) and MSA (9.75%, 0-20.4) (p<0.05). Patients with PD spend more time in Stage 2 (59.7%, 6.6-98.4) than patients with PSP (31%, 14.4-57.8) (p<0.05). Conclusions: The present study found that patients with MSA were diagnosed more frequently with PLM compared with PD and PSP, with no difference in the prevalence of RBD and OSAS between different groups. In relation to sleep phases; we found differences in the duration of non-REM phases in the different parkinsonisms. Further studies with a bigger sample size are needed to demonstrate this. References: 1.- Zanigni, S et al. REM behaviour disorder and neurodegenerative diseases. Sleep Medicine 12 (2011) S54-S58. 2.- Ding Y et al. Comparison Study of Polysomnographic Features in Multiple System Atrophy-cerebellar Types Combined with and without Rapid Eye Movement Sleep Behavior Disorder. Chin Med J 2016;129:2173-7 Surgical Therapy (Parkinson’s Disease and Other Movement Disorders) 101 Vertically-Stacked Single Segment Activation (V-SSA) as a Programming Approach for Directional DBS in GPi: First Clinical Case Series M. Waseem Anjum (Germantown, MD, United States), Islam Fayed (Washingtonw, DC, United States), Yasar Torres-Yaghi (Washington, DC, United States), Srivatsan Pallavaram (Austin, TX, United States), Simeng Zhang (Austin, TX, United States), Fahd Amjad (GAithersburg, MD, United S94 P 0.27* States), Christopher Kalhorn (Washingtonw, DC, United States), Fernando Pagan (McLean, VA, United States) Objective: Programming approach to optimize outcomes in Parkinson’s disease patients implanted in the globus pallidus internus (GPi) with directional deep brain stimulation systems and to analyze the volumes of tissue activation (VTAs) in the globus pallidus. Background: Subthalamic nucleus and GPi have been shown to be effective DBS targets for the treatment of advanced Parkinson’s disease. In patients with risk of cognitive decline, GPi is the preferred target for many clinicians. Furthermore, studies have indicated that stimulation of the globus pallidus (GP) as a whole had the most robust effect on bradykinesia. However, unless the DBS lead is placed proximal to the border of the GPe and GPi, it may not be possible to stimulate GP as a whole using conventional (non-directional) stimulation without stimulating neighboring structures, such as the internal capsule, and producing stimulation induced side effects. In this work, we report the ﬁrst clinical case series using directional DBS in the GP for PD. Methods: We conducted a retrospective case series of patients undergoing implantation of directional GPi-DBS. During monopolar review, we tested non-directional and directional electrode montages to determine the optimum therapeutic window (TW). We refer to co-activation of single segments aligned in the same direction from two segmented levels (e.g. 2A-3A, 2B-3B or 2C-3C) as vertically-stacked single segment activation (V-SSA). For post-hoc VTA analysis, we extracted the location and orientation of the DBS leads and contacts by combining postop CT, preop MRI, and a non-rigidly co-registered anatomical atlas2. We computed VTAs for the clinical settings using Sim4Life v4.0 and the MIDA model3 that accounts for tissue heterogeneity. Results: Review of the clinical settings of 7 GPi-DBS implantations in 4 PD patients optimized for outcomes and TW revealed that V-SSA (ﬁg. 1) was used in 6 out of the 7 implants. For these implants, mean and median VTA overlaps with the GP were 89% (12%) and 93%, respectively. The mean and median of ratio of VTAs in the GPe to GPi were MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS 5.75 (11.08) and 1.3, respectively. The mean settings were 2.67 mA at 67 μs and 153 Hz. The implant that was not on V-SSA was on a complex bipolar montage with only 60% of its VTA in the GP. It had a ratio of VTA in the GPe to GPi of 0.5 and settings of 2.1 mA at 150 μs (2.25 times that for the V-SSA leads) and 180 Hz. The bipolar montage is explained in ﬁg 2. with the lead appearing too medial. Conclusions: The results from this ﬁrst clinical case series using directional DBS leads in GPi for PD suggest that vertically-stacked single segment activation (V-SSA) may optimize therapy while focusing activation volumes to within the globus pallidus. A programming approach that prioritizes V-SSA screening may reduce time required to arrive at optimal settings. Consistent with some earlier work with non-directional Figure 1. DBS electrodes location in VIM nucleus of thalamus, all examinated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair sequence of RMI. C. Axial Flair sequence of MRI. D. 3D Reconstruction of VIM nucleus. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S95 ABSTRACTS leads, the optimal stimulation region may not necessarily be restricted to GPi but some combination of GPe and GPi. The study is limited to a small sample size since this is the ﬁrst clinical case series to be reported on the use of directional DBS in GPi DBS implantations. References: Johnson MD,Zhang J,Ghosh D, McIntyre CC, VitekJL. Neural targets for relieving parkinsonian rigidity and bradykinesia with pallidal deep brain stimulation. J Neurophysiol. 2012 Jul 15; 108(2): 567–577. Horn, A., Li, N., Dembek, T. A., Kappel, A., Boulay, C., Ewert, S., et al. (2018). Lead-DBS v2: Towards a comprehensive pipeline for deep brain stimulation imaging. NeuroImage. Iacono MI, Neufeld E, Akinnagbe E et al. MIDA: a multimodal imaging-based detailed anatomical model of the human head and neck. PLoS One. 2015;10: e0124126. Figure 2. DBS electrodes location in VIM nucleus of thalamus, all examinated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair sequence of RMI. C. Axial Flair sequence of MRI. D. 3D Reconstruction of VIM nucleus. Figure 3. DBS electrodes location in VIM nucleus of thalamus, all exanimated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair sequence of RMI. C. Axial Flair sequence of MRI. D. 3D Reconstruction of VIM nucleus. S96 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS 102 Use of a New Tool to Correlate the Clinical and Radiological Findings after Deep Brain Stilumation in Patients with Movement Disorders Andrea Camargo (Bogotá, Colombia), Francesc Valldeoriola (Barcelona, Spain), Almudena Sánchez-Gómez (Barcelona, Spain) Objective: Demostrate that the clinical ﬁndings found in the neurological evaluation of the patient are related to the positioning of the brain electrodes in the NST or the VIM shown by the Suretune program.Evaluated the clinical effects of DBS with the Uniﬁed Parkinson’s disease Rating Scale (UPDRS) for PD and the scale for the Fahn-Tolosa tremor Marín for ET and evaluated the quality of life with the Parkinson’s disease questionnaire 8 (PDQ8 scale). -Describe the adverse effects seen Figure 4. DBS electrodes location in VIM nucleus of thalamus, all exanimated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair sequence of RMI. C. Axial Flair sequence of MRI. D. 3D Reconstruction of VIM nucleus. Figure 5. DBS electrodes location in NST, all exanimated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair of RMI. C. Axial Flair sequence of RMI. D. 3D Reconstruction of NST (green ﬁgure) and red nucleus (red ﬁgure). MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S97 ABSTRACTS with acute deep brain stimulation and eventually the follow-up of patients at two months. Background: DBS has become a new option for management of movement disorders that are refractory to treatment or that have many side effects. The purpose of this study was to demonstrate that the position of DBS electrode in the basal ganglia nuclei (STN or VIM ) through the use of SureTune3 program which is related to the clinical outcomes and side effects. Methods: Descriptive and prospective study. Ten consecutive patients with Parkinson disease or Essential tremor were enrolled. A pre- Figure 6. DBS electrodes location in NST, all exanimated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair of RMI. C. Axial Flair sequence of RMI. D. 3D Reconstruction of NST (green ﬁgure) and red nucleus (red ﬁgure). Figure 7. DBS electrodes location in NST, all exanimated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair of RMI. C. Axial Flair sequence of RMI. D. 3D Reconstruction of NST (green ﬁgure) and red nucleus (red ﬁgure). S98 MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS surgical evaluation was performed and 3 months of follow-up; UPDRS or Fahn-Tolosa-Marín tremor rating to evaluated motor and NMS; and the quality of life scale in Parkinson’s disease PDQ8. The software Suretune3 was used to evaluate the positioning of the DBS electrodes. Results: Ten patients were included, six with PD, and four with ET. SureTune 3 showed us that three patients have both electrodes well localized in the basal ganglia nucleus; in one patient the electrodes were out of the target. The principals AE were dysarthria, behavioral Figure 8. DBS electrodes location in NST, all exanimated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair of RMI. C. Axial Flair sequence of RMI. D. 3D Reconstruction of NST (green ﬁgure) and red nucleus (red ﬁgure). Figure 9. DBS electrodes location in NST, all exanimated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair of RMI. C. Axial Flair sequence of RMI. D. 3D Reconstruction of NST (green ﬁgure) and red nucleus (red ﬁgure). MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S99 ABSTRACTS Figure 10. DBS electrodes location in NST, all exanimated electrodes are displayed together with neuroanatomical structures. The ﬁgure represents the head located at the lower left edge of the box allows the spatial location (anterior, posterior and lateral) of the electrode. A. Coronal ﬂair sequence of RMI. B. Sagittal ﬂair of RMI. C. Axial Flair sequence of RMI. D. 3D Reconstruction of NST (green ﬁgure) and red nucleus (red ﬁgure). changes and paresthesias. One patient did not improvement his symptoms. Conclusions: The SureTune 3 program is an effective tool to evaluate the position of the DBS electrodes and it is associated to the efﬁcacy of the surgery and the presence of side effects. References: Kalia L V, Lang AE, Shulman G. Parkinson’s disease 2015;386:896–912. 2. Neurochemistry JOF. Treatment of Parkinson’s disease The motor symptoms of Parkinson’s disease 2016:1–7. 3. Pfeiffer RF. Parkinsonism and Related Disorders Non-motor symptoms in Parkinson’s disease 2015:7–10. 4. Reichmann H. Premotor Diagnosis of Parkinson’s Disease 2017;33:526–34. 103 Safety of Non-Contrast Imaging Guided DBS Electrode Placement in Parkinson’s Disease Joacir Graciolli Cordeiro (Miami, FL, United States), Anthony Diaz (Miami, FL, United States), Jenna Davis (Miami, FL, United States), Ghulam Farooq (Phoenix, AZ, United States), Daniel Garbin Di Luca (Miami Beach, FL, United States), Corneliu Luca (Miami, FL, United States), Jonathan Jagid (Miami, FL, United States) Objective: To examine the safety and describe the University of Miami experience with non-contrast imaging guided DBS lead placement. Background: To date, the placement of brain electrodes for deep brain stimulation for Parkinson’s disease (PD) is performed using contrast imaging to enhance blood vessel identiﬁcation during stereotactic planning. Although rare, the use of IV contrast is associated with potentially serious clinical complications. Methods: Data was retrospectively obtained by electronic chart review evaluating all DBS surgery cases performed with non-contrast imaging at the University of Miami between 2012 and 2018. Clinical features, postoperative imaging and complications were analyzed. Results: A total of 287 deep seated electrodes were implanted in 152 patients. Leads were placed at the subthalamic nucleus (STN) and globus pallidus internus (GPi) in 258 and 29 hemispheres, respectively. We identiﬁed three cases with perioperative complications, being two cases of intracranial hemorrhage and one case with signiﬁcant peri-lead edema. S100 Conclusions: The rate of hemorrhagic complication was in line with other reported series in the DBS literature. Although the hemorrhagic complication rate from DBS is low and acceptable, this report suggests that enhanced imaging is not necessary to maintain this low complication rate. Given the potential risks of contrast enhanced imaging, the lack of need for this imaging modality may serve to improve the safety proﬁle of deep brain stimulation surgery. References: 1. Ponce FA, Lozano AM. Deep brain stimulation state of the art and novel stimulation targets. Prog Brain Res. 2010;184:311–24. 2. Hertel F, Züchner M, Weimar I, Gemmar P, Noll B, Bettag M, et al. Implantation of electrodes for deep brain stimulation of the subthalamic nucleus in advanced Parkinson’s disease with the aid of intraoperative microrecording under general anesthesia. Neurosurgery. 2006 Nov;59(5):E1138; discussion E1138. 3. Clement O, Dewachter P, Mouton-Faivre C, Nevoret C, Guilloux L, Bloch Morot E, et al. Immediate Hypersensitivity to Contrast Agents: The French 5-year CIRTACI Study. EClinicalMedicine. 2018 Jul 28;1:51–61. 4. Zsigmond P, Hemm-Ode S, Wårdell K. Optical Measurements during Deep Brain Stimulation Lead Implantation: Safety Aspects. Stereotact Funct Neurosurg. 2017;95(6):392–9. 104 Patient Characteristics and Safety of Deep Brain Stimulation Systems: Geographic Comparisons in a Real-World Population Alfonso Arellano Reynoso (City of Mexico, Mexico), Adriana-Lucia Lopez-Rios (Medellin, Colombia), Fabian Piedimonte (Buenos Aires, Argentina), Soﬁa Roa (Bogata, Colombia), Hans Carmona (Pereira, Colombia), Hui Xiong (Minneapolis, MN, United States), Keisha Sandberg (Minneapolis, MN, United States), Todd Weaver (Minneapolis, MN, United States) Background: Deep Brain Stimulation (DBS) has become a wellestablished therapy in the treatment of movement disorders such as Parkinson’s disease, essential tremor, and dystonia. However, real world long-term data speciﬁc to varying global populations is needed to better understand and characterize patient and product outcomes, provide insights in how DBS is utilized, and improve therapy access. Methods: The Product Surveillance Registry (PSR) is a prospective, long-term, multi-center global registry that started enrollment in DBS in MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Table 1: Device Indication Indication Parkinson’s Disease Essential Tremor Dystonia Other Indications % of Patients USA (n=1423) 62.5% (890) 30.3% (431) 6.2% (88) 1.0% (14) % of Patients Europe (n=910) 66.5% (605) 15.2% (138) 9.9% (90) 8.5% (77) % of Patients Latin America (n=160) 65.0% (104) 4.4% (7) 19.4% (31) 11.2% (18) % of Patients Overall (n=2493) 64.1% (1599) 23.1% (576) 8.4% (209) 4.4% (109) Table 2: Demographics (Therapy Naïve Patients) Variable Male Female Age at ﬁrst implant % / Mean of Patients USA (n=1109) 60.5% (671) 39.5% (438) 63.8 (10.5-87.8) % / Mean of Patients Europe (n=485) 64.7% (314) 35.3% (171) 57.4 (16.9-80.0) % / Mean of Patients Latin America (n=129) 63.6% (82) 36.4% (47) 51.8 (8.0-78.0) Table 3: Infection Rate (Leading to Surgical Intervention) Initial Device Replacement Device USA % (n/N) 3.8% (42/1109) 3.1% (9/292) Europe %(n/N) 3.5% (17/485) 6.6% (28/425) Latin America %(n/N) 3.1% (4/129) 9.7% (3/31) P-Value* 0.9780 0.0468 *Fisher’s Exact test 2009. The Latin America population represents centers in Colombia (3), Argentina (1), Brazil (1), and Mexico (1). The PSR enrolls patients receiving either their ﬁrst DBS implant or a replacement neurostimulator. Patients are followed prospectively for events related to the device, procedure, and/or therapy. Results: As of April 30, 2019, 39 centers worldwide have enrolled over 2,400 deep brain stimulation patients implanted in the registry, including 160 patients from Latin America. Indications and demographics were characterized by three geographies: USA, Europe, and Latin America [table 1, table 2]. More than 60% of patients were treated for Parkinson’s disease regardless of geography. Essential tremor was more commonly treated with DBS in the USA (30.3%), whereas the highest percent of patients treated for Dystonia was in Latin America (19.4%). More than 60% of patients treated with DBS globally were male. Patients in Latin America were younger at ﬁrst implant compared to other geographies and may reﬂect their higher percent of patients treated for Dystonia. Infection rates (leading to surgical intervention) were also compared across geographies as shown in Table 3 [table 3]. For newly implanted patients, they were similar across geographies (p-value=0.9780). For replacement patients, the infection rate was higher outside the US, but comparisons are limited by the sample size in other geographies. Speciﬁc to neurostimulator implants, there were 94 TYRX envelopes used in US patients, 2 in European patients, and 15 in Latin American patients and no infections leading to surgical intervention were reported with TYRX use. Quality of life outcomes are pending and will be presented. Conclusions: Information collected in the PSR can be used to better understand the performance and use of DBS systems globally and provide insight into both product related and non-product related issues with a goal of guiding future product development. 105 Deep Brain Stimulation Systems: Geographic Comparisons of Procedural Information in a Real-World Population Alfonso Arellano Reynoso (City of Mexico, Mexico), Adriana-Lucia Lopez-Rios (Medellin, Colombia), Fabian Piedimonte (Buenos Aires, Argentina), Soﬁa Roa (Bogata, Colombia), Hans Carmona (Pereira, Colombia), Keisha Sandberg (Minneapolis, MN, United States), Todd Weaver (Minneapolis, MN, United States) Background: Deep Brain Stimulation (DBS) has become a wellestablished therapy in the treatment of movement disorders such as Parkinson’s disease, essential tremor, and dystonia. However, real world long-term data speciﬁc to varying global populations is needed to better understand and characterize patient and product outcomes, provide insights in how DBS is utilized, and improve therapy access. Methods: The Product Surveillance Registry (PSR) is a prospective, long-term, multi-center global registry that started enrollment in DBS in 2009. The Latin America population represents centers in Colombia (3), Argentina (1), Brazil (1) and Mexico (1). The PSR enrolls patients receiving either their ﬁrst DBS implant or a replacement neurostimulator. Patients are followed prospectively for events related to the device, procedure, and/or therapy. Results: To date, 39 centers worldwide have enrolled over 1,700 deep brain stimulation patients receiving their ﬁrst implant (therapy naïve) in the registry, including 129 patients from 6 centers from Latin America. To provide a point of comparison, procedural information was summarized for therapy naïve patients between the USA, Europe and Latin America as shown below [table 1] [table 2] [table 3]. The greatest percentage of rechargeable devices were used in Latin American countries. StarFix and Leksell were the most common types of stereotactic equipment in the US and Europe, whereas Riechert Mundiger was more Table 1: Device Type Device Model Activa PC Activa SC Activa RC Other % of Implants USA (n=1105) 71.9% 20.0% 7.0% 1.1% % of Implants Europe (n=483) 81.4% 4.1% 10.8% 3.7% % of Implants Latin America (n=129) 24.8% 0.0% 75.2% 0.0% Table 2: Stereotactic Equipment Used % of Implants % of Implants % of Implants Latin USA (n=1108) Europe America (n=481) (n=129) Leksell 44.0% 69.4% 48.8% StarFix 48.3% 0.0% 0.0% Radionics CRW 7.1% 19.3% 0.0% Riechert Mundiger 0.0% 9.1% 20.2% Other 0.6% 2.2% 31.0% Equipment Name MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S101 ABSTRACTS 107 Table 3: Imaged Guided Navigation Equipment Name % of Implants USA (n=1108) % of Implants Europe (n=481) Stealthstation Leksell Stryker Leibinger BrainLab Other 42.7% 0.2% 0.5% 50.3% 0.6% 2.7% % of Implants Latin America (n=129) 0.8% 23.3% 20.2% 4.1% 52.6% 7.3% 39.1% 0.0% 55.8% likely in Latin American countries. Stealthstation Framelink were more commonly selected in Europe and the USA, whereas Leksell and Stryker Leibinger were more likely used in Latin America. Conclusions: Information collected in the PSR can be used to better understand the performance and use of DBS systems across geographies including centers in several Latin American countries; providing insight into both product related and non-product related issues with a goal of guiding future product development and better patient outcomes. 106 Adjustment of Stimulation to Dorsal Contacts Resolves STN Stimulation Induced Hypomania Anant Wadhwa (New Haven, CT, USA), Amar Patel (New Haven, CT, USA), Sara Schaefer (Guilford, CT, USA) Objective: Mood disorders and psychosis have been described as side effects of stimulation of the subthalamic nucleus (STN). While various strategies have been tried to address these issues, a clear consensus on management is lacking. Our case builds on anecdotal evidence of resolution of these side effects by switching stimulation to dorsal contacts. Methods: A 64-year-old male with essential tremor (ET) and Parkinson’s disease (PD) was treated with bilateral STN deep brain stimulation (DBS) for medically refractory, large amplitude rest and action tremor. Rest tremor had been refractory to sinemet 25/100mg TID. A postoperative head CT revealed appropriate placement of Medtronic 3389 leads caudal to the thalami bilaterally. Initial programming of singlechannel IPGs using parameters (1-, c+; 2.5 V, 60 us, 180 Hz), led to hypomania characterized by elated mood, pressured speech and restlessness. Results: These symptoms resolved after adjusting stimulation to more dorsal contacts (2-,3-,c+; 2.8 V, 60 us, 180 Hz) with improvement in tremor and bradykinesia. Conclusions: STN stimulation can cause psychiatric side effects of mania and psychosis. The differential diagnosis of stimulation-induced mania includes malpositioned leads stimulating ventral midbrain. Postoperative neuroimaging is recommended for this reason. When stimulation causes immediate manic symptoms, adjusting stimulation parameters to dorsal contacts is the most effective strategy for reducing these symptoms while preserving motor beneﬁts. Prior reports have mentioned failed strategies including levodopa reduction, electrode repositioning caudal to STN and turning the stimulator off. If post-operative mania does not improve with adjustment of stimulation parameters, additional interventions include addition of antipsychotic medications. References: 1. Antosik-Wójcinska A, Swiecicki L, Dominiak M, Soltan E, Bienkowski P, Mandat T. Impact of STN-DBS on mood, drive, anhedonia and risk of psychiatric side-effects in the population of PD patients. J Neurol Sci. 2017;375:342-7. 2. Kulisevsky J, Berthier ML, Gironell A, Pascual-Sedano B, Molet J, Parés P. Mania following deep brain stimulation for Parkinson’s disease. Neurology. 2002;59(9):1421-4. 3. Herzog J. Manic episode with psychotic symptoms induced by subthalamic nucleus stimulation in a patient with Parkinson’s disease Manic Episode After STN-DBS. Mov Disord.18(11):1382-4. 4. RaucherChene D, Charrel CL, de Maindreville AD, Limosin F. Manic episode with psychotic symptoms in a patient with Parkinson’s disease treated by subthalamic nucleus stimulation: improvement on switching the target. Journal of the neurological sciences. 2008;273(1-2):116-7. S102 Fine-tuning Brain Stimulation with Directional Leads Improves Outcomes Nicholas Fleming (Miami, FL, United States), Joacir Graciolli Cordeiro (Miami, FL, United States), Jonathan Jagid (Miami, FL, United States), Corneliu Luca (Miami, FL, United States) Background: Deep Brain Stimulation is and effective procedure in patients with PD and motor ﬂuctuations. The recent availability of directional leads has allowed a more precise control of the area being stimulated in order to obtain clinical beneﬁts and avoid side effects. Methods: We present here the strategy used to program a patient with Parkinson’s disease that underwent DBS with a directional system. Results: Monopolar programing identiﬁed the therapeutic current strength at 0.5 mA with excellent control of tremor bradykinesia and rigidity, however dysarthria was seen at 3 mA. Current was steered anteriorly to avoid dysarthria – threshold for side effects increased to 3.5mA. When further steering of current is used to fractionate the current between 2 contacts medal and anterior, further increase in the threshold for side effects is seen-4mA. Anodal stimulation was employed to further steer the current away from the internal capsule in a medial and anterior direction. In this case, the threshold for side effects increased to 6 mA. Conclusions: This is an illustrative example of current steering strategy employed to increase the therapeutic window and improve patient outcomes. As the segmented leads become widespread, programing strategies should include systematic testing of the directional segments. 108 Correlation of Semi-Macro Stimulation and Macrostimulation Thresholds Sarah Ozinga (Willoughby, OH, United States), Andre Machado (Cleveland, OH, United States), Sean Nagel (Cleveland, OH, United States), Srivatsan Pallavaram (Austin, TX, United States), Binith Cheeran (Austin, TX, United States), Benjamin Walter (Cleveland, OH, United States) Objective: To assess whether capsular side-effects from the microelectrode reference using traditional monopolar semi-macro stimulation correlate with the threshold to capsular side-effects from the permanent DBS lead using bipolar macrostimulation. Background: In subthalamic nucleus deep brain stimulation (STN DBS) for Parkinson’s disease (PD), involuntary muscle contractions may result from current spread in the internal capsule. An intraoperative approach to avoid side-effects is semi-macro stimulation. Methods: Internal capsule side-effect thresholds were documented for 20 PD patients (31 leads) implanted with STN DBS at Cleveland Clinic between January 2018 and November 2019. Thresholds were compared across the microelectrode and the DBS lead when placed along the same trajectory and depth location just below the top of the STN in PD subjects’ brains, as determined by microelectrode recordings. This depth was chosen to optimize use of the DBS lead, placing segmented contact #3 in the dorsolateral portion of the STN [ﬁgure1]. During semi-macro stimulation, the guide tube was used as the reference electrode (anode) and set to ground; whereas the SJM 6172 DBS lead was set to a bipolar conﬁguration with contact #1 as anode and contact #3 as cathode (full-ring via co-activation of segments). Results: Using internal capsule thresholds as reference, signiﬁcant correlation (Pearson’s correlation- 0.63, P<0.0002) was found between the reference contact of the semi-macro electrode and the DBS lead during intraoperative stimulation [ﬁgure2]. Conclusions: Patient response to semi-macro stimulation used for optimal target localization is maintained with the segmented DBS lead. Though differences in lead conﬁgurations and dimensions exist between the electrodes, predicting capsular side-effects during bipolar stimulation of the segmented DBS lead in full-ring mode does not deviate from traditional intraoperative semi-macro stimulation used for DBS lead placement. The current results provide quantitative evidence that semi-macro stimulation provides additional information based on real-time neurophysiology for which clinically meaningful decisions about DBS electrode placement can be based. Further studies are needed to evaluate how closely the side-effect thresholds observed during the intraoperative MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS procedure correlate with postoperative DBS thresholds and thus clinical outcomes. during routine clinical visits. Stable therapeutic stimulation parameters and the length of the STN (i.e. the distance between the MER entry into and exit out of the STN) were documented for each patient. Results: Seventy seven percent of STN DBS patients were actively programmed on directional stimulation [ﬁgure 1], and 55% of patients were programmed on one segment (single segment activation)[ﬁgure 2]. The point-biserial correlation revealed that STN lengths were independent to the use of directional stimulation (P-value, 0.79; r2 value, -0.03 [ﬁgure 3]. Conclusions: Stimulation parameters from the recently published post-market clinical trial (PROGRESS) characterizing clinical performance of the directional DBS lead are consistent with the current patient cohort whose parameters were determined in routine clinical care. Results from the PROGRESS Study showed that 91% of patients (N=202) had a wider therapeutic window using directional stimulation compared to 77% of patients in the current cohort. Even with seemingly optimally placed leads and STN lengths, advances in DBS leads and implantable pulse generators may increase the degrees of freedom to tailor DBS settings by directing stimulation appropriately toward desired targets. Figure 1. Figure 2. Capsular thresholds measured during monopolar semimacro stimulation compared to thresholds during bipolar stimulation with the DBS lead. 109 Directional programming in DBS: The Cleveland Clinic Experience Sarah Ozinga (Willoughby, OH, United States), Erica Hennigs (Cleveland, OH, United States), Jenera Scott (Broadview Heights, OH, United States), Kathy Wilson (Cleveland, OH, United States), Andre Machado (Cleveland, OH, United States), Sean Nagel (Cleveland, OH, United States), Srivatsan Pallavaram (Austin, TX, United States), Binith Cheeran (Austin, TX, United States), Benjamin Walter (Cleveland, OH, United States) Objective: To quantify the use of directional stimulation in PD patients with STN DBS who were programmed in routine clinical care and to determine the utilization of directional programming with STN length based on MER of the implanted track. Background: Subthalamic nucleus deep brain stimulation (STN DBS) is a well-established treatment for Parkinson’s disease (PD). Recent advances in implantable pulse generators and DBS electrodes may increase customization of DBS settings and optimize therapy. Methods: Data from 53 PD patients who were implanted with STN DBS between November 2017 and November 2019 were analyzed retrospectively. Patients underwent DBS programming at Cleveland Clinic Figure 2. Figure 3. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S103 ABSTRACTS 110 Effectiveness of 8 Contact Single Lead DBS: Management of Tremor in a Patient with ET and PD Alison Carlson (Cheshire, CT, United States), Duarte Machado (Hamden, CT, United States) Objective: Examine the effectiveness of a single 8 contact deep brain stimulation (DBS) lead on tremor control in mixed pathology of complex tremor with features of both essential tremor (ET) and Parkinson’s disease (PD). Background: Among the subset of ET patients who present with resting and kinetic tremor, effective and tolerable medication management entails polypharmacy and may provoke objectionable side effects while providing insufﬁcient tremor control. Likewise, resting tremor of PD is often refractory to medication. DBS allows for an electrical approach, using pacemaker generated neuromodulatory treatment within the deep nuclei of the brain. Newer devices with twice the number of contacts on a single lead have enabled more accurate targeting of both the ventral intermediate nucleus (Vim) of the thalamus and the subthalamic nucleus (STN). This technology allows for simultaneous control of both types of tremor using a single lead, with fewer side effects, in appropriate patients. Methods: This case report documents the pre- peri- and postoperative treatment experience of one patient with both ET and PD who underwent bilateral DBS for control of tremor. Results: Our patient is 66 year old left-handed engineer with young onset essential tremor, impacting ﬁne motor skills and voice quality. Around age sixty, he developed left resting tremor, bradykinesia, rigidity, and hypophonia. A DaT scan was positive for dopamine deﬁciency. While ET symptoms were somewhat responsive to both trihexyphenidyl and primidone, side effects of cognitive slowing and sedation, respectively, limited dosing. Resting tremor was improved but not eliminated on dopaminergic therapy. DBS (Vercise, Boston Scientiﬁc) into the bilateral Vim and STN using a single electrode per side was performed and well tolerated. Effective control of bilateral kinetic and postural tremor S104 was achieved at his second programming session, 6 weeks post-operatively. His third programming session targeted activation of additional contacts in the right STN, which provided marked improvement of left resting tremor, allowing for a decrease in medications. Conclusions: This case report is one of only a few to highlight how mixed types of tremor owing to multiple areas of pathologic signaling in the basal ganglia and thalamus can be effectively controlled with an 8 contact single lead DBS, thereby reducing the complexity of medication management and the burden of side effects. References: Deep Brain Stimulation Management, 2nd edn. Ed. William J. Marks, Jr. Published by Cambridge University Press. 2015. Kaptan H. Cakmur R. Technical Case Report of Deep Brain Stimulation: Is it possible Single Electrode Reach to Both of Subthalamic Nucleus and Ventral Intermediate Nucleus in One Stage? Open Access Maced J Med Sci. 2018 Apr 15; 6 (4); 659-662. https://doi. org/10.38889//oamjms.2018.137 111 Deep Brain Stimulation for Essential Tremor: A Single Institution Experience with a Unique Targeting Strategy Anthony Diaz (Miami, FL, United States), Iahn Cajigas (Miami, United States), Anil Mahavadi (Miami, FL, United States), Samir Sur (Miami, FL, United States), Daniel Garbin Di Luca (Miami Beach, FL, United States), Danielle Shpiner (Miami, FL, United States), Corneliu Luca (Miami, FL, United States), Jonathan Jagid (Miami, FL, United States) Objective: To evaluate the long-term clinical outcomes of ventral intermediate nucleus (Vim) deep brain stimulation (DBS) in patients with essential tremor (ET) and explore the use of open-source image normalization tools and stereotactic atlases for visualizing lead location. Background: High frequency stimulation of the Vim is an effective therapy for medication-refractory ET. Due to the difﬁcult visualization of this nucleus with current imaging modalities, suboptimal lead location has been previously noted to be one of the main culprits in waning efﬁcacy. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS Methods: 33 consecutive patients underwent DBS lead implantation using a unique indirect targeting strategy that is reﬁned using nearby anatomical relationships and intraoperative micro-/macro-stimulation. Clinical outcomes were evaluated from ﬁrst and last postoperative visits at a follow up >3 months. Cases were grouped based on signiﬁcant (=75%) vs suboptimal (<75%) tremor control with on-stimulation at last follow up visit. Active contact locations were obtained from reconstructed electrodes in Montreal Neurological Institute (MNI) space using Matlab Lead DBS toolbox. Surface and centroid coordinates of the Vim, subthalamic nucleus (STN), red nucleus (RN), and corticospinal tracts (CST) were also obtained from various MNI atlases to explore distance relationships with the location of active contacts. Results: Mean improvement of tremor control with on-stimulation was observed to be around 79.7 22.4% at a mean follow up of 31.1 18.4 months without a signiﬁcant decline from ﬁrst postoperative visit. Cases with signiﬁcant tremor control had active contact locations signiﬁcantly more inferior and closer to the Vim along the vertical axis. [Figure 1]. Conclusions: Our use of indirect targeting methods that employ standardized brain atlases as well as intraoperative micro-/macro-stimulation, for Vim DBS has resulted in robust clinical outcomes that are sustained long-term. Although active contacts for cases with signiﬁcant tremor control outcomes were signiﬁcantly closer to the Vim along the vertical axis, no other statistical correlations were observed with respect to the distance to either the surface or centroid of the explored subcortical structures. This provides further support that our unique targeting approach of realigning the planned tip location posteriorly and at the apex of the STN results in robust clinical outcomes. References: 1. Rincon F, Louis ED: Beneﬁts and risks of pharmacological and surgical treatments for essential tremor: disease mechanisms and current management. Expert Opin Drug Saf 2005;4:899-913. 2. Garcia Ruiz P, Muñiz de Igneson J, Lopez Ferro O, Martin C, Magariños Ascone C: Deep brain stimulation holidays in essential tremor. J Neurol 2001;248:725-726. 3. Papavassiliou E, Rau G, Heath S, Abosch A, Barbaro NM, Larson PS, Lamborn K, Starr PA: Thalamic deep brain stimulation for essential tremor: relation of lead location to outcome. Neurosurgery 2008;62 Suppl 2:884-894. 4. Horn A, Kühn AA: Lead-DBS: a toolbox for deep brain stimulation electrode localizations and visualizations. Neuroimage 2015;107:127-135. 5. Shih LC, LaFaver K, Lim C, Papavassiliou E, Tarsy D: Loss of beneﬁt in VIM thalamic deep brain stimulation (DBS) for essential tremor (ET): how prevalent is it? Parkinsonism Relat Disord 2013;19:676-679. Therapeutics of Movement Disorders in the Americas (Theme) 117 Withdrawn by Author 118 Nilotinib is Reasonably Safe and May Halt the Disease Progression in Moderately severe Parkinson’s Disease Patients Fernando Pagan (McLean, VA, United States), Michaeline Hebron (Wsahington, DC, United States), Barbara Wilmarth (Washington, DC, United States), Yasar Torres-Yaghi (Washington, DC, United States), Abigail Lawler (Washington, DC, United States), Elizabeth Mundel (Washington, DC, United States), Nadia Yusuf (Washington, DC, United States), Nathan Starr (Washington, United States), M. Waseem Anjum (Germantown, MD, United States), Shahnaz Miri (Washington, DC, United States), Steven Nakano (Silver Spring, MD, United States), Amelia Carwin (Arlington, VA, United States), Myrna Arellano (Washington, United States), Wangke Shi (Washington, United States), Sanjana Mulki (Washington, United States), Tarick Kurd-Misto (Washington, DC, United States), Sara Matar (Washington, United States), Xiaoguang Liu (Washington, DC, United States), Jaeil Ahn (Washington, United States), Charbel Moussa (Washington, DC, United States) Objective: The primary objective was nilotinib safety and tolerability in moderately severe PD patients. An exploratory objective is to assess nilotinib effects on motor and non-motor symptoms at baseline, 6, 12 and 15 months. Background: This is a phase II, randomized, double-blind placebocontrolled trial to evaluate the effects of a potential disease modifying drug in PD. Methods: Participants were conﬁrmed to have PD according to the UK Brain Bank diagnostic criteria, with Hoehn and Yahr stage 2.5-3, MDS-UPDRS-III motor score 20-40 and MoCA =22. 75 participants were randomized 1:1:1 into placebo, 150mg and 300mg nilotinib oral, once daily for 12 months. A total of 100 participants were screened, 25 did not meet the inclusion criteria due to QTc prolongation. Participants were on average 68.4 years of age and included 20 females and 55 males. 88% of participants completed the treatment and there were no drop-outs due to lack of drug tolerability. Results: Niltonib was found to be reasonably safe with no suspected drug related adverse effects. There was no QTc prolongation or myelosuppression. No differences were observed in MDS-UPDRS-I within and between all study groups tested by a single rater. All groups showed a signiﬁcant decline in MDS-UPDRS-II scores between baseline and 12 months and after washout. All groups slightly improved on MDS-UPDRS-III motor between baseline and 6 months. The placebo and the 300 mg nilotinib groups remained stable at 12 months and after washout but there was a signiﬁcant improvement in MDS-UPDRS-III score between baseline and 15 months in the 150 mg nilotinib group (-2.82 points, 95% CI, -4.75- -0.89). No signiﬁcant differences between each visit were observed in MDS-UPDRS-IV. No statistically signiﬁcant differences in MDS-UPDRS measurements were observed between groups. There were no statistically signiﬁcant differences in PDQ-39 between groups, but the placebo group signiﬁcantly deteriorated after 6 months, while the nilotinib groups did not change. Conclusions: In conclusion these exploratory measures show no clinical worsening in MDS-UPDRS in the nilotinib groups compared to the placebo group that worsened after 6 months of treatment. Taken together, our results will guide the future development of a deﬁnitive phase III study to evaluate the effects of nilotinib in PD. 119 Decreased Therapeutic Effect Over Time Amongst Botulinum Toxin Type A Agents Rashid Kazerooni (San Diego, CA, United States) Background: Sufﬁcient long term data comparing BoNT-A agents for antibody formation, resistance, and loss of effect is lacking. IncobotulinumtoxinA is the only botulinum neurotoxin type A (BoNTA) agent that has removed unnecessary proteins, leaving just the 150 kDa active component. Methods: The US Food and Drug Administration (FDA) Adverse Event Reporting System (FAERS) database was utilized. The analysis was conducted on data between March 2014 and June 2019. BoNT-A cases were included when it was considered the “Primary Suspect” drug. The primary outcome was relative rate of decreased therapeutic effect over time by drug, deﬁned as presence of ‘therapeutic response decreased’ and/or ‘drug effect decreased’ being reported as an adverse event. This relative rate methodology has been well described previously in the pharmacovigilance literature. Results: A total of 23,789 unique BoNT-A cases were included across a wide array of cosmetic and therapeutic indications. Relative incidence of decreased therapeutic effect for patients on >1 year of treatment versus <1 year was signiﬁcantly higher for onabotulinumtoxinA [14.4% (609/4219) vs 7.6% (753/9909); p>0.001] and abobotulinumtoxinA [9.9% (59/597) vs 3.3% (38/1144); p<0.001]. This phenomenon was not observed with incobotulinumtoxinA [0.0% (0/34) vs 3.7% (18/485); p=0.62]. Conclusions: Causal relationships cannot be established from pharmacovigilance analyses. However, a clear safety signal was detected in this analysis of one of the largest BoNT-A safety data sets ever researched. Long term differences in decreased BoNT-A therapeutic effect by agent warrants further study, including whether even lower dose indications are not immune from this phenomenon over time. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S105 ABSTRACTS 120 Botulinum Toxin Type A Overdoses and Associations with Medication Errors Rashid Kazerooni (San Diego, CA, United States) Objective: The aim of this analysis was to update a previous publication which assessed the respective botulinum toxin A (BoNT-A) agents for associations with overdose as well as explore any association with medication errors. Background: A previously published manuscript showed a 73-fold higher relative odds ratio for overdose with abobotulinumtoxinA versus other BoNT-A agents. This analysis has two additional years of data included that has been published since the original analysis. Methods: The US Food and Drug Administration adverse event reporting system (FAERS) was utilized. The analysis was conducted on data between March 2014 and June 2019. BoNT-A cases were included when they were considered the “Primary Suspect” drug. Overdose was deﬁned as incidence of ‘Overdose’ being reported as an adverse event. Primary outcome was incidence of ‘Overdose’ compared within the respective agents. Rates of overdose having a concomitant medication error were also reported between agents. Medication error was deﬁned as having a concomitant report of ’Product preparation error’ and/or ‘Wrong technique in product usage process’. These two terms are classiﬁed as “Medication errors” within the Medical Dictionary for Regulatory Activities system. Results: A total of 6,432,493 unique adverse events were reported during the study period for all drugs in the FAERS database. Of which, 23,789 were BoNT-A cases. The rate of adverse events involving overdose for abobotulinumtoxinA (14.2%; 342/2,415) was signiﬁcantly higher than both onabotulinumtoxinA (0.4%; 78/20,113; p < 0.0001) and incobotulinumtoxinA (<0.1%; 1/1,261; p < 0.0001). Additionally, a large percentage of abobotulinumtoxinA overdoses (37.1%; 127/342) had concomitant report of a medication error. This phenomenon was not seen with onabotulinumtoxinA (1.3%; 1/78) or incobotulinumtoxinA (0.0%; 0/1) overdoses. Conclusions: The analysis validates earlier ﬁndings which showed abobotulinumtoxinA adverse events were signiﬁcantly associated with overdose versus other BoNT-A agents. Furthermore, the present analysis showed abobotulinumtoxinA overdoses were often associated with medication errors, unlike the other agents. The relative rate methodology utilized is a commonly accepted metric for analyzing pharmacovigilance databases and well described in the literature. Overdoses and medication errors can be difﬁcult to research, particularly for in-clinic administered drugs. The FAERS database cannot establish causal relationships; however the analysis did identify a safety signal that future studies should venture to conﬁrm in new and novel ways. 121 Dopaminergic Neuroprotection Induced by Long-Term Intake of Yerba Mate: Behavioral and Histological Evidence in Hemiparkinsonian Mice Irene Taravini (Gualeguaychu, Argentina), Gimena Gomez (Buenos Aires, Argentina), Liliana Tribbia (Gualeguaychú, Argentina), Andrea Cura (Gualeguaychu, Argentina), Roy Rivero (Gualeguaychu, Argentina), Alejandra Bernardi (Buenos Aires, Argentina), Juan Ferrario (Buenos Aires, Argentina), Bertha Baldi Coronel (Gualeguaychu, Argentina), Oscar Gershanik (Buenos Aires, Argentina), Emilia Gatto (Buenos Aires, Argentina) Objective: We propose to characterize a yerba mate (YM) extract and evaluate if the consumption of YM provides a beneﬁt at behavioral level and favors the survival of dopaminergic neurons in hemiparkinsonian mice. Background: The consumption of mate infusion (made from Ilex paraguariensis leaves) has been inversely associated with the development of Parkinson’s disease (PD) in a case-control study in Argentina and it was shown to provide numerous health beneﬁts. Methods: The YM extract was obtained by “cebada simulada”. The concentration of its main bioactive compounds (caffeine, theobromine, chlorogenic acid and rutin) and their stability was determined by HPLC. Mice from experiment 1 received water (control) or YM (MATE) for 2 months before being lesioned by a 6-OHDA injection S106 into the striatum. Animals from experiment 2 received water (control) or YM infusion diluted by one half in water (dilMATE) for 4 months before the injury. After lesion, mice continued 1 more month with each treatment. During the treatment, locomotor activity was evaluated in an open box, and after sacriﬁce, tyrosine hydroxylase (TH) immunohistochemistry was performed to evaluate the degree of dopaminergic denervation. Results: Immunohistochemical evaluation of TH indicated that treatment with MATE for 2 months before the injection of 6-OHDA did not have any effect on the percentage of remaining dopaminergic terminals in the striatum. On the other hand, the area of remaining THimmunoreactive terminals was 12.75 % higher in the group treated with dilMATE for 4 months prior to the lesion compared to the animals treated with water. The behavioral evaluation showed no signiﬁcant difference between the control and dilMATE groups, which may be due to the scares number of animals. Conclusions: Our results suggest that the concentration of bioactive compounds on the diluted YM could not be enough to induce hyperlocomotion. However, long term administration of this diluted extract was enough to induce a moderate, but signiﬁcant, neuroprotection against 6-OHDA toxicity. Therapy in Movement Disorders 112 Withdrawn by Author 113 Virtual Reality Telerehabilitation for Patients with Spinocerebellar Ataxia: Case Study Marcos Maldonado (Santiago, Chile) Objective: To know the effect on the balance and stability of the gait of a patient with long-standing spinocerebellar ataxia, after 4 weeks of intensive rehabilitation through telerehabilitation with non-immersive virtual reality. Background: The evidence indicates that training based on repetitive tasks generates changes in motor balance learning. A virtual reality telerehabilitation program can promote self-management and provide objectivity in the rehabilitation measurement processes. Methods: Case Report. Patient diagnosed with spinocerebellar degeneration, 49 years old. It presents alterations of the balance and gait instability, at the beginning it requires total support for the displacement. He lives 68 miles from the clinical center, presenting logistical problems for easy access.It is submitted to the Telerehabilitation protocol of the Physical Medicine and Rehabilitation Service of the Clinica Alemana of Santiago. An initial face-to-face evaluation is carried out and the balance is measured according to the MiniBestest. Training is scheduled considering your current balance condition (Cluster 2). Rehametrics rehabilitation software is used. Do 4 weeks of training. Once a week, a routine is supervised by telephone with a therapist. At the end of the 4 weeks of training, it is evaluated face to face again.Each exercise is programmed at a standard difﬁculty level, so the increase in intensity, frequency and time depend on the patient’s performance. Results: The results according to the Initial MiniBestest (04.04.19) were the following: - Anticipatory control: 3; reactive control: 2; sensory orientation: 1; gait stability: 5The results according to the Final MiniBestest (05.22.19) were the following: - Anticipatory control: 3; reactive control: 2; sensory orientation: 5; gait stability: 5The Rehametrics software provided the following information: 17 days of training. 56% adhesion level. Difﬁculty: 37% Yield: 90%. -Initial static balance (04.04.19). Level 3/90. Average response time: 3.3 seconds. -Final static balance (05.22.19): Level: 60/90. Average response time: 2.0 seconds. Sit to stand: Level: 4/12. Average response time: 4.7 seconds. Sit to stand: Level: 12/25. Average response time: 1.6 seconds. Conclusions: It is possible to appreciate a minimum detectable change of 4 points in the MiniBestest, mainly in the sensory orientation. This could be related to the proper selection of exercises for their functional level and the amount of effective work time, since the patient MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS practices his routine only with supervision, demanding his balance systems in a sustained way.As for the results obtained from the software used with telerehabilitation, there is a decrease in the response times of exercises that require independent static equilibrium and move from sit to stand position. In addition, there is an increase in the level of difﬁculty as you repeat the scheduled activities daily. Studies of larger sample size are required. References: 1 .Matthis Synofzik and Winfried Llg. Motor Training in Degeneratice Spinocerebellar Disease: Ataxia- Speciﬁc Improvements by Intensive Physiotherapy and Exergames.BioMed Research International. Dx.doi.org/10.1155/2014/583507.2014. 2. Ashwini K. Rao, Elan D. Louis. Ataxic gait in essential tremor: A disease-associated feature. Tremor and other hyperkinetic movements. Doi: 10.7916/d8jq8t52.2019. 114 Nilotinib Alters MicroRNAs that Regulate Speciﬁc Autophagy and Ubiquitination Genes in the CSF of Individuals with Parkinson’s Disease Alan Fowler (Washington, United States), Yasar Torres-Yaghi (Washington, DC, United States), Fernando Pagan (McLean, VA, United States), Michaeline Hebron (Wsahington, DC, United States), Barbara Wilmarth (Washington, DC, United States), Abigail Lawler (Washington, DC, United States), Elizabeth Mundel (Washington, DC, United States), Nadia Yusuf (Washington, DC, United States), Nathan Starr (Washington, United States), M. Waseem Anjum (Germantown, MD, United States), Shahnaz Miri (Washington, DC, United States), Steven Nakano (Silver Spring, MD, United States), Amelia Carwin (Arlington, VA, United States), Wangke Shi (Washington, United States), Sanjana Mulki (Washington, United States), Tarick Kurd-Misto (Washington, DC, United States), Sara Matar (Washington, United States), Xiaoguang Liu (Washington, DC, United States), Jaeil Ahn (Washington, United States), Charbel Moussa (Washington, DC, United States) Objective: We evaluated the effects of nilotinib on microRNAs (miRNAs) in the cerebrospinal ﬂuid (CSF) of Parkinson’s disease patients. Background: MicroRNAs (miRNAs) are small, noncoding regulatory RNAs that may potentially regulate gene expression at the –post transcriptional level. Our preclinical data demonstrated that the principal effects of nilotinib is clearance of misfolded proteins, including hyperphosphorylated tau and oligomeric alpha-Synuclein via autophagy. Nilotinib increases the levels of key autophagy proteins like parkin, beclin-1, light chain protein (LC3-I/II), P62, and autophagy like proteins (ATGs) in animal models of neurodegeneration. Methods: CSF was collected as part of an open label phase I (12 participants, 6 months treatment)) and phase II randomized, double-blind, placebo-controlled study with 75 participants randomized 1:1:1 into placebo, 150 mg or 300 mg nilotinib groups. Nilotinib versus placebo was taken orally once daily for 12 months. Results: Next generation whole-genome sequencing of microRNAs in the CSF demonstrated that nilotinib signiﬁcantly increases (multiple fold with False Discovery Rate <0.001) microRNAs that speciﬁcally regulate the expression of autophagy and ubiquitination genes in individuals with PD. No effects were detected on genes that regulate tyrosine kinases, which are the likely protein targets of nilotinib. Conclusions: These data provide robust evidence that nilotinib effects on misfolded protein clearance is via autophagy. CSF microRNA sequencing is a valid biomarker of nilotinib effects on autophagy in a deﬁnitive phase III study to investigate nilotinib in PD and other neurodegenerative diseases. 115 Accompanying People with Multiple System Atrophy at the End of Their Lives: A Case Report Sara Tapia Saavedra (Santiago, Chile) Objective: To describe some of the challenges that become accompanying people with multiple system atrophy (MSA) at the end of their lives. Background: As therapists, we must think about the quality of life and autonomy of our patients, when we face people in the ﬁnal stage of their life. Analyzing our practice and the necessary considerations before making any medical decision. Methods: Descriptive study by participant observation Case report. Results: Patient CS, 72 years old with Multiple System Atrophy for 12 years. She has received speech therapy for 5 years, which has mainly focused on her autonomous communication, feeding and emerging needs (1,2,3). CS is currently in an advanced stage of the disease. For a year now she depends on a communication board and gastrostomy (conserving oral intake just for tasting pleasure). As her therapist I had to make my practice more ﬂexible in order to adapt my interventions to CS´s needs and desires, balancing my professional training and medical structure. It´s not easy knowing for sure our place towards making tough decisions. We had to face ethical dilemmas, having to make choices between respecting CS´s will and autonomy or obeying the technological imperatives (doing whatever necessary to increase life time), this along with the family´s fears and guilt(4,5). Conclusions: In degenerative health situations, the discussion focuses on reducing pain or ways of prolonging life, leaving aside the autonomy and sometimes the dignity of people, their right as citizens to decide on their life/death. It is difﬁcult to face death, and to know what is the limit of our role as therapists, make the decision? Educate?. The therapeutic obstinacy limits the autonomy of patients, for this reason, the importance of our role as educators since the decision-making by the user and his family must be informed (6,7). That is why talking about our function as therapists at the end of life and the ways of accompaniment are important and allow us to reﬂect on our practice (8) References: 1). Do, H. J., Seo, H. G., Lee, H. H., Oh, B. M., Kim, Y., Kim, A., … Han, T. R. (2019). Progression of Oropharyngeal Dysphagia in Patients with Multiple System Atrophy. Dysphagia, (0123456789). https://doi.org/10.1007/s00455-019-09990-z. 2). Vesey, S., Leslie, P., & Exley, C. (2008). A pilot study exploring the factors that inﬂuence the decision to have PEG feeding in patients with progressive conditions. Dysphagia, 23(3), 310–316. https://doi.org/10.1007/s00455-008-9149-0, 3). Causes of death in Chinese patients with multiple system atrophy. Aging and Disease, 9(1), 102–108. https://doi.org/10.14336/AD.2017.0711 5). Hortelano Martínez, J. E., Azulay Tapiero, A., Castillo Blasco, M., Hortelano Martínez Hospital Moliner Portacoeli, E., & Valencia, S. (2002). Decisiones éticoclínicas sobre la alimentación e hidratación artiﬁcial mediante sonda en la enfermedad terminal. Nutr. Hosp, (6), 279–283. 5) Quill, T. E., & Brody, H. (1996). Physician Recommendations and Patient Autonomy: Finding a Balance between Physician Power and Patient Choice. Annals of Internal Medicine, 125(9), 763–769. https://doi.org/10.7326/0003-4819-1259-199611010-00010. 6). Sogi, C., Zavala, S., Cárdenas, M., & Delgado, A. (2012). Autonimia del paciente y toma de decisiones en salud. Anales de La Facultad de Medicina, 73(1), 19–26. Retrieved from http://www.scielo. org.pe/scielo.php?script=sci_arttext&pid=S102555832012000100004%0A http://sisbib.unmsm.edu.pe/BVRevistas/anales/v73n1/pdf/a04v73n1.pdf. 7). Bearden, D. M., Childers, T., Howell, S., & Palmore, J. (2011). Lessons from the silence. Journal of Palliative Medicine, 14(1), 105–106. https://doi.org/10.1089/jpm.2010.0332. 8). Clarke, G., Galbraith, S., Woodward, J., Holland, A., & Barclay, S. (2014). Should they have a percutaneous endoscopic gastrostomy? The importance of assessing decisionmaking capacity and the central role of a multidisciplinary team. Clinical Medicine, Journal of the Royal College of Physicians of London, 14(3), 245–249. https://doi.org/10.7861/clinmedicine.14-3-245 116 Development of Acute Inﬂammatory Demyelinating Polyneuropathy after Initiation of Levodopa-Carbidopa Intestinal Gel in 48 yo Female with Parkinson’s Disease Kendall Hodges (United States), Duarte Machado (Hamden, CT, United States) Objective: To report a rare case of a patient who developed acute inﬂammatory demyelinating polyneuropathy (AIDP) in association with use of levodopa-carbidopa intestinal gel (LCIG). Background: Neuropathy in Parkinson’s disease (PD) is estimated to be present in 16% of patients[1]. Neuropathy is thought be related to poor absorption and low levels of vitamin B12 and vitamin B6 and elevated levels of homocysteine due to levodopa[2]. Methods: Our patient is a 48 yo female with PD who underwent percutaneous endoscopic gastrostomy with jejunal extension (PEG-J) MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S107 ABSTRACTS procedure for administration of LCIG. Four months into LCIG therapy, over 5 days, she developed weakness of bilateral lower extremities, the inability to ambulate without assistance, and dysesthesia. These symptoms were preceded by diarrheal illness. One month prior to development of weakness, she had serum studies that revealed vitamin B12 at 360pg/mL and homocysteine at 46.8umol/L. She was instructed to replete with cyanocobalamin 1000mcg/day. She had diminished pinprick and vibratory sense distally, was unable to raise legs against resistance and had absent patellar and Achilles reﬂexes. She was admitted to inpatient service. Lumbar puncture revealed elevated protein in CSF at 60.5 mg/dL. Serum studies revealed elevated ESR at 41 mm/hr, Ganglioside Asialo GM 1 Ab IgG elevated at 1:200, and elevated Campylobacter jejuni Ab at 1.32. Inpatient Nerve Conduction Study/Electromyography (NCS/EMG) revealed diffuse sensorimotor neuropathy with early features of acquired demyelination. Results: She started intravenous immunoglobulin (IVIG) treatment during her hospitalization and continues to receive IVIG. She underwent subsequent outpatient NCS/EMG study that again revealed evidence of a segmentally demyelinating sensorimotor polyneuropathy consistent with AIDP. Conclusions: This case serves as one of just a few examples of the correlation between LCIG and development of AIDP [3], albeit unique from already documented cases as this patient had antecedent diarrhea with positive campylobacter Ab. Further questions are whether the introduction of the PEG-J put her at higher risk for campylobacter jejuni infection, and what role levodopa itself plays in the development of neuropathy. References: [1] Zis, P., Grünewald, R.A., Chaudhuri, R.K., and M. Hadjivassiliou. 2017. Peripheral neuropathy in idiopathic Parkinson’s disease: a systematic review. J of Neurological Sciences. (378): 204-209. [2] Müller T., van Laar T., Cornblath D., Odin, P., Klostermann, F., Grandas, F. et al. 2013. Peripheral neuropathy in Parkinson’s disease: levodopa exposure and implications for duodenal delivery. Parkinsonism related disorders. (19): 501-507.[3] Galazky, I., Schoof, J., Stallforth, S., Kupsch, A., Heinze H.J., and C. Kluge. 2014. Guillain-Barre/CIDP-like neuroathy in two parinsonian patients following intestinal levodopa/carbidopa treatment. Parkinsonism Relat. Disord. 20 (1): 125-127. Tics/Stereotypies 189 Inhibitory Control Deﬁcits in Children with Tourette Syndrome Revealed by Object-Hit-and-Avoid Task Nicholas Cothros (Calgary, AB, Canada), Alex Medina (Calgary, AB, Canada), Davide Martino (Calgary, AB, Canada), Sean Dukelow (Calgary, AB, Canada), Rachel Hawe (Calgary, AB, Canada), Adam Kirton (Calgary, AB, Canada), Elaheh Nosratmirshekarlou (Calgary, AB, Canada), Tamara Pringsheim (Calgary, AB, Canada) Objective: To explore bimanual visuomotor performance in children with Tourette syndrome (TS) using a robot-based task that involves rapid motor selection. Background: TS is a childhood onset neuropsychiatric disorder characterized by tics - brief intermittent movements or sounds that may be suppressible. This may relate to inhibitory control, in turn reﬂecting sensorimotor integration and motor output selection. Methods: Sixty-four children diagnosed with TS (mean age 12.4 years; 7.5-18.5) were evaluated using a validated robotic bimanual exoskeleton protocol (Kinarm) in an object-hit-and-avoid task, in which the participants viewed target objects and distractor objects moving across a screen simultaneously, with instructions to hit only the target objects and avoid distractor objects. The Kinarm measured several kinematic and performance variables. Performance was compared to a database of 146 healthy control children (mean age 13 years; 6.1-19.9). The primary outcome was performance on task variables deemed consistent with rapid motor selection and inhibitory control (Bourke et al., 2016). Results: Measures of rapid motor selection and inhibition yielded statistically signiﬁcant differences between participants with TS and controls (ANCOVA; age as covariate), including distractor proportion (distractors hit as a percentage of targets), with adjusted means of 16.2% (SE .64) for S108 TS and 11.3% (.43) for controls; and object processing (number of targets hit plus distractors missed per second), with adjusted means of 1.85 (.028) for TS and 2.0 (.019) for controls. There were no signiﬁcant differences between children with TS and comorbid attention deﬁcit hyperactivity disorder (ADHD) and children with TS without comorbid ADHD. There were no reliable differences in other motor and kinematic variables unrelated to inhibitory control. Conclusions: In the bimanual object-hit-and-avoid task, children with TS showed worse performance on task variables related to rapid motor selection and inhibitory control. This appears to be independent of ADHD, as patients with and without ADHD performed similarly. The ﬁndings lend support to the hypothesis that TS is associated with poorer performance in rapid motor selection. This may explain apparent inconsistencies in the literature regarding impaired inhibition in TS, as rapid motor selection in bimanual tasks is not a feature of all inhibitory control tasks. References: Bourke, T.C. et al. (2016). A robot-based behavioural task to quantify impairments in rapid motor decisions and actions after stroke. Journal of Neuroengineering and Rehabilitation, 13(1): 91. 190 The Clinical Comorbidity of Tic Hyperkinesis with Impulsive Disorders Sabina Munasipova (Kazan, Russia), Zuleykha Zalyalova (Moscow, Russia) Objective: To establish the comorbidity of neuropsychiatric disorders in patients with tic hyperkinesis. Background: In terms of frequency, tics occupy one of the leading places among childhood neurological diseases. Tourette’s syndrome (TS), the most common cause of tics, manifests itself in a wide range of motor and behavioral disorders, such as obsessive-compulsive disorder (OCD) and attention deﬁcit hyperactivity disorder (ADHD). These behavioral disorders more than tics disrupt vital activity. If these disorders are not corrected, they can lead to signiﬁcant social and emotional maladaptation. Methods: The study included a total of 111 patients with tic hyperkinesis of various ages and sex who were outpatiently observed at the Clinical and Diagnostic Center of Movement Disorders and Botulinum Therapy of the Republic of Tatarstan from 2011 to 2014. Clinical and neurological research was carried out according to standard methods. Tic severity was assessed using the Yale Global Tic Severity Scale, OCD Yale-Brown Obsessive-Compulsive Scale, ADHD - SNAP-IV Questionnaire. The data obtained during the study were subjected to statistical processing on a personal computer Intel Core i5 - 450M processor using the Microsoft Ofﬁce Excel-2007 software package, including the use of the built-in statistical processing functions. Results: Based on clinical and medical history, taking into account reversibility, persons with TS prevailed (33%); patients with chronic simple motor tics (31%), with chronic simple and complex motor or vocal tics (17%), with chronic simple motor and vocal tics (15%), with transitional tics (4%) were less common. Tic severity signiﬁcantly prevailed in patients with Tourette’s syndrome. Regardless of the phenomenology of tics, a family history was traced in all subgroups. Obsessive-compulsive disorders were found in 54.1% of patients with tics. The direct dependence of the severity of chronic simple and complex motor or vocal tics (r = 0.61, p=0.01) and Tourette’s syndrome (r = 0.54, p=0.01) on the severity of OCD was determined. Tic disorders was associated with ADHD with a predominance of hyperactivity in 81% of cases. A reliable direct dependence of the severity of tics on the severity of ADHD was revealed in terms of inattention, hyperactivity, and impulsivity. Conclusions: TS is a complex neuropsychiatric disorder with tics (both motor and phonic), and a variety of neuropsychiatric comorbidities more commonly includes ADHD, OCD, impulse control disorder. The pathophysiology relating to comorbidities in TS is unclear, however thought to be at least in part related to dysfunction in the corticostriatothalamo-cortical circuit. Investigation and treatment of the comorbid conditions are an essential part of the treatment plan for all patients with TS. Practitioners should also be aware about the rare but severe neurological complications in these patients and consider treating tics aggressively. References: 1. Prevalence of tic disorders among schoolchildren in Warsaw, Poland / P. Stefanoff, T. Wolanczyk, A.S. Gawrys, E. Stefanoff, MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS A. Kaminska, M. Lojewska-Bajbus, et al // Eur Child Adolesc Psych. – 2008. - Vol.17(3). – ?.171-1782. Munasipova S.E. Tic hyperkinesis: clinical, laboratory-neurovisual heterogeneity. Abstract. dis. Cand. honey. sciences. - Moscow, 2015 .-- 24 p.3. Ashutosh Kumar, William Trescher, and Debra Byler Tourette Syndrome and Comorbid Neuropsychiatric Conditions Curr Dev Disord Rep. 2016; 3(4): 217–221 Tremor 191 Essential Tremor as a Waste Basket Diagnosis Christian Amlang (New Haven, CT, United States), Daniel Trujillo Diaz (New Haven, CT, United States), Elan Louis (New York, NY, United States) Objective: We aimed to (1) determine how frequently essential tremor (ET) diagnoses are misapplied, (2) determine which other neurological disorders are misclassiﬁed as “ET”, and (3) examine the clinical features that aligned with diagnostic misclassiﬁcation. Background: While ET is one of the most common movement disorders, its diagnosis remains a clinical one and diagnostic errors are quite common, with frequent misclassiﬁcation. Methods: 84 consecutive patients were included with the following criteria: (1) initial outpatient evaluation by one of the authors (E.D.L.) between January 2015 and July 2019 and (2) pre-evaluation diagnosis of ET. Data on an extensive number of clinical features were extracted from the electronic medical record. These related to the neurological history and examination. We ﬁrst compared patients with a post-evaluation diagnosis of ET to patients with an alternative post-evaluation diagnosis. We then compared patients with a post-evaluation diagnosis of ET to patients with a post-evaluation diagnosis of dystonic tremor. Results: Forty (47.6%) received a post-evaluation diagnosis of ET, 22 (26.2%) of dystonia and 22 (26.2%) of other diagnoses including Parkinson’s disease (PD) (5 [6.0%]). Factors associated with an alternative post-evaluation diagnosis other than ET were: pre-evaluation diagnosis made by a non-neurologist, shorter tremor duration, unilateral tremor, focal muscle hypertrophy, or presence of >1 cardinal feature of PD. Intention tremor and a single identiﬁable tremor axis on the handdrawn spiral test were more likely to be found in patients whose ET diagnosis was conﬁrmed. A post-evaluation diagnosis of dystonic tremor was associated with irregular tremor, abnormal limb postures, and several other features. Conclusions: Our study shows that diagnosing ET remains a challenge. More than one-half of the patients who were referred to our clinic with an intake diagnosis of ET were given an alternative post-evaluation diagnosis. While PD was reported to be the most frequently missed diagnosis in past study, dystonia was most commonly missed in our study. Possibly, PD is more accurately diagnosed nowadays due to greater societal awareness and availability of conﬁrmatory diagnostic imaging in unclear cases. Our data also provide additional evidence that certain clinical features can help to differentiate ET from other tremor disorders. prevalence among adults 18 years and older based on an explicit diagnosis of ET. We identiﬁed seven studies reporting the number of ET patients and the total population by speciﬁed non-overlapping age groups.4-10 Crude age-group speciﬁc prevalence values were calculated for each study and an age-group speciﬁc weighted average across studies was used for estimation. The weighted age-group speciﬁc prevalences were then applied to 2018 US population estimates for adults 18 and older. The lowest and highest estimates in each age group were used to generate an age-speciﬁc prevalence range. Results: An estimated 6.4 million (M) (4.6M – 7.6M) US adults aged 18 years and older have ET, based upon an overall prevalence of 2.6% (1.8% – 3.0%). The prevalence of ET is strongly correlated with increasing age, ranging from 0.8% among 18 to 30-year-old adults to 8.2% (6.2% – 10.9%) among adults 85 years and older. Conclusions: This analysis, which estimates that 6.4M adults 18 years and older in the US have ET, is similar, but not identical, to a prior estimate of ~7M across all age groups.3 Furthermore, we provide the ﬁrst estimates of prevalence within each decade of age. These data are of value in understanding the burden of this disease on a population level and in planning therapeutic and public health initiatives. Future studies should seek to understand the number of adults diagnosed and treated for ET, to assess the humanistic and economic burden for patients, their families, and society. References: Clark LN, Louis ED.Handb Clin Neurol. 2018 ; 147: 229–239. doi:10.1016/B978-0-444-63233-3.00015-4 Haubenberger D,Hallett M. N Engl J Med 2018; 378:1802-1810 doi: 10.1056/ NEJMcp1707928 Louis ED, Ottman R. How Many People in the USA Have Essential Tremor? Deriving a Population Estimate Based on Epidemiological Data (2014) https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4137360/ Sur H, Ilhan S, Erdogan H, Ozturk E, Tasdemir M, Boru UT. Prevalence of essential tremor: a door-to-door survey in Sile, Istanbul, Turkey. Parkinsonism Relat Disord 2009l;15:101–104. Dogu O, Sevim S, Camdeviren H, et al. Prevalence of essential tremor: door-to-door neurologic exams in Mersin Province, Turkey. Neurology 2003;61:1804–1806. Benito-Leon J, BermejoPareja F, Morales JM, Vega S, Molina JA. Prevalence of essential tremor in three elderly populations of central Spain. Mov Disord 2003;18:389–394. Louis ED, Thawani S, Andrews HF. Prevalence of tremor and essential tremor in a community-based, multi-ethnic study in northern Manhattan. Neuroepidemiology 2009;32:208–214. Bergareche A, De La Puente E, Lopez De Munain A, et al. Prevalence of essential tremor: a door-to-door survey in Bidasoa, Spain. Neuroepidemiology 2001;20:125–128. Louis ED, Factor-Litvak P. Screening for and Estimating the Prevalence of Essential Tremor: A Random-Digit Dialing-Based Study in the New York Metropolitan Area. Neuroepidemiology. 2016;46(1):51-6.doi: 10.1159/000442576. Epub 2015 Dec 17. Louis ED, Hafeman D, Parvez F, Alcalay RN, Islam T, Siddique AB, Patwary TI, Melkonian S, Argos M, Levy D, Ahsan H. Prevalence of essential tremor in Araihazar, Bangladesh: a population-based study. Neuroepidemiology. 2011;36(2):71-6. doi: 10.1159/000323389. Epub 2011 Jan 19. 193 192 How Many Adults in the US Have Essential Tremor? Using Data from Epidemiological Studies to Derive Age-Speciﬁc Estimates of Prevalence Stephen Crawford (Cambridge, MA, United States), Cathy Lally (Boston, MA, United States), Jennifer Petrillo (Cambridge, MA, United States), James Paskavitz (Cambridge, MA, United States), Elan Louis (New York, NY, United States) Objective: To evaluate published age-speciﬁc prevalence estimates for essential tremor (ET) to understand the overall burden of ET among adults 18 and older in the United States (US). Background: ET is one of the most common adult-onset movement disorders;1,2 there has only been one other attempt to derive the number affected in the US.3 This is the ﬁrst study to provide age-speciﬁc and overall estimates for the number of affected adults. Methods: A targeted literature review was conducted to identify population-based studies from 2001 to present that measured ET Task Speciﬁc Tremor Associated with Parkinson’s Disease: Case Series Vindhya Koneru (Houston, TX, United States), Vindhya Koneru (Houston, TX, United States), William Ondo (Houston, TX, United States) Objective: We present a series of patients with Parkinson’s disease (PD) conﬁrmed by DATscan, who previously demonstrated Task speciﬁc Tremor (TST). We discuss here the demographics and clinical characteristics including the age of onset of TST, time interval to PD, laterality of PD to assess for a true biologic association. Background: TST pathophysiology is unclear and clinically has features of both essential tremor (ET) and dystonia. PD can initially present with dystonia but TST preceding PD has been only rarely reported. The relationship between the two is not clear. Methods: We queried for patients with TST and PD from our database and chart review at the Methodist Neurological Institute Movement Disorder Clinic, and collected demographic, disease characteristics, and DATscan results. MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S109 ABSTRACTS Results: We identiﬁed 7 subjects, 5 male, mean age of TST onset was 55.114.3. Three patients had tremor with writing, 1 with holding a microphone, 1 with typing, 1 when applying mascara, and 1 had jaw tremor while drinking. PD was conﬁrmed by DATscan in the 6 subjects who were scanned. Three other subjects with TST who later developed rest tremor but without other PD signs all had normal DATscans and are not included. Mean age of PD onset was 66 8.7. The ﬁrst PD symptom was rest tremor in 6 patients and arm stiffness in one. The ﬁrst PD symptom started ipsilateral to arm TST in 5 and contralateral in 1. Family history was positive for ET in one patient. The TST responded dopaminergic treatment in only 2/7 cases. Conclusions: Task speciﬁc tremor can associate with PD. The majority of these PD cases presented ipsilaterally and with rest tremor, suggesting a pathophysiologic link. 194 Evaluation of Effectivity of Periphery Neuromodulation on the Device GRACE in Essential Tremor Pedro Chana-Cuevas (Santiago, Chile), Felipe Nagel (Santiago de Chile, Chile), Laura Botta (Santiago de Chile, Chile), Federico Jensen (Santiago de Chile, Chile), Cedric Little (Santiago de Chile, Chile), A Cariman (Santiago de Chile, Chile), W Nagel (Santiago de Chile, Chile) Objective: To evaluate: 1. The effectivity of periphery neuromodulation on the device GRACE in Essential tremor (ET). 2. The security of using neuromodulation. Background: ET pharmacologic treatment is still the ﬁrst-line therapy for ET patients, but a satisfactory tremor reduction is only reached in 50% of the patients. Another option is periphery electric neuromodulation which still is under study. Methods: A prospective, clinical trial, double-blind, randomized study was conducted in individuals with ET, with bilateral and symmetrical action tremor in both hands and forearms. Key exclusionary criteria were alterations in neurological exam and record or evidence of psychogenic tremor. The device Grace was used in the dominant hand of the patient and sent electrical stimulation to group of muscles located in the forearm, this device kept recording data in the whole process. Patients were examined with Brain tremor scale, ﬁnger-nose, pouring water, writing, during basal, stimulating and placebo. Characteristics derived from the measurements of linear and angular accelerations were obtained for the evaluation of tremor dissolution. Side effects were asked openly, and all patients signed an informed consent. Results: Six patients with ET (3 men and 3 women) participated in this study. Age media 40 16 yrs. Disease duration of 19 11 yrs. GRACE device (patent US 10,363,413). All patients were right-handed. As shown in table 1, kinetic tremor scale, spiral B and pouring water test, were statistical signiﬁcant (SS) (p<0.05) vs the basal, while only the S110 pouring water test had also SS vs the placebo, in the other hand postural tremor scale didn’t had SS at all. [table1] [table2] Conclusions: Even though the lack of patients examined in this study, the device GRACE had shown promising results in reducing tremor, so in a near future this study can be replicate in a larger scale, to conﬁrm that peripheric neuromodulation is a reasonable treatment for ET. References: Popovic Maneski, L., Jorgovanovic, N., Ilic, V., Došen, S., Keller, T., Popovic, M. B., & Popovic, D. B. (2011). Electrical stimulation for the suppression of pathological tremor. Medical & Biological Engineering & Computing, 49(10), 1187–1193. 195 Symptomatic Relief in Essential Tremor with Home Use of NonInvasive Neuromodulation Therapy Stuart Isaacson (Boca Raton, FL, United States), Elizabeth Peckham (Austin, TX, United States), Winona Tse (New York, NY, United States), Olga Waln (Houston, TX, United States), Christopher Way (Mountain View, CA, United States), Melita Petrossian (Santa Monica, CA, United States), Nabila Dahodwala (Philadelphia, PA, United States), Michael Soileau (Georgetown, TX, United States), Mark Lew (Los Angeles, CA, United States), Cameron Dietiker (San Francisco, CA, United States), Nijee Luthra (San Francisco, CA, United States), Rajesh Pahwa (Kansas City, KS, United States) Objective: This study evaluated the efﬁcacy and safety of repeated home use of an individualized, non-invasive neuromodulation therapy targeting the median and radial nerves for reducing hand tremor in essential tremor (ET) patients over three months. Background: Single-session studies have shown non-invasive neuromodulation therapy to be safe, well-tolerated, and effective for treatment of ET, but its beneﬁt as a longer-term treatment is unknown. Methods: This was a prospective, multicenter, open-label, single-arm study with 263 patients enrolled across 26 sites. Patients were ﬁtted with a wrist-worn therapeutic device with stimulation calibrated to their tremor, and were instructed to use the therapy twice daily for three months. Pre-speciﬁed co-primary endpoints were improvements on (1) clinician-rated Tremor Research Group Essential Tremor Rating Assessment Scale (TETRAS) and (2) patient-rated Bain & Findley Activities of Daily Living (ADL) dominant hand scores. Other endpoints included improvement in the tremor power detected by an accelerometer on the device, Clinical and Patient Global Impression scores (CGI-I, PGI-I), and Quality of Life in Essential Tremor (QUEST) survey. Results: 205 patients completed the study. The co-primary endpoints were met (p « 0.0001), with 62% (on TETRAS) and 68% (on ADL) of ‘severe’ or ‘moderate’ patients improving to ‘mild’ or ‘slight’. Similarly, clinicians (CGI-I) reported improvement in 68% of patients, and 60% (PGI-I) of patients reported improvement. QUEST also improved (p = 0.0013). Wrist-worn accelerometer recordings before and after 21,806 therapy sessions showed that 54% of patients experienced >= 50% improvement in tremor power. The therapy was effective for patients, regardless of concurrent ET medication usage. Device-related adverse events (e.g., wrist discomfort, skin irritation, pain) occurred in 18% of patients. No device-related serious adverse events were reported. Conclusions: This study demonstrated that non-invasive neuromodulation therapy used repeatedly at home over three months results in safe and effective hand tremor reduction in essential tremor patients. Despite the heterogeneity of ET presentation and the day-today symptomatic variability of the disorder, the therapeutic response was reproduced across multiple improvement measures, including clinicianrated TETRAS and CGI-I scores, patient-rated ADL, PGI-I, and quality of life scores, and objective accelerometer-measured tremor power improvements. The treatment efﬁcacy and favorable adverse event proﬁle suggest this therapy may be an attractive option for patients who are seeking symptomatic ET treatment with minimal side effects. References: 1. Lin PT, Ross EK, Chidester P, et al. Noninvasive neuromodulation in essential tremor demonstrates relief in a shamcontrolled pilot trial. Mov Disord John Wiley and Sons Inc.; Jul 1, 2018 p. 1182–1183. 2. Pahwa R, Dhall R, Ostrem J, et al. An Acute Randomized Controlled Trial of Noninvasive Peripheral Nerve Stimulation in Essential Tremor. Neuromodulation. Blackwell Publishing Inc.; Epub 2019. 3. Elble RJ. The Essential Tremor Rating Assessment Scale. J Neurol Neuromedicine. 2016;1:34–38. 4. Bain PG, Findley LJ, Atchison P, MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 ABSTRACTS et al. Assessing tremor severnty. J Neurol Neurosurg Psychiatry. BMJ Publishing Group; 1993;56:868–873. 5. Busner J, Targum SD. The Clinical Global Impressions Scale: Applying a Research Tool in Clinical Practice. 2007. 6. Ondo W, Hunter C, Vuong KD, Schwartz K, Jankovic J. Gabapentin for essential tremor: A multiple-dose, double-blind, placebo-controlled trial. Mov Disord. 2000;15:678–682. 7. Tröster AI, Pahwa R, Fields J a, Tanner CM, Lyons KE. Quality of life in Essential Tremor Questionnaire (QUEST): development and initial validation. Parkinsonism Relat Disord. 2005;11:367–373. 10.1016/j. parkreldis.2005.05.009. 196 Unilateral Standing Leg Tremor: Report of a Case with Excellent Response to Propranolol and Revision of Literature Gabriela Raina (Buenos Aires, Argentina), Maria Peralta (Buenos Aires, Argentina), Fatima Pantiu (Eldorado, Misiones, Argentina), Maria Bastianello (Buenos Aires, Argentina), Juan Paviolo (Eldorado, Misiones, Argentina) Objective: To describe a patient with unilateral SLT, without PD, with excellent response to propranolol. Background: Standing leg tremor (SLT) are exclusively present when the patients adopt the standing position. These tremors comprise primary orthostatic tremor (OT), OT plus and pseudo-OT. The etiology of OT is unknown. The existence of a central oscillator has been postulated, which could be located in the brain stem and/or cerebellum, although the motor, sensitive cortex and base ganglia could also intervene in its genesis. A few patients with unilateral SLT have been recently described, all of which developed Parkinson´s disease (PD). Methods: Description of a case with unilateral SLT and revision of literature. Results: A 49-year-old female patient presented a progressive right leg tremor, of 13 months of evolution. Tremor appeared a few seconds after adopting the standing position and disappeared when walking, sitting or lying down, without unsteadiness. She had no motor or non-motor symptoms of PD. She had no family history of movement disorders. Electromyographic recording showed a synchronous activity of 6 Hz in the antagonist muscles of the right lower limb (quadriceps and femoral biceps, and tibialis anterior and gastrocnemius), but not in the left lower limb when adopting the standing position. The tremor was not present during the rest. Laboratory tests had no abnormalities. MRI and 18-F-Dopa PET of the brain showed no alterations. Clonazepam was not effective. Tremor completely abated with Propranolol 40 mg bid, reappearing with its suspension and remitting again with re-introduction of propranolol. Conclusions: To the best of our knowledge this is the ﬁrst case of unilateral SLT, without PD, with complete clinical remission of tremor with propranolol. Only 7 cases of unilateral SLT have been described, all of whom developed PD months or years after the onset of tremor, generally with response to antiparkinsonians drugs. References: 1) Kang SY, Song SK, Kim JS, Sohn YH. Unilateral Standing Leg Tremor as the Initial Manifestation of Parkinson Disease. J Mov Disord. 2009;2:29-32 .2) Al-Hashel JY, Kamel WA, Damier P, Abdulsalam AJ. An unusual initial presentation of Parkinson’s disease: unilateral standing leg tremor. Acta Neurol Belg. 2018; May 5. 3) Benito-León J, Domingo-Santos A. Orthostatic Tremor: An Update on a Rare Entity. Tremor Other Hyperkinet Mov. 2016;22;6:411. 4) Thomas A, Bonanni L, Antonini A, Barone P, Onofrj M. DopaResponsive Pseudo-Orthostatic Tremor in Parkinsonism. Mov Disord 2007;22:1652-1656.5) Kim JS, Lee MC. Leg Tremor Mimicking Orthostatic Tremor as an Initial Manifestation of Parkinson’s Disease. Mov Disord 1993;8:397-398. Table. Unilateral leg standing tremor: Revision of Literature. AUTHOR CASE N 1 UNILATERAL SLT Side Frequency (Hz) right 6 Age at onset 61 PARKINSONIAN SIGNS At onset In follow up (years) No Yes (1) 2 left 6-7 60 Yes - 3 right 4-5 61 No Yes (2) 4 right 3-4 45 Yes - 5 left 16-17 47 No Yes (1) AlHashel et al. 6 left 14-16 51 No Yes (1,5) Thomas et al. 7 right Not assessed 34 No Yes (6) Kang et al. TREATMENT Medication Propranolol Clonazepam Benztropine Propranolol Clonazepam Levodopa Benztropine Clonazepam Benztropine Propranolol Clonazepam Levodopa Selegiline Good Ropinirole Benztropine Clonazepam Gabapentin levodopa Gabapentin Clonazepam Levodopa Entacapone ropirinol Response Good Poor Poor Poor Poor Poor Poor Good Good Good Good Good Good Good Good Good Poor Poor Good Poor Good Good Good Good MOVEMENT DISORDERS CLINICAL PRACTICE 2020; 7(S1): S13–S111. doi: 10.1002/mdc3.12905 S111

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