NEUROLOGICAL Pathology:

BRAIN TUMOURS

Common CNS Tumours:

- General Features:
o Adults – Most are Cerebral/Supratentorial
o Children – Most are Infratentorial (Cerebellum & Brainstem)
o NB: CNS tumours NEVER Metastasise to Outside the CNS.
o Aetiologies:
Typically Secondary (Ie. Mets Breast, Lung, GIT, Melanoma)
Some are Primary (Gliomas are the Most Common) – NOT from Neurons!
o Types of Primary CNS Tumours Only Covering 3 Types:
Adults (NB: Most are Supratentorial Cerebral):
1. Meningioma
o From the Arachnoid Layer
2. Gliomas (Astrocytoma [& Glioblastoma Multiforme], Oligodendroglioma)
Children (NB: Most are Infratentorial Cerebellum & Brainstem):
2. Gliomas (Specifically Pilocytic Astrocytomas)
3. Medulloblastoma (Germ Cells)

o Clinical Features:
Slow, Progressive
Crescendo, Chronic, Morning Headache
Local Damage Nerve & Tract deficits, Paralysis, Blindness, Anosmia, Seizures
Raised ICP Headache, vomiting, papilodema & bradycardia.
Irritation Seizures
o Clinical Features Depend on Tumour Location Focal Deficits:
Brain stem Compression Drowsiness, Obtundation
Frontal Lobe Personality, Memory, Executive, Intelligence
Temporal Lobe Speech, Language & Hearing
Motor Cortex Limb weakness
Cerebellum Balance/Stumbling
Occipital Vision, Eye Movements

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 Primary CNS Tumours:

Adult CNS Tumours:

- MENINGIOMAS:
Cell of Origin: - Meningothelial Cells (Cells of the Arachnoid Granulations)
Morphology Macro: - Well Demarcated
- Attached to the Dura
- Hard, Fibrous Tumour with Calcification
Clinical Features: - Very Common, But Clinically Benign
- Non-Invasive, but Compresses the Brain Symptoms (Headache)
- Chronic, Gradually Increasing Morning Headache (Years).
- Oestrogen-Responsive :. Worse in Pregnancy/Menstruation.
Treatment: - Surgical Removal
Prognosis: - Benign
- Good Survival with Surgery

- Adulthood Gliomas LOW-GRADE ASTROCYTOMA & GLIOBLASTOMA MULTIFORME:

o ASTROCYTOMAS:
Cell of Origin: - Astrocytes
- Low-Grade ASTROCYTOMA:
Morphology Macro: - Usually Cerebral (Supratentorial)
- Solid Tumour
Clinical Features: - Space-Occupying Syx – Chronic Worsening Morning Headache,
Vomiting, Altered Mental Status, Personality Change, ALOC.
- + Focal Neurology depending on Location in the Brain.
Treatment: - Surgical Resection of Tumour
Prognosis: - Benign
- Good – 90% 5yr Survival

- High-Grade Astrocytoma GLIOBLASTOMA MULTIFORME

Morphology Macro: - Usually Cerebral (Supratentorial)
- Solid Tumour – May see some Cystic Degeneration
- Grows so fast, it looks encapsulated, but it is not.
Clinical Features: - Morning Headache, Nausea/Vomiting, Seizures, Hemiparesis
- + Memory Loss & Personality Changes.
- + Focal Neurology (Language & Executive Fx)
Treatment: - Palliative: Surgery + Chemo/Radiotherapy
- + Anticonvulsants – To Seizures
- + Corticosteroids – To Peri-Tumoural Oedema ICP
- (NB: Total Surgical Resection Impossible due to diffuse Infiltration.)
Prognosis: - Malignant – (Fast-Growing & Infiltrative)
- Poor Prognosis - <1yr Survival Rate

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Childhood CNS Tumours:
- Childhood Glioma PILOCYTIC ASTROCYTOMAS:
Morphology Macro: - Usually Cerebellar (Infratentorial) – (Rather than Cerebral in Adults)
- Cystic Tumour full of Mucoid FLuid- (Rather than Solid in Adults).
- Well-Circumscribed
Clinical Features: - Gait Abnormality (Wide Gait), Uncoordination, & Nystagmus
- Nausea, Vomiting, Irritability
- Failure to Thrive - Anorexia
- (NB: Associated with Neurofibromatosis)
Treatment: - Surgical Resection
Prognosis: - Benign – (Low-Grade, Slow-Growing)
- Good: >90% 10yr Survival Rate.

- MEDULLOBLASTOMA:
Cell of Origin: - Neuroblast Cells (Ectoderm Cells of Neural Crest = Neuroectoderm)
Morphology Macro: - Cerebellar (Infratentorial)
- Usually Form in the 4th Ventricle
Clinical Features: - Initial: Hydrocephalus (& ICP) due to 4th Ventricle Obstruction
Listlessness, Morning Headache, Vomiting.
- Later: Cerebellar Stumbling Gait, Falls, Diplopia, Nystagmus
Treatment: - Maximal Surgical Excision + Radiation + Chemotherapy
Prognosis: - High-Grade, Malignant Tumour CSF Seeding (Unique to
Medulloblastom) & Infiltration through Meninges is Common.
- Poor: 70% 5yr Survival, 50% 20yr Survival

Other CNS Tumours:

- Acoustic Neuroma (AKA: Vestib lar Sch annoma :
Cell of Origin: - Schwann Cells
Morphology Macro: - Schwannoma of the Vestibular Nerve (Part of CN8).
- Usually in the Internal Auditory Canal
- May Cerebellopontine Angle CN 5,7,9 & 10 Compression
Morphology Micro: - Homogenous Tumour – Only Schwann Cells
- Tumour cells always stay on outside of Nerve, but may compress it
against a bony structure Damage.
Clinical Features: - Typically 50-60yrs
- Sensorineural Hearing Loss/Deafness, Tinnitus
- Vertigo, Ataxia, Nausea & Vomiting
- Cerebellopontine Syndrome:
o CN 5 (Trigeminal) Palsy: Ipsilateral Corneal Reflex,
Trigeminal Neuralgia, Sensation
o CN 6 (Abducens) Palsy: Diplopia, Ipsilat. Inward-Facing Eye
o CN 7 (Facial) Palsy: Ipsilateral Facial Weakness
o (CN 8 (V/C): Ipsilateral Deafness, Tinnitus, Vertigo)
o Ipsilateral Cerebellar Signs: Nystagmus, Ataxia
Malignant/Benign: - Benign, Slow-Growing
Treatment: - Surgical + Radiotheraly (NB: Risk to Facial Nerve)
- Conservative Monitoring if Elderly.
Prognosis: - 100% Survival with Treatment.
- But some morbidity.
-

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- PITUITARY ADENOMAS:
Cell of Origin: - Pituitary Gland Embryonic Tissue
Morphology Macro: - Partially Cystic with Solid Areas
Clinical Features: - Compression of Optic Chiasm: 1. Bitemporal Inferior
Quadrantanopsia 2. Bitemporal Hemianopsia
- Headaches
- + Any Pituitary Endocrine Failure Symptoms
Malignant/Benign: - Benign – but hard to Treat & Compressive Neurology
Treatment: - Surgical Resection Hormone Replacement
Prognosis: - Benign but often recurrence.
- Good Survival, but Morbidity.

- NEUROFIBROMATOSIS Type 1 & 2

Cell of Origin: - Neural Fibroblasts
Morphology Macro: - Encapsulated, Solid Nodular Tumours (Neurofibromas)
Morphology Micro: - Whorls of Fibroblasts Within Nerves
- Well-Differentiated
Clinical Features: - NF1 Familial & Sporadic
- NF2 Autosomal Dominant
- Associated with Acoustic Neuromas (Vestibular Schwannomas)
o Tinnitus, Vertigo, Hearing Loss
- Also Associated with Meningiomas & Juvenile Cataracts
Malignant/Benign: - Benign
Treatment: - Surgical Resection of Individual Lesions – But Recurrence is common
Prognosis: - Benign – But can Extreme Morbidity/Disfigurement

- CNS Lymphoma:
Cell of Origin: - B-Cell Non-Hodgkin Lymphomas
Morphology Macro: - Multiple areas of tumour
Clinical Features: - Caused by EBV + Immunocompromise (Ie. HIV)
- Symptoms: Headache, Seizures, Cranial Nerve Palsies, Mental
Status, Focal Neurology.
- + Constitutional B-Syx: Fever, Night Sweats, Weight Loss
Malignant/Benign: - Malignant
Treatment: - Chemo/Radiotherapy + Corticosteroids (Surgery is Impossible)
Prognosis: - Poor – Due to High Grade + Concomitant Immunosuppression
- Median survival = 10mths

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 Wk 11
Infectious Disease Notes
CNS Infections

The blood brain barrier restricts the entry of pathogens into the brain and meninges:
- NB: Haematogenous spread of organisms requires spread through at least 2 layers to infect the brain.

Mechanisms of Entry into the CNS:
• Haematogenous Spread (Bloodborne Invasion) into the CNS:
– Growing across:
§ Microbes can grow in the endothelial cells and then into the astrocytes or choroid plexus
– Passive:
§ Transported across in intracellular vacuoles
– Carried in infected cells:
§ Infected inflammatory cells can migrate into the brain and meninges, lyse and release
the organism or the organisms may pass from cell to cell
– (Eg. Infection of brain or meninges by enteric viruses e.g. polio):

• Invasion Via Peripheral Nerves:
o Rabies and other Lyssaviruses may invade Muscle Cells @ The Bite Site àMove up the Nerves to
the Dorsal Root Ganglia à Spinal Cord àBrain
o Herpesviruses may migrate up the nerves using normal retrograde transport mechanisms

NB: The Blood Brain Barrier in Pharmacokinetics:
• The BBB Blocks access of certain chemicals to the CNS.
• :. Antimicrobial Drugs MUST be able to cross the BBB in order to fight CNS Infection.

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Eg. Invasion of the CNS by Rabies – (Via Peripheral Nerves):
1) Myocytes are Infected @ the bite site
2) Growth up the Motor Nerves àDorsal Root Ganglia
3) Growth up the Spinal Cord Nerves àBrain
4) Growth down the Cranial Nerves to the Salivary Glands
5) (Hence, CNS Invasion of the Rabies Virus is REQUIRED for transmission because it has to get from the Bite
Site à Peripheral Nerves à CNS à Salivary Glands àNext Bite)

• Rabies – Post Exposure Prophylaxis:
o Administration of Human Rabies Immunoglobulin
§ Used when there is a high risk of infection but insufficient time for the body to develop
its own immune response.
o Vaccination:
§ Rabies Vaccine à Promotes Active Humoral Immunity of the Host.

• Bat lyssavirus - viral antigens in neurones - immunoperoxidase staining:

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Definitions:
- Viral Meningitis:
o Inflammation of the Meninges of the brain due to viral aetiology.
o (Eg. By Herpes Simplex Virus)
- Bacterial Meningitis:
o Inflammation of the Meninges of the Brain due to Bacterial Aetiology.
o (Typically: Nesseria Meningitidis, Streptococcus Pneumoniae, Haemophilus Influenzae)
- Encephalitis:
o Inflammation of the Brain
o (Typically due to Viruses – eg. Herpes Simplex)
- Meningoencephalitis:
o Inflammation of the Brain & the Meninges
- Myelitis:
o Inflammation of the Spinal Cord à Disrupts CNS functions liking the brain & limbs.
o (Eg. Poliovirus (Poliomyelitis))
- Encephalomyelitis:
o Inflammation of the Brain and Spinal Cord
o Typically Immune-mediated following a viral infection.
o (Eg. Acute Disseminated Encephalomyelitis – Following Influenza, enterovirus, measles, mumps,
rubella, varicella zoster, etc.)
- Brain Abscesses:
o Encapsulated Pus or Free-Pus in the Brain after an Acute Focal Purulent Infection.
§ (Focal Infections include: Otitis Media/Sinusitis)

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 MENINGITIS:

Presentation: Meningism:
- *Neck Stiffness
- *Photophobia
- *Headache
- (Fever/Malaise)

Meningitis - CSF Examination:
• Three Successive Samples are Taken:
o To Eliminate Contamination
o NB: By the 3rd Sample, there should be NO Contamination
o NB: RBC indicates contamination
o Sample 1: Used for Serology
§ Serology
§ or PCR
o Sample 2: Used for Biochemistry
§ Glucose
§ Protein
§ Antigen Agglutination
o Sample 3: Used for Bacteriology – Most Precious
§ Gram stain
§ Culture

CSF Changes During CNS Infection:
- Septic Meningitis (Bacterial – N.Meningitidis, H.Influenzae, S.Pneumoniae)
o ↑Cells (Mainly Neutrophils & other Polymorphs)
o ↑Protein (Exudate)
o ↓Glucose (Due to Bacterial Metabolism)
o Positive Culture & Gram Stain
- “Aseptic” Meningitis (Typically Viral/Fungal)
o ↑Cells (Mainly Lymphocytes)
o ↑Protein
o Normal Glucose

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Septic/Bacterial Meningitis:
• More Severe than Viral
• Less Common than Viral
• Pathogenesis:

• The 3 Common Bacterial Implicated in Bacterial Meningitis:
o #1. Neisseria Meningitis:
§ Gram Negative Diplococci
§ Usually in Stressed/Crowded
§ Severe toxin sequlae à Tissue damage
§ Vaccine only for Serotypes A & C (Not B – Which is the most common)
• (NB: Serotype B Capsule molecules mimic Neural Tissue à Vaccine Cross Reacts)
§ NB: Capsule Switching:
• By the time the immune system has mounted an Adaptive Immune Resposne, N.
Meningitidis Changes the Immunogenicity of its Capsule.
• à Immune System has to Start Again
• à N. Meningitidis Prevails.

(Diplococci = Nesseria Meningitidis)

o 2. Haemohpilus Influenza:
§ Gram Negative Cocco-bacilli
§ Usually in Children / Babies
§ Toxin productionàTissue damage
§ Vaccine Available (HIb Vaccine)

o 3. Streptococcus Pneumoniae:
§ Gram Positive Cocci
§ Predisposed Adults
§ Neonates

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 • Other Aetiologies:
o Neonatal Meningitis:
§ Escherichia coli
§ Group B Streptococci
§ (High Mortality Rates (35% of cases))
o Tuberculous Meningitis:
§ Mycobacterium tuberculosis:
• Acid fast bacilli (Stains with zheil Nielson stain)
• Patients Typically have a Focus of Infection Elsewhere
• :. Most of cases are associated with Miliary (disseminated) Tuberculosis

Features Suggestive of Aetiology
1. Rash - erythematous, petechial / purpuric.
a. Suggests meningococcus (rarely Pneumococcus or Haemophilus influenzae type b)
2. CSF rhinorrheoa or otorrhoea - basal skull fracture:
a. Pneumococcus, H. influenzae, Haemolytic Strep.
b. (CSF Rhinorrhoea refers to the drainage of Cerebrospinal Fluid through the nose. It is a sign of
Basal Skull Fracture.)
3. Prominence of seizures or focal signs early:
a. Consider Listeria monocytogenes, Herpes simplex.

Aetiology Suggested by Age Group:
neonate Group B Streptococcus
E. coli,
Salmonella
Listeria monocytogenes
child< 6 Neisseria meningitides (meningococcus)
Streptococcus pneumonia (pneumococcus)
Haemophilus influenzae B
child >6 N. meningitidis
S. pneumonia
Healthy Adult S. pneumoniae,
N. meningitides
Immunosupressed, debilitated, L. monocytogenes,
elderly Gram negative enteric
organisms e.g. E. coli

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NB: Papilloedema < 1%:
• = Swelling of the Optic Disc secondary to the ↑Intracranial Pressure during Meningitis.
o Usually Bilateral
o May develop over hours to weeks.
• How it Occurs?
o The subarachnoid space of the brain is continuous with the optic nerve sheath.
o :. as CSF Pressure Increases à Pressure is transmitted to the optic nerve àOptic Nerve Sheath
acts as a Tourniquet around the Axon.

Meningitis Management:
1. Early Antibiotic Therapy is Essential for Good Outcome!!!
a. Even if they are Pre-Diagnosis.
2. Always Do Blood Cultures!!
3. Antibiotics must be:
a. Effective Against Likely Pathogens
b. Able to cross an Inflamed Blood Brain Barrier
c. Given Parenterally and in high dose.
4. Corticosteroids (Dexamethasone) are given prior to antibiotics à↓CNS Inflammation:
a. àImproves Neurological Outcome in all cases of suspected bacterial meningitis.
5. Prophylactic Measures in close contacts:
a. Meningitis Prophylaxis: Rifampicin, Ceftriaxone or Ciprofloxacin:
1. Prophylaxis with the above antibiotics WILL NOT abort infection in those already infected.
2. RATHER, It aims to Eliminate Nasopharyngeal Carriage àPrevent subsequent transmission.
3. Offered to Household, child care and CLOSE CONTACTS.
4. No evidence for salivary spread.

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Brain Abscesses:
• Incapsulated or free-pus in the substance of the brain after an acute focal purulent infection is known as
a brain abscess.
o Sites of Focal Infection that could lead to brain abscesses:
§ Otitis Media
§ Sinusitis
§ Penetrating trauma
§ Haematogenous dissemination
o Given the Possible Sites of Entry, Which Organisms are Most Likely to be Involved?
§ Otitis Media – Strep Pneumoniae
§ Sinusitis – Strep Pneumoniae
§ Penetrating Trauma – Probably Staph Aureus
o Diagnosis:
§ Blood culture should be performed, but often is not diagnostic
§ CT or MRI are Essential for Diagnosis.
§ Lumbar Puncture is Contraindicated (Due to ↑ICP)
§ Inflammatory Markers WBC, CRP & ESR are raised.

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Fungal Meningitis:
• Mainly Cryptococcus Neoformans
• Typically in Immunosuppressed
• Can be treated with antifungal drugs

Viral Meningitis:
• Less Severe than Bacterial
• More Common than bacterial
• Presentation:
o Usually are milder disease than bacterial meningitis
o Headache, fever and and general illness but less neck stiffness
o Generally Complete Recovery
• Examination of CSF:
o The CSF is clear and free of bacteria
o CSF Contains Mainly Lymphocytes
• Viruses Implicated in Viral Meningitis:
o Herpes Simplex
§ Uncommon; may follow congenital infection with HSV2
o Mumps
§ A quite common complication
o Poliovirus, cocsackievirus, echovirus
§ Commonly seen especially due to echoviruses
o Enterovirus 71
§ May follow hand foot and mouth disease
o Japanese encephalitis
§ India, Southeast Asia, Japan
o Eastern and Western equine encephalitis
§ Eastern and Western USA
o HIV
§ May occur early after infection

(NB: “Perivascular Cuffing” by monocytes around the vessels)

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 ENCEPHALITIS:

Encephalitis – Infection of the Brain:
• Encephalitis Is Usually Caused By Viruses:
o **Herpes Simplex Virus
§ The infection progresses back to the temporal lobe of the brain
§ 70% mortality rate in untreated patients
§ Treatment with Acyclovir à ↓Mortality rate
§ (Including Varicella Zoster, & Cytomegalovirus)
§ VZV: Encephalitis generally occurs as a sequel to reactivation
§ CMV: Either during primary infection (in utero) or reactivation due to immunodeficiency
(HIV)
o Poliovirus:
o Rabies:
• Other causes of encephalitis can include:
o Parasites such as Toxoplasma gondii and Plasmodium falciparum
o Fungi such as Cryptococcus neoformans
o Bacteria such as Treponema palidum
• Pathogenesis:
o àCharacteristically there are signs of cerebral dysfunction:
§ Abnormal Behaviour
§ Seizures
§ Altered Consciousness
§ Nausea/Vomiting
o and fever
• Pathogenesis of Viral Encephalitis:

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Poliovirus:
• Epidemiology:
o Non Existent in Aus (A single case would be an epidemic)
• Prevention:
o Vaccination Available
§ Live Attenuated (Oral Polio Vaccine):
• Advantages:
o Easy Administration - Given Orally
o Cheap
o Induces intestinal local immunity
o More Robust Immune Response
• Disadvantage:
o Rarely causes paralysis (1 in 2.5million)
§ Inactivated Polio Vaccine (IPV):
• Advantages:
o Carries NO risk of Vaccine-Associated Polio Paralysis
o Very Robust Immune Response
• Disadvantage:
o Difficult Administration - Has to be injected
o Confers little Mucosal Immunity in the Intestinal Tract.
o 5 Times more expensive than OPV.
o (NB: 1 in 2.5Mil recipients develop paralysis)
• 3x Serological Types:
o PV1, PV2, PV3.
o Have little cross-reaction :. Vaccination must contain ALL 3 Serotypes for Full Immunity.
o (NB: Serotype 1 causes most of the problems – Ie. Paralysis)
• The major lesion results in a flaccid paralysis
• Transmission:
o **Faecal Oral
o Respiratory
• Pathogenesis:
o Poliovirus Acquired Faecal-Orally or Respiratory Route.
o Virus Replicates in Lymphoid Tissue in the Pharynx and Gut.
o Viraemia follows à Extension to the Nervous System
o à Lytic Infection of Neurons à Paralysis
§ Anterior Horns of Spinal Cord are Most Affected.
• Clinical features:
o The incubation period = 7 to 14 days
o A minor illness with malaise, fever and a sore throat may occur
o Paralysis may extend from a single muscle to virtually every skeletal muscle
o There may be involvement of respiratory muscles à Lifelong Assisted Ventilation

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Rabies Encephalitis:
• Organism:
o Rhabdovirus (A Bat Virus)
• Transmission:
o by the bite of an infected animal
o The virus is present in the saliva of the infected animal (Dogs, foxes and other wild species)

Flavivirus Encephalitis:
• Japanese Encephalitis (JEV) is the most common cause of this infection:
o A vaccine is available for this virus
o This virus is common throughout Asia
o *Of particular importance in North Queensland
• Other members of the encephalitic subgroup of the flaviviruses include:
o Kunjin
o Murray Valley encephalitis
o West Nile virus
o St Louis encephalitis

Togavirus Encephalitis:
• Some of the togaviruses in the Americas (Eastern and Western encephalitis) can produce encephalitis
• These viruses usually infect various animal species and occasionally infect humans
• Vaccines are available for the animal species
• No vaccines are available for humans

Acute Flaccid Paralysis:
• While acute flaccid paralysis is a key clinical feature of polio infection, this syndrome can be produced my
several other factors:
• These include other picornaviruses such as enterovirus 71
• This virus also produces the syndrome hand foot and mouth disease
• In 1998 an outbreak in Taiwan involving 300,000 children resulted in 56 deaths
• In 1999 an outbreak in Perth resulted in six cases of acute flaccid paralysis

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PARASITIC INFECTIONS OF THE BRAIN:
• Toxoplasma Gondii:
o Life cycle:
§ à Cysts in Brain (Contained by Macrophages & Helper T-Cells)

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Helminthic & Protozoal Organisms Capable Of Causing CNS Infections:
Helminth Reservoir Neurological Method of Treatment
symptoms diagnosis
Tapeworm Eating Cysts in Brain Detectino of Albendazole or
uncooked Pork à Convulsions specific praziquantel
antibody in +
serum or CSF Corticosteroids
Visual detection
of cysts by MRI
Roundworm Cats and dogs Cysts in Brain Serum can be Antihelmentic
à Convulsions tested for therapy (But
Retinal Antibodies by not in ocular)
Detachment à ELISA
Blindness
Protozoan
Malaria People + Convulsions RDT Antigen Artemesinin
Mosquitoes Coma Test
Toxoplasma Cats and mice Hydrocephalus PCR of blood Antibiotics
Gondii Inctracerebral samples Antimalarials
calcification Immunostaining Atovaquone à
Kills Cysts
Trypanosome Kissing bug Abnormal gait Microscopy of: Melarsoprol
Abnormal Blood Smears Eflornithine
speech CSF
Mental state Lymph Node
change Aspirates
Other abnormal
movement RDT Antibody
Sleep Detection test.
Disturbance

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PRION INFECTIONS OF BRAIN:
• Scrapie like agents referred to as prions can produce infections in a range of domestic animals and in
humans
• NO DNA or RNA!! (Important for Exams)
• The disease is produced when there is a conformational change in the proteins referred to as prion
protein C
• The PrPSC protein:
o l The two forms of the protein have different properties
§ l PrPC is anchored to the cell membrane by a glyco-phospho-inositol (GPI) anchor
§ l PrPSC accumulates in plaque deposits in the brain of affected individuals
o l The two proteins have the same posttranslational modifications and cannot be distinguished by
monoclonal antibodies
• How the prions damage the cells:
o Accumulation in Neurons à Death of Neurons

• Human prion diseases:
o There are four human diseases classified as TSEs (Transmissible Spongiform Encephalopathies):
§ (NB: All are Progressive and Fatal.)
§ Creutzfeldt-Jacob disease (CJD) (Most Common In Humans)
• Iatrogenic (Person-Person – Eg. Transplant)
• Inherited
• Sporadic
§ Gertsmann-Straussler syndrome (GSS)
§ Fatal familial insomnia (FFI)
§ Kuru confined to one tribe in New Guinea and related to cannibalism in the past

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OTHER INFECTIOUS DISEASES AFFECTING THE CNS:
• Toxins produced by bacterial infections can affect the CNS:
o Eg. Tetanus
§ Organism:
• Clostridium tetani
• Found in Soil & Faeces of Domestic Animals
• Like Anaerobic Conditions
§ Pathogenesis:
• à Toxin à Tetanic Spasms (Acts on the nerves)
• (Acts by binding to Ganglioside Receptors à Blocking Release of Inhibitory NTs
à Convulsive Contractions of Voluntary Muscles)
§ Prevention:
• Tetanus Vaccine
o Eg. Botulism
§ Organism:
• Clostridium Botulinum
§ Pathogenesis:
• à Toxin à Flaccid Paralysis
o Most commonly absorbed in the gut.
• (Blocks Acetylcholine Release from Peripheral Nerves à Paralysis)
§ Presentation:
• Weakness and paralysis
• Dysphagia
• Diplopia
• Vomiting
• Vertigo
• Respiratory muscle failure
§ Treatment:
• Antibodies (Antitoxins)
• Respiratory Support

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 MULTIPLE SCLEROSIS . . . CONT.

Clinical Pearl
❏ MS is a common cause of internuclear ophthalmoplegia.

Diagnosis
❏ evidence from history and examination of multiple lesions disseminated in both time and space
❏ slowing of evoked potentials (visual/auditory/somatosensory)
❏ CSF (oligoclonal Ig bands in 90%, increased IgG concentration, mild lymphocytosis and increased protein)
❏ MRI (plaques show as hyperintense lesions on T2 MRI in periventricular distribution)
Management
❏ patient education and counseling (disclosure, prognosis, future expectations, support groups,
psychosocial issues: divorce, depression, suicide not uncommon)
❏ acute treatment
• corticosteroids are the most commonly used treatment for acute attacks
• current recommendation is to treat disabling attacks with 500 to 1,000 mg of IV methylprednisolone
for 3-5 days with or without short oral tapering dose of prednisone
❏ symptomatic treatment
• symptomatic treatment for spasticity (baclofen), painful symptoms, bladder dysfunction (ditropan),
fatigue (amantadine), depression
• monitor closely for infection especially UTI
• physiotherapy, speech therapy, occupational therapy, nutrition, social work
❏ disease suppressing agents
• interferons, copolymers are being used to suppress disease activity
• mechanism uncertain
• disease suppressing medications are indicated for ambulating patients with frequent relapses
• ß-interferon (beta 1b, Betaseron) shown to decrease relapse rate, decreased rogression of disability
in patients with relapsing/remitting and progressive disease
• ß-interferon (beta 1a, Avonex) appears promising with similar efficacy to betaseron
• Copolymer also decreases relapse rate in relapsing remitting disease
and is currently under investigation for use in secondary progressive
• trials under way for chronic and primarily progressive

CNS INFECTIONS
❏ see Infectious Diseases Chapter
MENINGITIS
❏ inflammation of the meninges
Predisposing Factors
❏ systemic (especially respiratory) or parameningeal (otitis media, odontogenic, sinusitis) infections
❏ head trauma
❏ anatomical meningeal defects
❏ previous neurosurgical procedures
❏ cancer, alcoholism, and other immunodeficiency states
Etiology
❏ bacterial
• neonates: E. coli, Group B Streptococcus, Listeria monocytogenes
• infants and children: H. influenzae, S. pneumoniae, N. meningitidis
• adolescents and adults: S. pneumoniae, N. meningitidis
• elderly: S. pneumoniae, N. meningitidis, Gram negatives
• CSF leak: S. aureus, Gram negatives
• immunocompromised: Listeria monocytogenes
❏ viral (“aseptic”)
• Enteroviruses, H. influenzae, HIV, HSV, Adenovirus
❏ fungal
• cryptococcus
❏ other
• Treponema pallidum (meningeal neurosyphillis)
• Borrelia burgdorferi (Lyme disease)
• TB

N54 – Neurology MCCQE 2002 Review Notes

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 CNS INFECTIONS . . . CONT.
Signs and Symptoms
❏ neonates and children: fever, vomiting, lethargy, irritability, and poor feeding
❏ older children and adults: fever, headache, neck stiffness, confusion, nausea and vomiting, lethargy,
meningeal signs (i.e. Kernig’s, Brudzinski’s)
❏ other signs include altered level of consciousness, petechial rash
(septic microemboli), seizures, focal neurological signs (i.e. CN palsies)
Diagnosis
❏ CBC + differential
❏ lytes for SIADH
❏ X-rays may indicate primary infection site (CXR, sinuses, mastoid bone)
❏ CSF profile (see Table 20)
❏ Gram stain, culture
❏ PCR +/– serology (viral)
❏ do CT, EEG if focality
Treatment
❏ initial choice of antibiotics is empirical, based upon the patient’s age and predisposing factors
❏ therapy is adjusted as indicated when Gram stain, C&S results become available
❏ neonates: ampicillin + cefotaxime (better CSF penetration than gentamicin)
❏ infants and children: ampicillin + ceftriaxone/cefotaxime
❏ adolescents and adults: penicillin
❏ elderly: penicillin + ampicillin
❏ CSF leak: cloxacillin + gentamicin
❏ reportable to Public Health
Complications
❏ headache, seizures, cerebral edema, hydrocephalus, SIADH,
residual neurological deficit (especially CN VIII), death
Morbidity and Mortality
❏ S. pneumoniae: about 25%; N. meningitidis: 10%; H. influenzae: 5%
❏ worse prognosis with extremes of age; delays in diagnosis and treatment; complicating illness;
stupor or coma; seizures; focal neurological signs
Prevention
❏ regular childhood immunization against H. influenzae
❏ vaccinate against N. meningitidis if traveling to endemic meningitic areas
❏ prophylactic Rifampin for household and close contacts of H. influenzae and
N. meningitidis meningitis-affected patients

Table 20. CSF Profile for CNS Infections

Normal Bacterial Viral/Syphilis TB/Fungal Aseptic
Meningitis

Appearance Clear Normal/cloudy Normal/cloudy Cloudy

Glucose (mmol/L) 2.8-4.4 Decreased Normal Decreased Normal

Protein (g/L) 0.2-0.45 Increased Increased Increased Increased

Cell Count <6 Increased Increased Increased Increased

(cell #/mm3)

Predominant Cell Lymphocytes PMNs Lymphocytes Lymphocytes Lymphocytes

Pressure (mmHg) 100-200 Maybe N/A Increased N/A

< 20 cm H2O increased

Clinical Pearl
❏ For suspected tuberculosis (TB) meningitis, confirm with PCR, AFB stain, TB culture.

MCCQE 2002 Review Notes Neurology – N55

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 CNS INFECTIONS . . . CONT.
ENCEPHALITIS
Pathophysiology
❏ an acute inflammatory disease of the brain due to direct viral invasion or to hypersensitivity initiated by a
virus / foreign protein
❏ common portals of viral entry into host include respiratory (mumps, measles, influenza),
enteric (rabies, CMV, HIV), genitourinary tract (enteroviruses), and venereal spread (HSV, CMV, HIV)
❏ other viruses reach CNS via peripheral nerves (rabies, HSV)
Etiology
❏ viral (usual cause): HSV, mumps, measles, rabies, arbovirus, HIV, poliovirus, CMV, varicella zoster
❏ bacterial, mycobacterial and spirochetal: Mycoplasma pneumoniae, syphilis, Listeria, TB, typhoid fever
❏ fungal: cryptococcosis, histoplasmosis, candida, coccidiomycosis
❏ parasitic: toxoplasmosis, falciparum malaria, protozoal (cysticercosis)
❏ rickettsial: Rocky Mountain spotted fever
❏ unclassified: CJD (prion)
Signs and Symptoms
❏ acute febrile illness, malaise, chills, nausea, vomiting
❏ meningeal involvement: headache, stiff neck
❏ parenchymal disease: seizures, mental status changes, focal neurological signs
❏ increasing ICP if a significant brain volume is damaged by the infectious process
Diagnosis
❏ typically is based on clinical picture
❏ CSF can confirm inflammatory process
❏ CSF profile (cell count and differential, glucose, protein), cultures, stains may help provide specific diagnosis
or limit possibilities
❏ serologic studies are valuable in diagnosing encephalitis
❏ CT/MRI/EEG to define anatomical substrata affected may show focus
❏ brain tissue biopsy for culture, histological examination, ultra structural study, and immunocytochemistry
Treatment
❏ general supportive care plus measures directed against specific infecting agent
❏ monitor vital functions carefully (BP, HR, respirations)
❏ maintain nutritional status (hyperalimentation/gastroscopy feeds)
❏ reportable to Public Health

Clinical Pearl
❏ With meningitis, cerebral function remains normal.
❏ With encephalitis, patients often have altered mental status (speech, movement).

Herpes Simplex Encephalitis

❏ pathophysiology
• acute, necrotizing, asymmetrical hemorrhagic process with lymphocytic and plasma cell reaction, which
usually involves the medial temporal and inferior frontal lobes
• associated with HSV-1, but herpes encephalitis can also be caused by Varicella
❏ signs and symptoms
• typically sudden onset
• headache, stiff neck, vomiting, hemiparesis, and focal or generalized seizures
• note signs of temporal lobe (HSV target) dysfunction (olfactory hallucinations, behavioral disturbance,
complex partial seizures)
• usually rapidly progressive over several days and may result in coma or death
• common sequelae in surviving patients are memory and behaviour disturbances
(reflecting limbic involvement)
• can present as supratentorial mass lesion
• may also cause acute myelopathy
❏ diagnosis
• CT/MRI: medial temporal lobe necrosis
• EEG: early focal slowing, periodic discharges
• biopsy: when diagnosis uncertain
• PCR of CSF for HSV DNA: for rapid diagnosis
❏ treatment
• IV acyclovir if diagnosis is suspected

N56 – Neurology MCCQE 2002 Review Notes

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 CNS INFECTIONS . . . CONT.
Arbovirus Encephalitis
❏ epidemic, sporadic, virus type by location (Eastern Equine is the worst, summer worst)
❏ newly recognized in North America - West Nile virus
❏ severe mortality/morbidity > 50%
❏ signs and symptoms
• rapid onset drowsiness, stupor, or coma with convulsive seizure, headache, vomiting,
neck stiffness, high fever
• CN palsies, hemiplegia, and other focal neurological signs common
• patients who recover often have sequelae: mental deficiency, cranial nerve palsies, hemiplegia,
aphasia, and convulsions are common
❏ treatment
• entirely supportive in acute stage
INTRACRANIAL ABSCESS (see Neurosurgery Chapter) (see Colour Atlas NS8)
❏ etiology: focal infection leading to hematogenous, or local spread,
• sometimes idiopathic
❏ extension from ear
• Group A Streptococcus, S. pneumoniae, H. influenzae, anaerobic Streptococcus, Bacteroides sp., Enterobacteriaciae
• treatment: penicillin G + metronidazole + ceftriaxone/cefotaxime
❏ extension from paranasal sinuses
• anaerobes, Strep sp., S. pneumoniae, H. influenzae
• treatment: penicillin G + metronidazole
❏ post-surgery or trauma
• S. aureus, Enterobacteraciae
• treatment: cloxacillin + ceftriaxone/cefotaxime + rifampin
❏ spread from extracranial site
• site-specific organisms
❏ HIV-infected
• Toxoplasma gondii
• treatment: pyrimethamine + sulfadiazine or pyrimethamine + clindamycin
❏ chronic abscess
• M. tuberculosis, C. neoformans

NEUROLOGIC COMPLICATIONS OF SYSTEMIC DISEASE

METABOLIC DISEASES
Alcohol Intoxication
Seizures
❏ alcohol withdrawal seizures
• arise 12-48 hours after ingestion
• patient is tremulous
• cluster of 2-3 seizures
• normal or slow interictal EEG
• treatment: benzodiazepines
❏ seizure precipitated by alcohol
• intrinsic CNS lesion (e.g. subdural hematoma, meningitis)
• focal seizure, EEG has focal abnormality
• occurs during the time of intoxication
Delirium Tremens
❏ mortality rate 20% if left untreated
❏ occurs from 24 h – 7 days after reduction/cessation of drinking, lasts 3-5 days
❏ signs and symptoms
• tremulousness
• symptoms of delirium
• visual hallucinations
• autonomic hyperactivity - tachycardia, fever, sweating
❏ treatment
• high dose chlordiazepoxide, lorazepam or diazepam +/– haldol
• clonidine, atenolol for autonomic hyperactivity
• maintain fluid and electrolyte balance

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 NEUROLOGICAL Pathology:
CNS INFECTIONS

MENINGITIS:
- Aetiology:
o Bacterial/Septic Meningitis Nesseria meningitides, Haemophilus influenza, Group B Streptococci.
Adults = Neisseria meningitides (NB: Vaccine preventable Meningococcal A & C)
Children = Haemophilus influenza (Vaccine Preventable HIB Vaccine)
Neonates = Group B Streptococci (or E.coli)
o Viral/Aseptic Meningitis Herpes Simplex Virus, Enteroviruses (Echo/Coxsackie), Influenza
o Chronic Meningitis Miliary Tuberculosis
- Pathogenesis:
o Meningeal Infection Inflammation & Oedema ICP Vomiting, Drowsiness.
o NB: Meningococcal Sepsis can Thrombocytopaenia Maculopapular Rash ... DIC
- Morphology:
o Bacterial Exudate within Meninges (Pus beneath the meninges)
o Viral No pus
o Engorged Meningeal Vessels
- Clinical Features:
o ***Meningism:
*1. Neck Stiffness (Due to Inflammation of the Meninges)
Br d inski s Sign Posi i e (Flex the Neck Pt bends knee)
Kernig s Sign Posi i e (Flex the hip and attempt knee extension Pain

*2. Photophobia
*3. Headache
o + Constitutional Syx:
Fever/Malaise
Nausea/Vomiting
May eventually have loss of consciousness. (Rare)
Irritability
Poor Feeding
o Features Suggestive of Aetiology
Non-Blanching Maculopapular Rash Suggests Meningococcus
CSF Rhinorrheoa/Otorrhoea - basal skull fracture Suggests Pneumococcus, HiB, Strep.
- Diagnosis:
o **Clinical Suspicion: (Meningism +/- Rash +/- Fever/Malaise/Vomiting +/- Headache/ALOC
+/- Brud inski s Sign Kernig s Sign
o Blood Cultures BEFORE IV Antibiotics!!
o L3-L5 Lumbar Puncture CSF Examination:
LP can Coning if ICP DO NOT do LP if:
1. Papilloedema
C shing s Response Triad BP HR Irregular Breathing)
3. Unresponsive Pupils
Can Cerebral Hernia ion Aka Cis ern Obli era ion Often Fatal
o CSF Samples (Take 3):
Sample 1 Serology (or PCR)
Sample 2 Biochemistry (Glucose, Protein)
Sample 3 Bacteriology Most Precious (Gram Stain + Culture)

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 o CSF Interpretation:
Normal Bacterial Meningitis Viral/Aseptic
Meningitis
(Usually Herpes
Virus)
CSF Pressure Normal Normal-Raised Normal-Raised
White Cell Count Normal Raised (Polymorphs) Raised
(Lymphocytes)
Glucose Same as Serum Lower than Serum Normal
(Hungry Bacteria)
Protein Normal Raised (produced by the Raised (produced by
organisms) the organisms)
Gram Stain None Presence of Bacteria Nothing Aseptic
Meningitis
- Treatment:
o (Bacterial Meningitis = Emergency Can be Fatal)
o (Viral Meningitis = Usually Self-Limiting & Less Fulminant Clinically)
o ***Treat on Suspicion!! (Don t wait for lab results )
o 1. Blood Cultures BEFORE IV Antibiotics!!
o 2. Early Antibiotic Therapy is Essential for Good Outcome!!!
IV Benzylpenicillin G, or IV Cephtriaxone (why? Because they can enter the BBB)
o 3. Corticosteroids (Dexamethasone) WITH the Antibiotics CNS Inflamma ion
Improves Neurological Outcome of bacterial meningitis.
o 4. Fundoscopy, Then Lumbar Puncture (Check for Papilloedema before doing LP)
CSF MCS
o (+ Prophylactic Measures for Close Contacts):
Meningitis Prophylaxis: Rifampicin, Ceftriaxone or Ciprofloxacin:
Offered to Household, child care and CLOSE CONTACTS.
- Prognosis:
o Good prognosis with Aggressive Treatment.
:. Treatment on Suspicion: Empirical Antibiotics (or Antivirals).
- Complications:
o Acute:
Encephalitis
Cerebral infarction
Oedema
Herniation
Waterhouse-Frederichson Syndrome (Acute Adrenal Infarction)
( Petechial Haemorrhages, DIC, Septic Shock)
o Late:
Abscess
Subdural Empyema
Epilepsy
Leptomeningeal Fibrosis & Consequent Hydrocephalus

(Diplococci = Nesseria Meningitidis)

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ENCEPHALITIS:
- Aetiology:
o Almost Always Viral (**Herpes Simplex Virus, VZV, CMV, Poliovirus, Rabies [Rhabdovirus], JEV)
- Pathogenesis:
o Viraemia Crosses BBB CNS Infection Cerebral Oedema ICP Neurological Signs
- Clinical Features:
o Infective Syx Fever, Nausea, Vomiting
o + Cerebral Syx Encephalopathy (Altered Mental State/Abnormal Behaviour/ALOC/Drowsiness)
+/- Seizures
- Treatment:
o Treat on Suspicion (Acyclovir + Dexamethasone)
- Prognosis:
o Poor - Once symptomatic, rapid inflammation & necrosis Brain-Death or Neurological Deficit
o 70% Mortality Untreated
- Investigations:
o FBC (Lymphocytosis)
o LP L mphoc es Normal Gl cose Pro ein Nega i e C l res

Normal Bacterial Viral Meningitis Encephalitis

Meningitis (Usually Herpes (typically viral)
Virus)
CSF Pressure Normal Normal-Raised Normal-Raised Markedly Raised
White Cell Count Normal Raised Raised Raised
(Polymorphs) (Lymphocytes) (Lymphocytes)
Glucose Same as Serum Lower than Normal Normal
Serum (Hungry
Bacteria)
Protein Normal Raised (produced Raised (produced Raised
by the organisms) by the organisms)
Gram Stain None Presence of Nothing Aseptic Nothing
Bacteria Meningitis

BRAIN ABSCESSES:
Incapsulated pus within the brain occurring after an acute focal purulent infection.
o Sites of Focal Infection that could lead to brain abscesses:
Otitis Media
Sinusitis
Penetrating trauma
Haematogenous dissemination
o Given the Possible Sites of Entry, Which Organisms are Most Likely to be Involved?
Otitis Media Strep Pneumoniae
Sinusitis Strep Pneumoniae
Penetrating Trauma Probably Staph Aureus
o Diagnosis:
Blood culture should be performed, but often is not diagnostic
CT or MRI are Essential for Diagnosis.
Lumbar Puncture is Contraindicated Due to ICP
Inflammatory Markers WBC, CRP & ESR are raised.

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 NEUROLOGICAL Pathology:
DEMENTIAS

Global Degeneration Dementias (Age-Related (Senile), Alzheimers, Lewy-Body, & Fronto-Temporal Pick’s):
- Dementia:
o = Acquired Global Impairment of Intellect, but with no ALOC.
- Epidemiology:
o 5% of >55yrs are demented
o 20% of >80yrs are demented
o Prevalence Doubles every 5yrs Beyond Age:60.
o 50% of dementia pts have clinically significant behavioural/psychological symptoms.
- Common Symptoms: (In order of prevalence):
o Early Cognitive:
**Memory loss
o Later Non Cognitive:
Apathy/Depression
Delusions (False Beliefs)
Anxiety
Agitation/Aggression
Hallucinations
- Types of Primary Dementias:
o Age-Related (Senile) Dementia
o Alzheimers Disease
o Lewy-Body Dementia
o Fronto-Tem al Deme ia Pick Di ea e
- Clinical Diagnosis:
o Timeline of Symptom Progression (Memory Loss Agitation/Aggression, Wandering, Apathy)
o Impact on ADLs (Especially Medications & Financials)
o MMSE (Mini-Mental State Examination)

- AGE-RELATED (SENILE) DEMENTIA:

o Aetiology:
Old Age
o Pathogenesis:
Old-Age Neuronal Atrophy (Particularly Cortex & Hippocampus) Progressive Neuronal
Loss with Time
B ai Ma De d i ic B a che
Neurons are replaced by glial cells
o Morphology:
Macro:
Cortical atrophy
E la gi g f e icle C m e a H d ce hal
Thickening of Leptomeninges (Pia Mater & Arachnoid Mater) (The “Thin”
Meninges)
o Clinical Features:
Dementia: All Spheres of Intellect affected

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- ALZHEIMERS DISEASE:
o Commonest Cause of Dementia
o Aetiology:
Exact Aetiology Unknown
Genetic & Environmental Components
(NB: Inevitable in Down-Syndromes)
o Pathogenesis:
Excess β-Amyloid Protein Formation (A Degradation product of Amyloid Precursors)
β-Amyloid Protein Deposition around Neurons Neuritic Plaques
β-Amyloid Protein Deposition in Blood Vessels Amyloid Angiopathy
o Morph:
Severe Cortical Atrophy (Widened Sulci, Narrow Gyri)
T icall S a a d B ca A ea F al A ea Extends to the rest of the
brain (Affecting motor and sensory areas may even have paralysis)
Secondary Ventricular Dilation (Compensatory Hydrocephalus)

o Clinical Features:
May be as young as 50yrs old
SLOW Insidious Onset (Years) (Cf. Lewy-Body Dementia)
Early Signs: (Neuronal Atrophy Starts in the Hippocampus)
Memory Loss is :. the First Sign
Progressive Signs: (Neuronal Atrophy Progresses to the Cortex)
Mild Cortical Atrophy:
o Increased Memory Loss
o Confusion, Apathy, Anxiety
o Difficulty Handling Money
Moderate Cortical Atrophy:
o Difficulty Recognising People
o Difficulty with Language
o Wandering & Disorientation
Late Signs: (Extreme Global Cortical Atrophy)
Seizures, Incontinence
Groaning/Moaning/Grunting
o Treatment:
Acetylcholine-Esterase Inhibitors
o Prognosis:
Mean Survival = 7yrs from Onset.
Death Typically From Aspiration Pneumonia or Other Infections.

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- DIFFUSE LEWY-BODY DEMENTIA:
o 3rd Most Common (Behind Alzheimers & Vascular)
o Aetiology:
Unknown But Genetic Link.
o Pathogenesis:
Development of Abnormal Proteins (Alpha-Synuclein) in Neurons throughout the Brain
Impair the Functioning of Neurons Rapid Decline in Cognition, Memory, Attention &
Motor
o Morphology:
Macro:
Significant Cortical Atrophy
T icall S a a d B ca A ea Extends to the rest of the brain
o Clinical Features:
Rapid Onset (within few months) Cf Al heime Di ea e
Early:
Fl c a i g C g i i Deme ia STMemory, Confusion, Language Problems)
Vivid Visual Hallucinations (Eg. Strange Faces, Frightening Creatures, or Children)
Impairment in Attention
Later:
Parkinsonism with shuffling gait & cog-wheel rigidity..
Delusions
T a ie ALOC
o Treatment:
Pharmacological Cholinesterase Inhibitors are promising.

- FRONTO-TEMPORAL DEMENTIAS Incl. Pick’s Disease :

o 4th most common dementia af e Al heime Va c la Le -Body
o Aetiology:
Other Fronto-Temporal Dementias = Genetic
Pick Di ea e U k
o Pathogenesis:
Selective Build-up of Tau Proteins within Frontal & Temporal Lobe Neurons
Frontal Lobe Dysfunction Executive Function, Personality, Disinhibition.
Temporal Love Dysfunction Aphasias (Expressive & Receptive)
o Morphology:
Macro:
Selective Atrophy of Frontal & Temporal lobes
Sparing of the Parietal & Occipital Lobes
o Clinical Features:
Younger Patients (40-65yr olds)
Dysexectutive Syx: Inability to Coordinate & Execute Tasks
**Behaviour Changes: Behaviour & Personality Change & Disinhibition
NB A Defi i g fea e f Pick Di ea e i ha Beha i Pe ali Cha ge
cc PRIOR Mem L Cf Al heime he e Mem L cc Fi
Language Changes: Progressive Aphasia (Expressive & Receptive)
NB: Memory is Preserved until Late Stages.

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- VASCULAR DEMENTIA (Multi-Infarct Dementia):
o Epidemiology:
2nd M C mm behi d Al heime f All Deme ia
o Aetiology:
Cumulative Ischaemic Brain Damage
(Mostly due to Hypertension & Atherosclerosis)
o Pathogenesis:
Either Sudden Onset Following a CVA
Or Gradual Deterioration after Successive (often unnoticeable) CVAs.
Generalised Intellectual Loss Dementia
o Morphology:
Macro:
May have Necrotic/Fibrotic Foci (If Multi-Infarct) Often Visible on MRI/CT
May have a single, large Necrotic/Fibrotic Focus (Single, Large Infarct)
May have Hypertensive Lacunar Lesions
o Clinical Features:
Memory Loss
C g i i eF c i
Confusion
Mood Changes (Depression/Irritability)
Language Problems
Executive Dysfunction ADL Ea i g D e i g Sh i g ec
Ra id Sh ffli g Gai S me ime called A he cle ic Pa ki i m
Hx of Vascular Pathology (Past Hx of CVA, TIA, HTN & Focal Neurology)
o Treatment:
No Cure, but Preventable
o Prevention:
Control Hypertension
Reduce Cholesterol
Control Diabetes
Stop Smoking
Antiplatelet Drugs (Aspirin/Clopidogrel)

- DEMENTIA PUGILISTICA “Punch Drunk Syndrome :

o Aetiology:
Repetitive Trauma/Concussion
o Pathogenesis:
Repeated Concussive/Sub-Concussive Blows to the Head Cumulative Loss of Neurons,
Fibrosis, Hydrocephalus, Diffuse Axonal Injury & Cerebellar Damage.
o Morphology:
Hydrocephalus
Thinning of Corpus Callosum
o Clinical Features:
Slow Progression (Over Decades)
Deme ia Mem C g ii Pe ali
Pa ki i m T em C di a i
Unsteady Gait
Dysphasias

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 - WERNICKES-KORSAKOFF SYNDROME (Alcoholic Encephalopathy):
o Aetiology:
Alcohol Abuse Vit B1[Thiamine] Deficiency
o Pathology:
Alcohol Abuse Vit B1[Thiamine] Deficiency
Vit B1[Thiamine] is a cofactor for Glucose Metabolism :. Deficiency Neuronal ATP
Neuronal Atrophy (Particularly Cortex & Mamillary Bodies)
(Vit B1[Thiamine] Deficiency) Ataxia
o Morphology:
Cortical Atrophy
Mamillary Body Atrophy & Haemorrhages
Cerebellar Atrophy
o Clinical Features:
Cortical Atrophy Impaired Memory (Anterograde & Retrograde) + Confabulation
Mamillary Body Damage Vision Changes, Nystagmus, Unequal Pupils
Cerebellar Atrophy Ataxia
o Treatment:
Supplemental Thiamine + B12
(NB: B12 to prevent subacute degeneration of the cord)
o Prognosis:
By the time Amnesia & Psychosis are apparent, complete recovery is unlikely.

Amnesia:
- Typically Declarative Memory Loss. (Therefore Hippocampal Damage)
- Commonly caused by Temporal Lobe Damage (Hippocampus and/or Thalamus)
o NB: L-Hippocampus = Language
R-Hippocampus = Spatial Memory
- Anterograde:
o - Inability to form new memories from time of Injury/Damage Onwards.
o Non-Declarative Memory is Unaffected
- Retrograde:
o - Inability to recall memories from time of Injury/Damage Backwards.

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 BEHAVIOURAL NEUROLOGY . . . CONT.
Management
❏ treat underlying cause
❏ supportive measures
• nurse in a well-lit room
• IV therapy (for fluid/lyte disturbance)
• chlorpromazine or haloperidol (5-10 mg IM) if patient’s behaviour disruptive
• respiridone can be used for agitation
• diazepam if DTs (delerium tremens)
DEMENTIA
❏ a clinical syndrome of acquired and progressive decline in higher cortical functioning in comparison
with previous level of functioning, occurring in an alert patient
❏ remember:” IMP” - Intelligence, Memory, Personality
Operating Criteria for Dementia
❏ memory impairment plus at least one of the following:
aphasia, apraxia, agnosia, disturbance in executive functioning
❏ disturbance significantly interferes with work, social activities, or relationships
❏ disturbance does not occur exclusively during delerium
❏ memory impairment: recent before remote memory impairment
❏ other cognitive signs
• learning and retaining new information
• handling complex tasks
• reasoning/ impaired judgment
• spatial abilities and orientation
• language (word finding)
❏ personality change
• decline in personal manners/social awareness
• disinhibited behaviour (sexually aggressive/criminal)
• coarsening: an exaggeration of premorbid character traits
• delusions may develop
• deterioration in grooming/hygiene; urinary/fecal incontinence
Epidemiology
❏ incidence increases with age
❏ 4% population > 65 years severely demented
❏ 11% population > 65 years mild-moderate dementia
❏ 60-80% of dementia due to Alzheimer’s disease; 10-20% due to vascular disease;
10-15% due to a mixed picture
• ~ 75 other causes
• medications and depression are important mimics
❏ risk factors: age, family history, diabetes
Approach to Dementia
❏ want to elicit treatable causes
❏ history
• rate of cognitive decline: weeks, months/years, stepwise (strokes)
• degree of impairment of social function
• general health
• nutritional status
• drug history
• family history of dementia
• important to obtain collateral information
❏ physical exam
• mental status exam (Folstein with a cutoff of 24/30 - sensitivity 87%, specificity 82%)
• visual spatial testing, frontal lobe testing, supplementary memory tests
• focal neurological signs
• involuntary movements
• pseudobulbar signs
• primitive reflexes (e.g. glabellar, pout, snout, palmomental, grasp)
❏ investigations
• all patients: CBC, lytes, BUN, creatinine, glucose, AST, ALT, ALP, PT/PTT,
albumin, Ca2+, TSH, vitamin B12, folate, and VDRL
• as clinically indicated: phosphorus, ESR, FTA, urinalysis
• CXR, ECG, EEG, LP
• CT or MRI
• neuropsychological testing may help
CAUSES OF DEMENTIA (D-E-M-E-N-T-I-A)
D-Degenerative
Alzheimer’s Disease
❏ most common dementia; females > males; ~ 15% of cases familial
❏ progression is slow over years
❏ triad of memory loss, language impairment and visual spatial dysfunction
❏ also visual agnosia (ability to see but not recognize objects), apraxia
(inability to perform certain motor tasks in absence of paralysis)

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 BEHAVIOURAL NEUROLOGY . . . CONT.
❏ diagnosis
• exclusion of all other causes of dementia by history, physical and labs
• physical exam: primitive reflexes; 8 motor tone with motor dyspraxias; myoclonus/seizures may follow
❏ pathology: cortical atrophy, ventricular dilatation, neuritic plaques, neurofibrillary tangles,
decreases in cholinergic neurons
❏ investigations: to find a treatable cause if present
❏ treatment
• symptom relief and support (family/caregiver relief)
• mild sedation (Trazadone) if aggressive behavior
• support groups
• new acetylcholinesterase inhibitors (donepezil, tacrine, rivastagmine, galactamine) may be used for
symptom relief but does not modify progression of disease
Lewy Body Disease
❏ extrapyramidal motor signs, progressive dementia
• prominent fluctuations in mental status
• visual hallucinations common
❏ management: DO NOT USE Haldol (phenothiazines) risk of severe extrapyramidal toxicity
❏ pathology: Lewy bodies throughout cortex and brainstem nuclei
Pick’s Disease
❏ degenerative dementia affecting frontal and temporal lobes
❏ clinically similar to Alzheimer’s
❏ personality changes of frontal lobe syndrome: disinhibition, loss of social graces, jocularity
and apathy punctuated by irritability
❏ difficulty concentrating
❏ language dysfunction: decreased verbal output, word-finding difficulty (anomic aphasia)
❏ temporal lobe involvement: transcortical or fluent aphasias and memory loss
❏ peak onset 55-65 years, slightly greater female predominance
❏ thought to be autosomal dominant although cause unknown
❏ pathology: pick bodies in the neocortex and hippocampus
Other Degenerative Causes of Dementia Include
❏ Parkinson’s disease, Huntington’s disease (see Movement Disorders section)
❏ progressive supranuclear palsy, olivopontocerebellar degeneration
E-Emotional
❏ depression, schizophrenia (see Psychiatry Chapter)
M-Metabolic
❏ hypothyroidism/hyperthyroidism, hypocalcemia/hypercalcemia
❏ hypoglycemia/hyperglycemia
❏ hyperaldrenocorticism (Cushing syndrome)
❏ electrolyte abnormalities
❏ systemic organ failure - renal failure (uremia), hepatic encephalopathy
❏ Wilson’s disease
❏ metachromatic leukodystrophy
E-Eyes and Ears
❏ severe hearing and visual impairment
N-Nutritional and Normal Pressure Hydrocephalus (NPH)
❏ vitamin B12 deficiency
• subacute combined degeneration of spinal cord and brain
❏ folate deficiency
❏ other water soluble vitamin deficiency
❏ niacin deficiency
• pellagra (diarrhea, dermatitis, dementia, death)
❏ normal pressure hydrocephalus (NPH)
• history
• temporal sequence of gait apraxia, incontinence, dementia
• if sequence not followed, NPH unlikely
• history of SAH, meningitis, trauma may be important and related to etiology
• diagnosis: history, physical (frontal gait pattern), CT scan (markedly dilated ventricles without cortical
atrophy), RISA scan, diagnostic CSF tap (Miller Fisher test – objective assessment of gait before and
after removal of 30cc CSF)
• treatment: CSF shunting may lead to improved clinical state
• positive response to CSF tap is a good prognostic indicator
• unlikely to benefit if demented
T-Trauma, Tumours, Toxins
❏ subdural hematoma
• headache usually present
• no history of trauma in 1/3 cases
• suspect if drowsiness in elderly with recent personality change
❏ head injury
❏ primary or metastatic brain tumours
❏ drugs (e.g. barbiturates, anticholinergics, anticonvulsants, methyldopa)
❏ alcohol - Wernicke-Korsakoff syndrome (thiamine deficiency)
❏ heavy metals - lead, mercury, arsenic, thallium
N18 – Neurology MCCQE 2002 Review Notes

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 BEHAVIOURAL NEUROLOGY . . . CONT.
I-Infection
❏ tertiary syphilis
❏ AIDS - HIV encephalopathy
❏ chronic meningitis (e.g. TB)
❏ encephalitis
❏ Creutzfeldt-Jacob Disease (CJD)
• rapidly progressive, inevitably fatal prion disease of CNS characterized by
progressive dementia, myoclonus and affecting adults in midlife
• spread of disease: iatrogenic: corneal transplantation, injection of human growth
hormone (prepared from pooled cadaveric pituitary glands)
• new variant CJD - “mad cow disease” by oral ingestion
• prodromal symptoms: fatigue, depression, weight loss, insomnia, anorexia
• delirium, changes in behavior, emotional response, and intellectual function
• cerebellar ataxia, visual disturbances, myoclonic contractions, dysarthria
• startle myoclonus evocable by sensory stimuli of all sorts or may be spontaneous
• stupor, coma
• EEG pattern distinctive: triphasic waves changing over course of disease from
diffuse nonspecific slowing to stereotyped periodic high voltage slow and sharp wave complexes
on an increasingly flat background (burst suppression)
• pathology: widespread neuronal loss and gliosis accompanied by a
striking vacuolation of cerebral and cerebellar cortices
A-Atherosclerotic and Vascular
❏ multi-infarct dementia
• most common vascular dementia, but often over diagnosed
• history
• abrupt onset
• stepwise deterioration
• history of strokes
• focal motor/sensory/cognitive symptoms and signs
• diagnosis: history; Hachinski Ischemic Score; confirmed by CT, MRI, SPECT
• treatment (see Stroke Section)
❏ cerebral hemorrhage
❏ post-anoxic
❏ vasculitis
❏ severe stenosis of large neck vessels
APHASIA (Dysphasia)
❏ a disorder of language produced by a cerebral lesion characterized by errors in speech production,
impaired comprehension, reading, writing and word-finding difficulty
❏ aphasia is an important localizing symptom usually indicative of dominant hemispheric dysfunction
(usually the left hemisphere)
Preassessment Information Needed
❏ handedness (writing, drawing, using toothbrush, scissors)
❏ educational level
❏ native language
❏ preexisting learning difficulties
Language Representation
❏ in left hemisphere for almost all right-handed people and most left-handed people (75%)
Neuroanatomy of Aphasia (see Figure 12)
❏ posterior inferior frontal lobe (Broca’s area) used for motor speech production
❏ Wernicke’s area (posterior superior temporal and inferior parietal) used for comprehension of
spoken language and for initiation of reply or action
❏ visual stimuli reach Wernicke’s area through angular gyrus which is thought to be important for comprehension
of written language
❏ these two areas connected through association bundle (arcuate fasciculus) and altogether comprise
the perisylvian language zone
❏ aphasias may also result from damage to areas of the brain outside the perisylvian language zone
(transcortical aphasias)
motor cortex
arcuate fasciculus central sulcus
Broca’s area sensory cortex

Wernicke’s area

Figure 12. Location of the

Speech Centres
Illustration by Aimee Warrell
MCCQE 2002 Review Notes Neurology – N19

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 NEUROLOGICAL Pathology:
EAR PATHOLOGY

Ear Complaints:
- Deafness:
o Conductive:
Due to a condition that blocks the conduction of sound through the Outer & Middle Ear.
Originating in the Outer Ear:
o External auditory canal obstruction. (Wax/Foreign Bodies/Infection/Pus)
Originating in the Tympanic Membrane:
o Perforation or Scarring due to Infection or Trauma.
Originating in the Middle Ear:
o Otitis Media Fills the Tympanic Cavity with pus.
o Otosclerosis Ossicles become fused together

o Sensorineural:
Due to a disorder of the inner ear, the cochlear nerve, or its central connections to the
brain.
Noise-Induced Damage to Cochlear Hair Cells.
Stroke in the Auditory Cortex causing hearing loss.
Acoustic Neuroma (tumour of CN8) causing deafness on the affected side.
Ototoxic Drugs Damage to Cochlea Nerve
Meningitis
Presb c sis Progressive age-related hearing degradation.

- Tinnitus:
o A Percieved Ringing in the ears Or buzzing hissing clicking roaring etc
o Commonly occurs immediately after Acoustic Trauma (Eg. Clubbing)
o Is a problem of the Peripheral auditory system (Not the brain)

- Vertigo:
o Sensation of Spinning Distinct from faintness
o Usually a problem of the Vestibular Apparatus or the Vestibulocochlear Nerve (CN8)
Benign Paroxysmal (sudden onset) Positional Vertigo
Vestibular Neuronitis
Drugs (Eg. Alcohol)
Brainstem Lesions, Multiple Sclerosis, Migraine.

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- Otalgia:
o Pain of the Ear
o Most often caused by some form of Otitis:
Acute Otitis Media (Acute Middle Ear Infection).
Caused by Respiratory Pathogens which enter via Eustachian Tube.
Can Present with:
o URTI; Rhinitis; Cough; Fever
o Ear ache Ear-pulling
Otoscopy may show:
o Swollen, bulging, red Ear-drum.
o Fluid behind drum (fluid in middle ear)

Chronic Otitis Media (Chronic Middle Ear Infection):

Caused by:
o Chronic Respiratory Infections
o Nasopharyngeal colonisation in early infancy.
o Eustachian tube dysfunction
Can Present with:
o Chronic ear discharge.
o Hearing loss
o Developmental delay/poor school performance.
Can result in a variety of Conditions:
o Retraction or Perforation of Eardrum.
o Loss of Eardrum Elasticity
o Fluid behind an intact eardrum.
o Scarring of ossicles.
o Erosion of the bony cavity of the middle ear.
o Cholesteatoma (Keratinizing Squamous epithelioma in mid.ear or eardrum)
Grommets can relieve middle ear pressure and allow draining of exudate.

Otitis Externa (External Ear Infection):

Caused by:
o Humidity/Swimming Bacterial/Fungal Infection.
Can Present with:
o Itch; Pain; Ear Pulling
o Fever
o Discharge

- Otorrhoea:
o Runny Ear

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 o Can be Caused by:
Otitis Media:
Acute with perforation
Chronic
Otitis Externa
Foreign Body
Cholesteatoma Chronic Infection/Erosion

Ear-Related History:
- Past Medical:
o Ear Infections?
o Ear Trauma?
o Diabetes?
o MS?
- Past Surgical:
o Ear Surgery?
- Medication?
o NB: Some are ototoxic.
- Noise Exposure:
o Occupational
o Recreational

Ear Examination:
- External Ear:
o Inspection
o Palpation (tenderness) + Cervical & Occipital Lymph Nodes.
- Otoscopy:
o Inspection of Tympanic Membrane & Middle Ear
- Hearing Tests:
o Whisper Test
o Tuning Fork Tests
o Testing for Nystagmus (Involuntary eye movement due to head movement)
o Romberg s Test standing feet together then closing eyes Loss of balance positive
o Tympanometry (tests the eardrum & ossicle mobility)
o Audiometry tests a person s responses to different pitches volumes

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 AUDIOLOGY . . . CONT.

Static Compliance
❏ volume measurement reflecting overall stiffness of the middle ear system
❏ normal range: 0.3 to 1.6 cc
❏ negative middle ear pressure and abnormal compliance indicate middle ear pathology
Acoustic Stapedial Reflexes
❏ stapedius muscle contracts when ear is exposed to loud sound and results in increased stiffness or
impedance of middle ear system (TM and ossicles)
❏ acoustic reflex thresholds occur at 70-100 dB above hearing threshold
❏ if hearing threshold is greater than 85 dB, the reflex is likely to be absent
❏ stimulating either ear causes reflex to occur bilaterally and symmetrically
❏ reflex pathway involving vestibulocochlear cranial nerve, cochlear nucleus, trapezoid body, superior olivary
nucleus, facial nucleus, and facial nerve (i.e. a measure of central neural function)
❏ for reflex to be present, CN VII must be intact and there must be no conductive hearing loss in the monitored
ear. If reflex is absent without conductive loss or severe sensorineural loss, suspect CN VIII lesion
❏ acoustic reflex decay test: tests the ability of the stapedius muscle to sustain contraction for 10 s at
10 dB stimulation
❏ normally, little reflex decay occurs at 500 and 1000 Hz
❏ with cochlear hearing loss the acoustic reflex thresholds are typically 25-60 dB
❏ with retrocochlear hearing loss (e.g. acoustic neuroma) may find absent acoustic reflexes or significant reflex
decay (> 50%) within 5 second interval
AUDITORY BRAINSTEM RESPONSE (ABR)
❏ the patient is exposed to an acoustic stimulus while an electroencephalogram is recorded to assess any
changes in brain activity
❏ delay in brainstem response is suggestive of cochlear or retrocochlear abnormalities (for the latter think
tumour or multiple sclerosis (MS))

Clinical Pearl
❏ This objective test can be used in screening newborns or much more rarely to uncover
normal hearing in malingering patients.

HEARING LOSS
*common
Hearing Loss +less common

Conductive Sensorineural

External Ear Canal Middle Ear Congenital Aquired

* cerumen * acute otitis media - hereditary defects * presbycusis common in elderly
* otitis externa * serous otitis media - prenatal TORCH infection + Meniere’s disease
+ foreign body * perforation of TM - perinatal TORCH infection + noise-induced
+ congenital atresia + otosclerosis - postnatal TORCH infection + ototoxic drug
+ keratosis obturans + congenital: ossicular fixation + head injury
+ tumour of canal + trauma (hemotympanum) + sudden SNHL
+ tumour (cholesteatoma) + labyrinthitis
+ meningitis
+ demyelinating disease
+ trauma (temp bone #1)
+ tumour

OT10 – Otolaryngology MCCQE 2002 Review Notes

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 HEARING LOSS . . . CONT.

OTITIS EXTERNA (OE)

Clinical Pearl
❏ Otitis externa has two forms: a benign painful infection of the outer canal that could occur
in anybody and a potentially lethal less painful (damaged sensory nerves) disease which
usually occurs in old, immunosuppressed or diabetic patients.
Etiology
❏ caused by
• bacteria: P. aeruginosa, P. vulgaris, E. coli, S. aureus
• fungi: Candida albicans, Aspergillus niger
❏ more common in summer
❏ associated with swimming ("swimmer's ear"), mechanical cleaning (Q-tips, skin dermatitides)
Presentation
❏ acute
• pain aggravated by movement of auricle (traction of pinna or pressure over tragus)
• +/– unilateral headache, +/– low grade fever
• otorrhea - sticky yellow purulent discharge
• conductive hearing loss - due to obstruction of external canal with purulent debris
• post-auricular lymphadenopathy
❏ chronic
• pruritus of external ear +/– excoriation of ear canal
• atrophic and scaling epidermal lining
• +/– otorrhea, +/– hearing loss
• wide meatus but no pain with movement of auricle
• tympanic membrane appears normal
Treatment
❏ clean ear under magnification with irrigation, suction, dry swabbing, and C+S
❏ bacterial etiology
• if membrane intact, give topical aminoglycoside antibiotics +/- corticosteroids
(e.g. Garamycin, Neosporin, Corticosporin)
• if perforated membrane, give ciprofloxacin otic drops, because aminoglycosides can be ototoxic
• introduction of fine gauze wick (pope wick) if external canal edematous
• +/– 3% acetic acid solution to acidify ear canal
• systemic antibiotics if either:
1. cervical lymphadenopathy
2. cellulitis
❏ fungal etiology
• alcohol/acetic acid instillation, clotrimazole, locacortin (Vioforme)
❏ +/– analgesics
❏ chronic otitis externa pruritus without obvious infection - corticosteroid alone e.g. diprosalic acid
Malignant Otitis Externa (rare)
❏ osteomyelitis of temporal bone, 99% of which are due to Pseudomonas
❏ associated with diabetics, elderly, perichondritis, cellulitis, parotitis, +/– chronic symptoms
❏ requires hospital admission, debridement, IV antibiotics and emergent CT scan
ACUTE OTITIS MEDIA (AOM) AND OTITIS MEDIA WITH EFFUSION (OME)
(see Pediatric ENT section)
CHOLESTEATOMA (see Colour Atlas OT5)
❏ in growth of keratinized squamous epithelium in middle-ear or mastoid as a result of retraction of the TM
Congenital
❏ behind an intact tympanic membrane “small white pearl", not associated with otitis media
❏ usually presents with conductive hearing loss
Acquired
❏ frequently associated with retraction pocket in pars flaccida and marginal perforations of the tympanic
membrane
❏ erodes mastoid bone, then ossicles
❏ associated with chronic otitis media with painless otorrhea
Complications
❏ chronic otitis media
❏ CNS dysfunction/infection
❏ late complications: hearing loss, vertigo, facial palsy
Treatment
❏ excision via cortical, modified radical, or radical mastoidectomy depending on the extent of disease
+/– tympanoplasty
❏ tympanic membrane repair and ossicle reconstruction if no sign of recurrence
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 HEARING LOSS . . . CONT.

MASTOIDITIS
❏ osteomyelitis (usually subperiosteal) of mastoid air cells, most commonly seen approximately two weeks after
onset of untreated (or inadequately treated) acute suppurative otitis media
❏ previously common but is now rare due to rapid and effective treatment of acute otitis media with antibiotics
Presentation
❏ pinna displaced laterally and inferiorly
❏ persistent throbbing pain and tenderness over mastoid process
❏ development of subperiosteal abscess, post-auricular swelling
❏ spiking fever
❏ hearing loss
❏ otorrhea with tympanic membrane perforation (late)
❏ radiologic findings: opacification of mastoid air cells by fluid and interruption of normal trabeculations of cells
Treatment
❏ IV antibiotics with myringotomy and ventilating tubes
❏ cortical mastoidectomy
• debridement of infected tissue allowing aeration and drainage
• requires lifelong follow-up with otolaryngologist
❏ indications for surgery
1. failure of medical treatment after 48 hours
2. symptoms of intracranial complications
3. aural discharge persisting for 4 weeks and resistant to antibiotics
OTOSCLEROSIS (see Figure 7)
❏ commonest cause of conductive hearing loss between 15 and 50 years of age
❏ autosomal dominant, variable penetrance approximately 40%
❏ female > male - progresses during pregnancy (hormone responsive)
❏ 50% bilateral
Presentation
❏ progressive conductive hearing loss first noticed in teens and 20s
(may progress to sensorineural hearing loss if cochlea involved)
❏ +/– pulsatile tinnitus
❏ tympanic membrane normal +/– pink blush (Schwartz's sign) associated with the neovascularization
of otosclerotic bone
❏ characteristic dip at 2,000 Hz (Carhart's Notch) on audiogram (Figure 7)
Treatment
❏ stapedectomy with prosthesis is definitive treatment
❏ hearing aid may be used, however usually not a good long term solution
CONGENITAL SENSORINEURAL HEARING LOSS
❏ genetic factors are being identified increasingly among the causes of hearing loss
Hereditary Defects
❏ non-syndrome associated (70%)
• often idiopathic
• autosomal recessive
❏ syndrome associated (30%)
• Waardenburg's - white forelock, heterochromia iridis, wide nasal bridge and increased distance
between medial canthi
• Pendred's - goiter
• Treacher-Collins - first and second branchial cleft anomalies
• Alport's - hereditary nephritis
Prenatal TORCH Infections
❏ Toxoplasmosis, Others (e.g. HIV), Rubella, Cytomegalovirus (CMV), Herpes simplex
Perinatal
❏ Rh incompatibility
❏ anoxia
❏ hyperbilirubinemia
❏ birth trauma (hemorrhage into inner ear)
Postnatal
❏ meningitis
❏ mumps
❏ measles
OT12 – Otolaryngology MCCQE 2002 Review Notes

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 HEARING LOSS . . . CONT.

High Risk Registry (For Hearing Loss in Newborns)

❏ risk factors
• low birth weight/prematurity
• perinatal anoxia (low APGARs)
• kernicterus - bilirubin > 25 mg/dL
• craniofacial abnormality
• family history of deafness in childhood
• 1st trimester illness - CMV, rubella
• neonatal sepsis
• ototoxic drugs
• perinatal infection, including post-natal meningitis
• consanguinity
❏ 50-75% of newborns with sensorineural hearing loss have at least one of the above risk factors, and 90% of
these have spent time in the NICU
❏ presence of any risk factor: Auditory Brainstem Response (ABR) study done before leaving NICU and at
3 months adjusted age
❏ refer for hearing assessment
❏ if not identified and rehabilitated within six months, intellectual deterioration in deaf children occurs
❏ must detect and rehabilitate hearing loss near birth in every case so that the child can reach his/her potential
PRESBYCUSIS (very common) (see Figure 7)
❏ hearing loss associated with aging - 5th and 6th decades
❏ most common cause of sensorineural hearing loss
Etiology
❏ hair cell degeneration
❏ age related degeneration of basilar membrane
❏ cochlear neuron damage
❏ ischemia of inner ear
Presentation
❏ progressive, gradual bilateral hearing loss initially at high frequencies, then middle frequencies
❏ loss of discrimination of speech especially with background noise present -
patients describe people as mumbling
❏ recruitment phenomenon: inability to tolerate loud sounds
❏ tinnitus
Treatment
❏ hearing aid if hearing loss > 30-35 dB
❏ +/– lip reading and auditory training
SUDDEN SENSORINEURAL HEARING LOSS (UNILATERAL)
❏ presents as a sudden onset of significant hearing loss (usually unilateral) +/– tinnitus
❏ unexplained etiology (?autoimmune, viral, microcirculation, trauma)
❏ rule out transient ischemic attack (TIA) and systemic lupus erythematosus (SLE)
❏ CT to rule out tumour or cerebrovascular attack (CVA) if associated with any other focal neurological signs
(e.g. vertigo, ataxia, abnormality of CN V or VII, weakness)
❏ treat with
• low molecular weight dextran
• corticosteroids (systemic or intratympanic)
• bedrest
❏ prognosis
• 70% resolve spontaneously within 10-14 days
• 20% experience partial resolution
• 10% experience permanent hearing loss
DRUG OTOTOXICITY
Aminoglycosides
❏ increased toxicity with oral administration
❏ can occur with topical preparations in patients with perforated tympanic membranes
❏ destroys sensory hair cells
❏ high frequency hearing loss develops earliest
❏ ototoxicity occurs days to weeks post-treatment
❏ streptomycin (vestibulotoxic), kanamycin and tobramycin (toxic to cochlea),
gentamycin (vestibulotoxic and cochlear toxic)
❏ must monitor levels with peak and trough levels when prescribed, especially if patient has neutropenia,
history of ear or renal problems
❏ q24H dosing, with amount determined by creatinine clearance not serum creatinine alone
Salicylates
❏ hearing loss with tinnitus, reversible if discontinued

MCCQE 2002 Review Notes Otolaryngology – OT13

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 HEARING LOSS . . . CONT.

Quinine and Antimalarials

❏ tinnitus
❏ reversible if discontinued but can lead to permanent loss
❏ treat drug ototoxicity with IV low molecular weight dextrans
NOISE-INDUCED SENSORINEURAL HEARING LOSS (see Figure 7)
❏ may be occupational, often associated with tinnitus
❏ 85-90 dB over months or years causes cochlear damage
❏ early-stage hearing loss at 4000 Hz (because this is the resonant frequency of the temporal bone),
extends to higher and lower frequencies with time
❏ speech reception not altered until hearing loss > 30 dB at speech frequency, therefore considerable damage
may occur before patient complains of hearing loss
❏ difficulty in discriminating, especially in situations with competing noise
Phases of Hearing Loss
❏ dependent on intensity level and duration of exposure
❏ temporary threshold shift
• when exposed to loud sound, decreased sensitivity or increased threshold for sound
• with removal of noise, hearing returns to normal
❏ permanent threshold shift
• hearing does not return to previous state
Treatment
❏ hearing aid
❏ prevention
• ear protectors: muffs, plugs
• machinery which produces less noise
• limit exposure to noise with frequent rest periods
• regular audiologic follow-up
ACOUSTIC NEUROMA (AN) (see Neurosurgery Chapter)
❏ Schwannoma of the vestibular portion of CN VIII
❏ most common intracranial tumour causing hearing loss
❏ starts in the internal auditory canal and expands into cerebellopontine angle (CPA),
compressing cerebellum and brainstem
❏ may be associated with Type 2 neurofibromatosis (NF2) (bilateral tumours of CN VIII in internal auditory canal,
cafe-au-lait lesions, multiple intracranial lesions) (see Neurology Chapter)
Presentation
❏ usually presents with unilateral sensorineural hearing loss
❏ dizziness and unsteadiness may be present, but no true vertigo
❏ facial nerve palsy and trigeminal (V1) sensory deficit (corneal reflex) late complications
Diagnosis
❏ enhanced CT/MRI
❏ audiogram - puretone threshold elevated
❏ poor speech discrimination and stapedial reflex
❏ absent or significant reflex decay
❏ Acoustic Brainstem Reflexes (ABR) - increase in latency of the 5th wave
❏ electronystagmography (ENG)
Treatment
❏ conservative “wait and see”
❏ definitive management is surgical excision
❏ other options, such as gamma knife
TEMPORAL BONE FRACTURES
HEARING LOSS . . . CONT.
Types
1. transverse fractures
• extends into bony labyrinth and internal auditory
meatus (20%)
2. longitudinal fractures
• extends into middle ear (80%)
1
2 • in reality, the fractures rarely adhere to either of these patterns

Figure 8. Types of Temporal Bone Fractures

Illustration by Teddy Cameron
OT14 – Otolaryngology MCCQE 2002 Review Notes

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 HEARING LOSS . . . CONT.

Table 2. Features of Temporal Bone Fractures (see Figure 8)

Transverse Longitudinal

Incidence 10-20% 70-90%

Etiology frontal/occiptal lateral skull trauma

CN pathology CN VII palsy CN VII palsy (10-20%)

Hearing loss sensorineural loss due to direct cochlear injury conductive hearing loss secondary
to ossicular injury

Vestibular symptoms sudden onset vestibular symptoms due to direct rare

semicircular canal injury (vertigo, spontaneous nystagmus)

Other features • intact external auditory meatus, tympanic membrane • torn tympanic membrane
+/– hemotympanum with hemotympanum
• spontaneous nystagmus • bleeding from external auditory canal
• CSF leak in eustachian tube to nasopharynx +/or • step formation in external auditory canal
rhinorrhea (risk of meningitis) • CSF otorrhea
• Battle’s sign = mastoid ecchymoses
• Raccoon eyes = periorbital ecchymoses

Diagnosis
❏ otoscopy
❏ do not syringe or manipulate external auditory meatus due to risk of inducing meningitis via TM perforation
❏ radiology
• CT
❏ facial nerve tests (for transverse fractures), EMG, Schirmer's test, gustometry, stapedial reflexes, ENG
Treatment
❏ hemotympanum signifies significant force sustained, therefore monitor hearing until it returns to normal
❏ medical - expectant, prevent otogenic meningitis
• IV antibiotics if suspect CSF leak (penicillin G for 7-10 days)
❏ surgical - explore temporal bone, indications are
1. early meningitis (mastoidectomy)
2. bleeding from sinus
3. CSF otorrhea
4. CN VII palsy (complete)
5. gunshot wound
6. depressed fracture of external auditory meatus
Complications
❏ acute otitis media +/– labyrinthitis +/– mastoiditis
❏ meningitis / epidural abscess / brain abscess
❏ post-traumatic cholesteatoma
AURAL REHABILITATION
❏ dependent on degree of hearing loss, communicative requirements and difficulties, motivation and
expectations, age, and physical and mental abilities
❏ factors affecting prognosis with hearing aid/device
• poor speech discrimination
• narrow dynamic range (recruitment)
• unrealistic expectations
• cosmetic
❏ types of hearing aids
• behind the ear - BTE
• all in the ear - ITE
• bone conduction
• contralateral routing of signals (CROS)
❏ assistive listening devices
• direct/indirect audio output
• infrared, FM, or induction loop systems
• telephone, television, or alerting devices
❏ cochlear implant
• electrode is inserted into the cochlea to allow direct stimulation of the auditory nerve
• for profound bilateral sensorineural hearing loss not rehabilitated with conventional hearing aids
• established indication: post-lingually deafened adults and children

MCCQE 2002 Review Notes Otolaryngology – OT15

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 VERTIGO
EVALUATION OF THE DIZZY PATIENT
❏ patients can present with a wide range of subjective descriptions of their symptoms: dizziness, spinning,
lightheadedness, giddiness, unsteadiness
❏ true vertigo is defined as an illusion of rotary movement of self or environment, made worse in the absence of
visual stimuli
❏ it is important to distinguish vertigo from other disease entities that may present with similar complaints
(e.g. cardiovascular, psychiatric, neurological, aging)
❏ diagnosis is heavily dependent upon an accurate history
• description of rotary movement
• onset and duration
• hearing and tinnitus
• effect of dark/eye closing
• relation to body position
• alcohol and drug history (antihypertensives, aminoglycosides)
• medical history (vascular disease, anxiety disorder)

DIZZINESS

Dysequilibrium Vertigo Presyncope Psychological Physiological

Without Vertigo Dizziness Dizziness
-Ototoxicity -Cardiac Arrhythmias -Anxiety -Motion Sickness
(bilateral vestibular hypofunction) -Vasodepressor syncope -Panic Disorder -Mal de Debarquement
-Presbystasis -Orthostatic Hypotension -Phobic Dizziness
-Sensory Ataxia -Hypoglycemia
-Myelopathy or peripheral -Hyperventilation
neuropathy -Anemia
-Cerebellar Atrophy
-Apraxia Syndromes
-Extrapyramidal Disorders
-Endocrine Disorders
(Hypothyroidism)

Peripheral Central Systemic

-Benign Paroxysmal Postional Vertigo -Tumour -Medications
-Meniere’s Disease -Multiple Sclerosis (aminoglycosides,
-Recurrent Vestibulopathy (post-viral or -Transient Ischemic Attacks alcohol, anticoagulants)
post traumatic) -Vertebrobasilar Artery Insufficiency
-Transient Ischemic -Migraine-associated dizziness
-Labyrinthitis -Seizure Disorders
-Acoustic Neuroma -Syphilis
-Trauma (skull fractures, barotrauma)

Figure 9. Differential Diagnosis of Dizziness (all those in bold = Otolaryngologist’s domain)

Table 3. Differential Diagnosis of Vertigo

Condition Duration Hearing Loss Tinnitus Aural Fullness Other Features

Benign Paroxysmal seconds none none none
Positional Vertigo (BPPV)
Meniere's Disease minutes-hours uni/bilateral + pressure/warmth
precedes attack
Recurrent Vestibulopathy minutes to hours none none none
Vestibular Neuronitis hours-days unilateral none none
Labyrinthitis days unilateral whistling none recent AOM
Acoustic Neuroma chronic progressive none none ataxia CN Vll palsy
(see OT14)

Clinical Pearl
❏ True nystagmus and vertigo will never last longer than a couple of weeks if caused
by a peripheral lesion because compensation occurs; such is not true for a central lesion.

OT16 – Otolaryngology MCCQE 2002 Review Notes

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 VERTIGO . . . CONT.

Benign Paroxysmal Positional Vertigo (BPPV)

❏ most common cause for episodic vertigo
❏ acute attacks of transient vertigo initiated by certain head positions lasting seconds to minutes, accompanied
by nystagmus that fatigues on repeated testing
❏ due to migration of a small flake of bone or detached mineral crystals from utricular otolith organ
(cupulolithiasis) into posterior semicircular canal ––> floats to rest on one of the sensitive balance organs
• may occur following a head injury, viral infection (URTI), degenerative disease or idiopathic
• results in slightly different signals being received by the two balance organs
resulting in sensation of movement
❏ diagnosed by history and positive Dix-Hallpike manoeuvre (see Otoneurological Examination section)
❏ treat symptomatically and reassure patient that process resolves spontaneously
• commonly treated with particle repositioning maneuvers
(Epley’s manoeuvre or Brandt-Daroff exercises)
• drugs to suppress the vestibular system delay eventual recovery and are therefore not used
Meniere's Disease (endolymphatic hydrops)
❏ peak incidence (40-60 years)
❏ characterized by vertigo, fluctuations in hearing loss, tinnitus, and aural fullness, +/– drop attacks (N/V)
❏ vertigo (lasting minutes to hours) disappears with time and patient is left only with hearing loss
❏ early in the disease, hearing returns to normal in the attack-free states
❏ later stages are characterized by a unilateral, fluctuating low-frequency hearing loss and a persistance
of tinnitus (most hearing loss becomes bilateral with time)
❏ attacks come in clusters and may be very debilitating to the patient, may be triggered by stress
❏ pathogenesis: inadequate absorption of endolymph leads to endolymphatic hydrops
(over accumulated) that distorts membranous labyrinth
❏ treatment
• acute management may consist of bedrest, IV antiemetics, antivertiginous drugs (Serc),
and low molecular weight dextrans
• longterm management may be
• medical
• low salt diet, K+ sparing diuretics (e.g. triamterene, amiloride)
• local application of gentamicin to destroy vestibular end-organ
• surgical - selective vestibular neurectomy or transtympanic labyrinthectomy
• may recur in opposite ear after treatment
Recurrent Vestibulopathy
❏ peak age 30-50 years old, M = F
❏ episodic vertigo lasting hours to minutes
❏ no hearing loss, tinnitus, or focal neurological deficit
❏ etiology unknown (?post-traumatic, ?post-viral, ?deafferentation of CN VIII)
❏ treatment: symptomatic, most eventually go into remission
Vestibular Neuronitis
❏ severe vertigo with nausea, vomiting, and inability to stand or walk
❏ symptoms can last for 3 to 4 days (risk of dehydration from vomiting)
❏ attacks leave patient with unsteadiness and imbalance for months
❏ repeated attacks can occur
❏ unknown etiology (microvascular upset due to infection, autoimmune process, or a metabolic disorder)
Labyrinthitis
❏ sudden onset of vertigo, nausea, vomiting, whistling tinnitus, and unilateral hearing loss,
with no associated fever or pain
❏ lasts for days
❏ may occur through spread from a cholesteatomic fistula or throughdirect infection after a transverse fracture of
the temporal bone or post-operative infection
❏ treat with IV antibiotics, drainage of middle ear +/– mastoidectomy
❏ beware of meningeal extension and labyrinth destruction

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 TINNITUS
❏ an auditory perception in the absence of stimulation, often very annoying to the patient
❏ etiology
• presbycusis (most common cause in elderly)
• serous otitis media (most common cause in young)
• Meniere's Disease
• acoustic trauma
• labyrinthitis = acoustic neuronitis
• Acoustic Neuroma
• multiple sclerosis (MS)
• drugs (NSAIDs, salicylates, aminoglycosides, antimalarials, caffeine, alcohol)
❏ pulsatile (objective) tinnitus (rare)
• bruits due to vascular lesions (e.g. glomus jugulare, hemangiomas, carotid body tumours, AVM, internal
carotid artery bruits)
• patulous eustachian tube
❏ clicking tinnitus
• myoclonus of muscles - stapedius, tensor tympani, levator and tensor palati
• tetany
Treatment
❏ avoid loud noise to prevent worsening of symptoms
❏ mask tinnitus
• white noise masking devices
• hearing aid
• music earphones
❏ tinnitus workshops
❏ psychotherapy
❏ trial of tocainamide

OTALGIA
Local Causes
❏ furuncle (boil), usually as a result of a Staph Aureus infection
❏ foreign body in external auditory canal/impacted cerumen
❏ infection
• otitis externa
• acute otitis media and its complications
• acute mastoiditis and its complications
❏ trauma to tympanic membrane and canal
❏ barotrauma
Referred (10 T's + 2)
❏ CN V and CN X refer to external canal and CN IX to middle ear
1) eustachian Tube
2) TMJ (tempromandibular joint) syndrome
3) Trismus (i.e. pterygoids, quinsy)
4) Teeth - impacted
5) Tongue
6) Tonsillitis, tonsillar cancer, post tonsillectomy
7) Tic (CN IX) - glossopharyngeal neuralgia
8) Throat - cancer of larynx, vallecula, pyriform fossa
9) Trachea - foreign body, tracheitis
10) Thyroiditis
11) Geniculate herpes and Ramsey Hunt Syndrome
12) +/– CN VII palsy

OT18 – Otolaryngology MCCQE 2002 Review Notes

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 NEUROLOGICAL Pathology:
EPILEPSY

EPILEPSY:
- Terminology:
o Epileps A Recurrent Spontaneous Seizure Activity, NOT Attributable to External Cause.
(Clinical Dx Depends on the an arbitrary cutoff, usually 3/more seizures)
o Sei res Spontaneous Abnormal Electrical Activity within the Brain.
- Aetiology:
o 70% Idiopathic (Often Familial)
o Others: Post-Injury, Developmental, Tumour, Stroke, Febrile Convulsion, Trauma, Stroke, ICP,
Alcohol Withdrawal, Metabolic, Infection, Drugs.
- Common Triggers (Among Epileptics):
o **Strobe Lights are most common (Often used for Diagnosis)
- Common Triggers (Among Non-Epileptics):
o Drug/Caffeine OD
o Fever
o Alcohol Withdrawal
o Toxins
o Head Injury
o Metabolic/Electrolyte Disturbances
o NB: The above triggers have to be eliminated before Epilepsy is Diagnosed.
(Epilepsy is an ‘Innocent until proven guilty’ disease.)
o NB Sei re Epileps
- Pathogenesis:
o Hyperexcitable Neurons (Lower Threshold, Ion-Channelopathy, or Neurotransmitter Imbalance)
Inappropriate, uncontrolled, spontaneous Electrical Activity within the Brain (Seizure)
- Clinical Features:
o Prevalence: 0.5 - 1% of Adults
o Age of Onset:
Generally before 20yrs.
1st seizure before 10yrs
o Presentation:
1. Pre-Sei re Aura Eg. Deja-vu, Abdominal Discomfort, Flashing Lights, Strange Smells,
Sounds, Tastes.
2. Seizure Many Different Types
3. Post-Ictal Symptoms: Eg. Headache, Confusion, Myalgia, Temporary Weakness
- Diagnosis: a Clinical Diagnosis; Requiring:
o >2 Seizures, for which all external triggers have been eliminated.
o Positive EEG
o Seizure Induction Test
o (+ Detailed History)
o (+ Detailed Description (or video) of the Seizures)
o (No single test is enough to diagnose.)
o NB: 1x Seizure Epilepsy.
- Treatment:

(NB: Valproate “Epilim”; Carbamazepine “Tegretol”)

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Types of Seizures:
- ICES-Classified Seizures:
o Simple Par ial Sei re (Conscious & Localised):
Symptoms:
Typically – Small, Rapid Muscle Movements
May include - Focal Motor/Sensory/Autonomic/Psychic Symptoms
Duration: Very Short Duration (Less than 1min)
NB: Preservation of Consciousness & Memory is Key.
o Comple Par ial Sei re (ALOC & Localised):
Symptoms:
‘Impaired Consciousness’ Dazed/Dopey.
+ Purposeless Movements – (Hand-Wring/Pill-Rolling/Face-Washing)
Duration: Less than 2 min
NB: Impaired Consciousness Little/No Memory of Seizure.
o Par ial i h Secondar Comple -Generalised Sei re - Tonic-Clonic
Ie. Simple or Complex Partial Seizure, Progressing to Complex (Unconscious) Widespread
(Generalised) Seizure.
4 Phases:
1. Pre-Seizure Period (Aura)
2. Tonic Phase (Sustained Generalised Tonic Contraction)
3. Clonic Phase (Repetitive Generalised Synchronous Jerks)
4. Post-Ictal Coma (Sustained Post-Seizure Unconsciousness)
(May include Central Apnoea & Incontinence)
Duration:
1-2mins
However, can last for many minutes.
- Unique Seizure Types:
o M oclonic
Symptoms:
Brief, Marked Contraction of Muscles (Ie. A “Shock-Like Jerk” or a “Startle”)
Typically Upper Body
Typically Bilateral
(May be in a specific muscle group/s)
Duration:
Typically 1-5sec
o Temporal Lobe Epileps
Symptoms: Typically Behavioural Alteration:
- Automatic Activity but Without Consciousness or Memory
- Sexually Inappropriate Behaviour
- Religiosity
- Aggression
- Relived Experiences
Duration:
Can last for hours
Treatment:
Carbamazepine (Tegretol)
o Absence Sei res - (The Classic Pe i Mal
Symptom:
Abrupt Onset of Impaired Consciousness + Amnesia
Pt appears to “Zone Out”
May Purposeless Movements (Eg. Lip Smacking, Eye Blinking)
Then Pt resumes Exactly where they left off (unaware of time lapse)
Duration:
Up to 30sec
Treatment:
Ethosuximide (Thalamic Ca-Channel Blocker)

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 o S a s Epilep ic s (Unremitting Seizure; or Multiple Successive Seizures):
= An Episode of Seizures of Any Type that Either:
1. Seizures Don’t Stop Spontaneously.
2. Seizures Occur in Rapid Succession Without Recovery.
A Status Epilepticus Seizure = Absolute Neurological Emergency:
High Risk of Cerebral Hypoxia
High Risk of Permanent Brain Damage
Often Results in Permanent Loss of Neurons due to Excito-Toxicity.
o (Hippocampus & Pyramidal Tracts are Particularly Sensitive Memory
& Motor)
Surviving Neurons may exhibit Synaptic Reorganisation.
The Problem = Cell Death:
Seizures can Trigger Cell Death; How?:
o In racell lar Ca+ from Ca-Mediated-NT-Release. Release of
Cytochrome-C from Mitochondria Triggers Apoptotic Pathway.
o Energy Depletion Free Radicals Widespread
Protein/Membrane/DNA Damage.
Also, Attempts made by the brain to Restore Function favour The Excitatory
Pathways Seizures.
Occurs mostly in the Young and the Elderly (Typically not middle-aged)
NB: Mortality is highest in Elderly Patients.
Average Mortality Rate 20
Treatment:
1st Line: Benzodiazepines (GABA-Channel Agonist)
o *Diazepam – (Generally #1; But Short Acting)
o Lorazepam – (Some argue that it’s #1 due to Higher Seizure-Termination
Rate)
o Midazolam
+/- Phenytoin – As an Adj nc o Ben os (Usage Dependent VG-Na Channel
Blocker):
o (Unless Absence Seizures or TLE)

- Treatment - Anti-Convulsants:
• The Jack of All Trades (Valproate):
• 3 Mechanisms of Action - (Na+ Channel Blocker, Ca+ Channel Blocker & GABA Activator):
• Repetitive Firing of Neurons.
• Prevents Spread of Signals from Epileptic Focus.
• General Neuronal Inhibition of the Brain.
• ALL Seizure Types
• VG-Na+ Channel Blockers (Carbamazepine[Tegretol], Lamotrigine, Phenytoin):
• Use Dependent VG-Na+ Channel Blockers
• Useful in All Seizures EXCEPT Absence Seizures
• VG-T-Ca+ Channel Blockers (Ethosuximide):
• Blocks VG-T-Ca+ Channels in the Thalamus Prevents Propagation of Seizure Activity.
• Used ONLY in Absence Seizures
• GABA Channel Modulators (Benzodiazepines - Diazepam):
• Activate GABA Channel Cl- Influx Hyperpolarises & :. Stabilises Neuron.
• Useful in All Seizures EXCEPT Absence Seizures
• NB: Benzodiazepines (Diazepam) = 1ST LINE FOR STATUS EPILEPTICUS
• GABA Analogues (Gabapentin):
• Activate GABA Channel Cl- Influx Hyperpolarises & :. Stabilises Neuron.
• Useful in Partial Seizures

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 Surgical Interventions:

Why Surgery:
- Up to 30% of Epilepsies are Unresponsive to Pharmacological Treatment
- If the Epilepsy is Unresponsive to drugs, Surgery is Essential to prevent Permanent Progressive Brain
Damage
o NB: Risk of Brain Damage Increases the longer the condition continues.
o NB: Seizures bring about More Seizures. (Ie. Untreated Seizures make Future Seizures more Likely)

Surgical Options:
- 1. Resections:
o Removal of Epileptic Focus.
o Hemispherectomy (Removal of an entire Hemisphere)
o Anteromedial Temporal Lobectomy
- 2. Disconnections:
o Cut the Corpus Callosum (Bridge between Hemispheres)
o Multiple Sub-Pial Transections (Small cuts made into cortex hoping to isolate neuronal networks)
Prognosis:
- 0 of Surgery Patients are Seizure-Free 10yrs later.
- (NB: Precise mapping of the Epileptic Focus is an Essential Prerequisite to Surgery to ensure that removal
won’t render the patient Paralysed/Unable to Speak/Other Serious Deficit.)

Dietary Intervention: The Ketogenic Diet:

What is the Ketogenic Diet?

- 1gram/KgBody-Weight of Protein.
- 5-10grams of Carbs/Day. (Ie. Virtually NO Carbs)
- Remainder of Calories is made up in Fats.
- NB: Side Effect – Bloating & Constipation (Lack of Fibre).

Proposed Mechanism/s of Action:

- GABA Availability:
o Through Metabolic Conversion of Ketone Bodies GABA.
GABA Availability General Inhibition of Neuronal Activity.
- Altered Metabolic Activity:
o Protein & Carbs Forced Re-Adaptation of Energy-Utilisation Glutamate Availability.
Glutamate Availability Decreased Stimulation of Neuronal Activity.
- Ac i i of Na K-ATPase:
o Drives Neurons Away from Threshold Hyperpolarises.

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 DIAGNOSTIC INVESTIGATIONS . . . CONT.

Contraindications
❏ signs and symptoms of increased ICP (papilledema, decreased LOC, progressive deficit, headache)
due to mass lesion
• do CT first and then proceed to lumbar puncture (LP) if there is no shift
❏ neurologic findings suggestive of localized mass lesion
❏ obstructive hydrocephalus, or evidence of blood
❏ infection at LP site
❏ coagulopathy (e.g. anticoagulatn drugs) or thrombocytopenia
❏ developmental abnormality (i.e. tethered spinal cord)
Diagnostic Tests
❏ opening pressure, protein, glucose, cell counts, colour, VDRL, viral PCR, IgG levels, oligoclonal bands,
fungal antigens, microbiological stains (Gram, ZN, fungal), bacterial culture and PCR
Typical
❏ see CSF Findings in CNS Infections section
Complications
❏ most common is bifrontal or generalized headache (10-40%)
❏ tonsillar herniation
❏ infection
❏ spinal epidural hematoma

SEIZURE DISORDERS AND EPILEPSY

Definitions
❏ a seizure is a paroxysmal alteration of behavior and/or EEG changes that results from abnormal
and excessive activity of cerebral neurons
❏ epilepsy is a condition characterized by a tendency to have recurrent, unprovoked seizures

CLASSIFICATION OF SEIZURES

Seizures

Partial (focal) Seizures Generalized Seizures

Absence
Typical
Simple Complex Partial Atypical
Partial Seizures Partial Seizures
(without altered LOC) (with altered LOC) evolving to Clonic
2º generalized
seizure Myoclonic

Tonic
with with with Autonomic with Psychic
Motor Somatosenory Signs Symptoms Tonic clonic
Signs or Special
Sensory Signs Atonic

Unclassified

Figure 11. Classification of Seizures

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 SEIZURE DISORDERS AND EPILEPSY . . . CONT.
CLINICAL APPROACH TO SEIZURES
History
❏ age of onset: primary generalized seizures rarely begin < 3 or > 20 years of age
❏ precipitants: sleep deprivation, drugs, EtOH, TV screen, strobe, emotional upset
❏ presence of aura: implies focal onset
❏ patient’s responsiveness during attack
❏ nature of neurological features suggests location of focus
• motor = frontal lobe
• visual/olfactory/gustatory hallucinations = temporal lobe
❏ salivation, cyanosis, tongue biting, incontinence
❏ Jacksonian march: one body part is initially affected, followed by spread to other areas
(e.g. fingers to hands to arm to face)
❏ adversive: head or eyes are turned forcibly to the contralateral frontal eye field
❏ automatisms: patterns of repetitive activities that look purposeful, (e.g. chewing, walking, lip-smacking)
❏ temporal lobe epilepsy: unilateral posturing, behavioral disturbances, automatisms, olfactory or gustatory
hallucinatons
❏ post-ictal symptoms - limb pains, tongue soreness, headache, drowsiness, Todd’s paralysis (hemiparesis)
❏ duration: ictus is short (seconds - minutes), post-ictus can be long (minutes - hours)
❏ family history of seizures
❏ past history of neurological insult: birth injury, head trauma, stroke, CNS infection, drug use/abuse
❏ fever: febrile seizures affect 4% of children between 3 months and 5 years of age, benign if brief solitary,
generalized tonic clonic lasting less than 15 minutes and not more frequent than once/24 hours
Clinical Pearl
❏ Stroke is the most common cause of late-onset (> 50 years of age) epilepsy, accounting
for 50-80% of cases.

Physical Examination
❏ pulse (especially rhythm), BP, heart auscultation
❏ complete neurological examination (CN, motor, reflexes, tone, sensory, coordination, mini mental exam)
❏ absence seizures can be precipitated by hyperventilation: have patient take up to 100 deep breaths and
watch for a brief, transient cessation of activity and “glassy stare”
❏ asymmetry of fingernail, toe, and limb size (clue to early damage of contralateral hemisphere)
❏ arteriovenus malformations (AVM’s) may present as focal seizures:
auscultate for bruits (carotid, orbital, cranial, spinal), visual fields, optic fundi
❏ head exam for evidence of trauma (look, then feel)
❏ skin exam: look for characteristic lesions of neurocutaneous syndromes
(neurofibromatosis (NF), tuberous sclerosis complex, Sturge-Weber syndrome)
Investigations
❏ CBC, sodium, glucose, calcium, magnesium, creatinine, urea, LFTs
❏ CXR, ECG
❏ EEG (symmetric bursts of sharp and slow, 4-7Hz in primarily generalized
tonic clonic, focal epileptiform in secondarily generalized, spikes and slow
waves at 3/second in absence) - interictal EEG is normal in 60% of cases
❏ increased prolactin level with generalized tonic-clonic seizures
❏ CT / MRI except for definite primary generalized epilepsy
❏ LP if signs of infection and no papilledema or midline shift of brain
structures (generally done after CT or MRI, unless suspicious of meningitis)
Etiology
❏ generalized
• idiopathic (family history in up to 40% of cases)
• diffuse cerebral damage (encephalitis, anoxia, storage diseases)
• metabolic (hypocalcemia, hypoglycemia, hyponatremia, porphyria,
hypoxia, renal failure, hepatic failure)
• drugs (EtOH withdrawal, TCAs, MAOIs, neuroleptics, cocaine, amphetamines)
❏ partial (focal)
• cerebral trauma
• birth injury
• vascular (cerebral hemorrhage, cortical infarcts, AVM, cavernoma)
• cerebral tumours
• infections (meningitis, encephalitis, cerebral abscess, subdural empyema, syphilis, TB, HIV)
• inflammation (sarcoidosis, SLE)

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 SEIZURE DISORDERS AND EPILEPSY . . . CONT.
DDx
❏ syncope
• causes
• neurogenic vasodepressor and vasovagal reaction
• sympathetic nervous system (SNS) failure
• decreased cardiac output (CO) or inadequate intravascular volume
• others (e.g. hypoxia, anemia)
• NOTE: syncope may induce a seizure – this is not epilepsy
❏ pseudoseizure
• can be impossible to differentiate without EEG
• often occur in conjunction with epilepsy
• history of sexual abuse
• patterned after witnessed seizure (i.e. health care worker, sibling or friend with seizures)
• see Table 5
❏ narcolepsy (cataplexy)
❏ migraine: associated with sensory or motor symptoms or vertebrobasilar migraine
❏ anxiety: hyperventilation, panic attacks
❏ transient ischemic attack
❏ hypoglycemia
❏ pheochromocytoma
Table 4. Seizures versus Syncope
Characteristic Seizure Syncope
Time of onset Day or night Day
Position Any Upright, not recumbent
Onset Sudden or brief aura Gradual (vasodepressor)
Aura Possible specific aura Dizziness, visual blurring, lightheadedness
Colour Normal or cyanotic (tonic-clonic) Pallor
Autonomic features Uncommon outside of ictus Common
Duration Brief or prolonged Brief
Urinary incontinence Common Rare
Disorientation, post-ictal Can occur with tonic-clonic, Rare
complex partial
Motor activity Can occur Occasional brief tonic
seizure or clonic jerks
Injury Common Rare
Automatisms Can occur with absence or None
complex partial
EEG Frequently abnormal, Normal
may be normal

Table 5. Seizures versus Pseudoseizures (non-epileptic "seizures")

Characteristic Pseudoseizure Epileptic Seizure
Age Any, less common in the elderly Any
F>>M F=M
Triggers Emotional disturbance Uncommon
Duration May be prolonged Brief
Motor activity Opisthotonus Automatisms in complex
Rigidity Partial seizures
Forced eye closure Stereotypic
Irregular extremity movements Synchronous movements
Side-to-side head movements
Pelvic thrusting
Crying
Timing Usually day; usually present other people Day or night

Physical injury Non-serious and only witnessed May occur

Urinary incontinence Rare May occur
Reproduction of attack Suggestion above or stimuli plus suggestion Spontaneous

EEG normal ictal and post-ictal patterns Inter-ictal discharges frequent

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 SEIZURE DISORDERS AND EPILEPSY . . . CONT.
TYPES OF SEIZURES
Simple Motor
❏ arise in precentral gyrus (motor cortex), affecting contralateral face/trunk/limbs
❏ ictus
• no change in consciousness
• rhythmical jerking or sustained spasm of affected parts (i.e. clonus)
• characterized by forceful turning of eyes and head to side opposite the discharging focus
(adversive seizures)
• may start in one part and spread “up/down the cortex” (Jacksonian march - remember the homunculus)
• duration from seconds to hours (which may result in Todd’s paralysis for hours)
Simple Sensory
❏ somatosensory
• arise in sensory cortex (postcentral gyrus), affecting contralateral face/trunk/limbs
• numbness/tingling/“electric” sensation of affected parts
• a “march” may occur
❏ other forms include: visual, auditory, olfactory, gustatory, vertiginous (may resemble
schizophrenic hallucinations but patients recognize the unreality of phenomena)
Clinical Pearl
❏ Motor and/or sensory partial seizures indicate structural disease until proven otherwise.
Simple Autonomic
❏ symptoms/signs include: epigastric sensation, pallor, sweating, flushing, piloerection and pupillary dilatation
Simple Psychic
❏ disturbance of higher cerebral function
❏ symptoms rarely occur without impairment of consciousness and are much more commonly experienced as
complex partial seizures
Complex Partial (Temporal Lobe Epilepsy, Psychomotor Epilepsy)
❏ often incorrectly called “petit mal” by patients
❏ seizures causing alterations of mood, memory, perception
❏ common form of epilepsy, with increased incidence in adolescents, young adults
❏ ictus
• aura of seconds-minutes; forms include:
dysphasic, dysmnesic (déjà vu, jamais vu), cognitive (dreamy states, distortions of time sense),
affective (fear, anger), illusions (macropsia or micropsia), structured hallucinations
(music, scenes, taste, smells), epigastric fullness
• then patient appears distant, staring, unresponsive (can be brief and confused with absence seizures)
• automatisms occur in 90% of patients (chewing, swallowing, lip-smacking, scratching, fumbling, running,
disrobing, continuing any complex act initiated prior to loss of consciousness)
❏ recovery is characterized by confusion +/– headache
❏ can resemble schizophrenia, psychotic depression (if complex partial status)
Generalized Tonic-Clonic (Grand Mal)
❏ common
❏ all of the classic features do not necessarily occur every time
❏ prodrome of unease, irritability hours-days before attack
❏ ictus
• aura (if secondary generalized from a partial onset) of olfactory hallucinations, epigastric discomfort,
déjà vu, jerking of a limb, etc. seconds-minutes before attack
• tonic phase: tonic contraction of muscles, with arms flexed and adducted, legs extended, respiratory
muscles in spasm (“cry” as air expelled), cyanosis, pupillary dilatation, loss of consciousness,
patient often “thrown” to the ground); lasting 10-30 seconds
• clonic phase: clonus involving violent jerking of face and limbs, tongue biting, and incontinence;
lasting 1-5 minutes
• post-ictal phase of deep unconsciousness, with flaccid limbs and jaw, extensor plantar reflexes, loss of
corneal reflexes; lasts a few minutes to several hours; headache, confusion, aching muscles, sore
tongue, amnesia; serum CK elevated for hours
Absence (Petit Mal)
❏ relatively uncommon; onset in childhood
❏ hereditary
• autosomal dominant
• incomplete penetrance (~1/4 will get seizures, ~1/3 will have characteristic EEG findings)
• 3 Hz generalized spike and slow-wave activity on EEG
❏ ictus
• child will stop activity, stare, blink/roll eyes, be unresponsive; lasting
approximately 5-10 seconds or so, but may occur hundreds of times/day
• may be accompanied by myoclonus or akinetic/drop attacks
• may be induced by hyperventilation
❏ often associated with decreasing scholastic performance
❏ 1/3 “convert” to tonic-clonic in adolescence

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 SEIZURE DISORDERS AND EPILEPSY . . . CONT.
Myoclonic
❏ sudden, brief, generalized muscle contractions
❏ may be seen in association with absence and clonic-tonic-clonic seizures
❏ most common disorder is juvenile myoclonic epilepsy (benign, onset after puberty)
❏ also occurs in degenerative and metabolic disease (e.g. hypoxic encephalopathy)
Management
❏ psychosocial
• educate patients and family
• advise about swimming, boating, locked bathrooms, operating dangerous machinery,
climbing heights, chewing gum
• pregnancy issues: counseling and monitoring blood levels closely, teratogenicity of antiepileptic drugs,
folate 4-6 mg/day for 3 months prior to conception (throughout child-bearing years)
• inform of prohibition to drive and requirements to notify government
• support groups, Epilepsy Association
• follow-up visits to ensure compliance, evaluate changes in symptoms/seizure type (re-investigate)
❏ pharmacological
• begin with one major anticonvulsant with a simple dosage schedule (see Table 6)
• adjust dose to achieve plasma level in low therapeutic range
• if no seizure control, increase dose until maximum safe dose or side-effects become intolerable
• if no seizure control, change to or add second drug
• clonazepam: mostly used for refractory myoclonic seizures
• adjunctive therapy: clobazam (Frisium), gabapentin (Neurontin), vigabatrin (Sabril),
lamotrigine (Lamictal)
❏ surgical
• for selected cases of complex partial epilepsy with an identifiable focus

Table 6. Indications and Important Side-Effects of Major Antiepileptic Drugs

Drug Indication Major Side-Effects
Dose-Related Idiosyncratic

Carbamazepine Partial or generalized Diplopia Morbilliform rash

(Tegretol) tonic-clonic seizures Dizziness Agranulocytosis
Headache Aplastic anemia
Nausea Hepatotoxic effects
Drowsiness Stevens-Johnson syndrome
Neutropenia Teratogenicity
Hyponatremia
Phenytoin Partial or generalized Nystagmus Acne
(Dilantin) tonic-clonic seizures Ataxia Coarse facies
status epilepticus Nausea Hirsutism
Vomiting Blood dyscrasias
Gingival hyperplasia Lupus-like syndrome
Depression Rash
Drowsiness Stevens-Johnson syndrome
Paradoxical 8 in seizures Dupuytren’s contracture
Megaloblastic anemia Hepatotoxic effects
Teratogenicity
Valproate All generalized seizures Tremor Acute pancreatitis
(Epival, Depakene) or partial seizures Weight gain Hepatotoxic effects
Dyspepsia Thrombocytopenia
Nausea Encephalopathy
Vomiting Teratogenicity
Alopecia
Peripheral edema
Ethosuximide Absence seizures Nausea Rash
(Zarontin) Anorexia Erythema multiforme
Vomiting Stevens-Johnson syndrome
Agitation Lupus-like syndrome
Drowsiness Agranulocytosis
Headache Aplastic anemia
Lethargy

STATUS EPILEPTICUS
❏ a life-threatening state ( 5-10% ) with either a continuous seizures lasting at least 30 minutes
or a series of seizures occurring without the patient regaining full consciousness between attacks
❏ risks: repetitive grand mal seizures impair ventilation, resulting in anoxia, cerebral ischemia
and cerebral edema; sustained muscle contraction can lead to rhabdomyolysis and renal failure
❏ may result in excitotoxic damage

N12 – Neurology MCCQE 2002 Review Notes

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 SEIZURE DISORDERS AND EPILEPSY . . . CONT.
ABCs
❏ lateral semi-prone position, mandible pushed forward;
use oropharyngeal/endotracheal tube with high-flow oxygen
❏ monitor RR, HR, BP and temperature
Interrupt Status
❏ give 50 ml 50% glucose IV and thiamine 100 mg IM
❏ lorazepam IV 2-4 mg (0.05 mg/kg)
❏ set up IV infusion of phenytoin (15-18 mg/kg loading dose with maintenance started 12 hours later);
monitor BP and ECG during infusion
• phenobarbital if no response (watch for hypotension and respiratory depression)
• general anesthesia in ICU (e.g. pentothal) if no response to phenobarbital
❏ monitor lytes, glucose, urea, creatinine, lactate, myoglobin, blood gases, ECG
❏ midazolam drip (in ICU) or pentobarbital “coma”
Assess the Cause of the Status
❏ accucheck (rule out hypogycemia)
❏ draw metabolic and drug screen (most common is EtOH)
❏ measure anticonvulsant levels
❏ CXR, EEG and consider stat CT or MRI if first seizure or if focal neurological deficits elicited

ALTERED LEVEL OF CONSCIOUSNESS

APPROACH TO ALTERED LEVEL OF CONSCIOUSNESS (LOC)
Decreaed LOC

Structural Metabolic

Trauma H-hypoxia, hypoglycemia, hypercapnia

Abscess I-infection (meningitis, encephalitis)
Stroke T-toxins (street drugs, benzodiazepines, alcohol)
Bleed (eg. subarachnoid) M-MUDPILES (methanol, uremia, DKA, lactic acidosis, ethanol etc.)
Increased intracranial pressure E-endocrine (TSH, ACTH), electrolytes (hyponatriemia, hypocalcemia)
Tumor N-nutritional supplement (vit B12, thiamine)
Hypertension S-system failure (hepatic encephalopathy, heart failure)
Coma

COMA
Definition
❏ a state in which patients show no meaningful response to environmental stimuli
from which they cannot be aroused
Pathophysiology
❏ consciousness consists of 2 components
• arousal - alertness, sleep-wake cycle
• content - responding to external stimuli
• i.e. seeing, feeling, hearing, speaking
❏ consciousness requires
1. intact cerebral hemispheres
2. intact reticular activating system (RAS) in brainstem
❏ lesions diffusely affecting hemispheres or directly affecting the
RAS cause impairment of consciousness and potentially coma
❏ focal hemispheric damage does not alter consciousness except
by mass effect or by precipitating seizures
Classification
❏ structural lesions (tumour, pus, blood, CSF); (1/3 of all cases of coma)
• expanding supratentorial mass: causes transtentorial herniation, leading to brainstem compression
(and thus RAS dysfunction) or major shift (horizontal) with bilateral hemispheric dysfunction
• posterior fossa lesion: may directly destroy the neurons of the brainstem RAS
❏ metabolic disorders/diffuse hemispheric damage (2/3 of comas)
• deficiency of essential substrates, i.e. oxygen, glucose, vitamin B12
• endogenous/exogenous toxins, i.e. drugs, heavy metals, solvents
• systemic metabolic diseases, i.e. uremia, hepatic encephalopathy, electrolyte imbalances
MCCQE 2002 Review Notes Neurology – N13

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 SENSORY Pathology:
EQUILIBRIUM DISORDERS

Common Equilibrium Disorders:

- Vertigo:
o Hallucinatory sensation of movement (Referred to dizziness)
o Labyrinthitis or vestibular neuronitis (subsequent to viral/bacterial infection/metabolic disturbance
– eg. hypoglycaemia)
o Elderly patients – due to reduced blood supply to the labyrinth.
- Menie e S nd me
o Labyrinthine disorder – affects both semicircular canals & cochlea:
Repeated attacks of vertigo, nausea & vomiting
Tinnitus is common & hearing is impaired
- Positional Vertigo:
o May often follow trauma
o May follow drug overdose
o Anxiety & depression may contribute (psychogenic)
- Motion Sickness:
o Mismatch between visual & vestibular information.

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 NEUROLOGICAL Pathology:
GUILLIAN-BARRE SYNDROME

- Guillain-Barre Syndrome:
o Aetiology:
Post-Viral Autoimmune
An Acute Inflammatory Demyelinating Peripheral Polyneuropathy that occurs following
an Acute Viral Illness
o Pathogenesis:
Acute Viral Illness Triggers a Misdirected Immune Response
T-cell Mediated, Autoimmune Attack & Demyelination of Peripheral Nerves
Motor: Ascending Paralysis (Weakness beginning in Feet & Hands Migrating
toward the Trunk)
Sensory: A cending Parae he ia Proprioception & Areflexia (Altered Sensation
in Feet/Hands Trunk)
(NB: Distinct from Multiple Sclerosis since it does not affect the CNS)
o Clinical Features:
Hx of Recent Viral Illness
Acute Progresses over Hours Days
Rapid, Symmetrical Ascending Paralysis & Paraesthesia (Initially Distal Limbs only, then
Proximal Muscles)
Areflexia
NO Fever
Is Life threatening - Death usually due to respiratory paralysis
o Diagnosis:
LP CSF Pro ein
EMG (Electromyography) & Nerve Conduction Studies.
o Treatment:
Hospitalisation
**Ventilation
Prompt IV-Immunoglobulins Or Plasmapheresis Recovery

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 FOCAL AND MULTIFOCAL NEUROPATHY . . . CONT.
❏ diagnosis
•The 4 P’s of GBS
•Pain (back, flanks, hips, thighs)
•Parasthesia (toes and fingers)
•Paralysis (ascending pattern involving proximal muscles more than distal muscles)
•Palsies in 45-75% of cases (typically cranial nerves VII, IX-X)
•roughly symmetric weakness, often but not always in ascending pattern
(legs, up trunk to arms and face)
• rapidly progressive weakness with absent reflexes and little/no sensory changes is almost always GBS
• can progress to total muscle paralysis and death from respiratory failure
• autonomic disturbances 50% (labile BP, cardiac dysrhythmia, paralytic ileus, bladder dysfunction,
and abnormal sweating)
• rise in total protein in CSF by end of first week of symptoms, normal glucose, opening pressure,
and few or no cells (albumino-cytologic dissociation)
• electrophysiological studies most specific and sensitive diagnostic tool
• Miller-Fisher variant (5% of the GBS cases) characterized by areflexia, ataxia and ophthalmoplegia
❏ course
• monophasic course, with weakness progressing for several days to weeks, reaching a plateau,
and then recovering over a period of several weeks to months
• 10% of patients have lasting disability
• 3% of patients do not survive due to respiratory failure, a complication of immobility
such as pulmonary embolism (PE)
• 10% have a relapsing or fluctuating course
❏ treatment
• IV gamma globulin or plasmapheresis
• shortens disease course
• supportive management focuses on day-to-day concerns of respirators, vital signs (autonomic function),
nutrition, and other aspects of critical care
Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
❏ similar to GBS with following differences:
❏ uncommon to have preceding illness
❏ develops slowly, maximal severity after weeks or months
❏ more likely to have relapsing or fluctuating course
❏ motor weakness with areflexia
❏ increase in protein in CSF
❏ treatment: IVIG, steroids

AXONOPATHIES
❏ degeneration of the distal ends of long axons, usually causing an equal loss of all sensory modalities with
motor involvement in “stocking/glove" distribution occurring as sensory loss spreads more proximally
❏ associated with systemic disease
• DM, hypothyroidism
• uremia: 60% patients in end-stage renal failure, usually painless mild symmetrical distal weakness
• porphyria: may be proximal > distal and may have atypical proximal sensory deficits
• SLE, RA, PAN, scleroderma, amyloidosis, sarcoidosis
❏ alcohol-nutritional
• legs > arms
• sensory usually occurs before distal motor weakness
• “burning feet”
• small fiber (i.e. pain/temperature, absent ankle jerk)
❏ malignancy
• subacute motor neuropathy in lymphoma
• sensory motor neuropathy in lung carcinoma
• sensory in paraneoplastic small cell lung or breast carcinoma
❏ drugs
• usually dose-related: cisplatin, disulfiram, dapsone, antiretroviral, nitrofurantoin, ISH,
metronidazole, hydralazine
❏ toxins
• heavy metals, lead (motor, wrist drop)
❏ infection
• HIV, Lyme disease
NEURONOPATHIES
❏ affects cell body of sensory/motor nerves, causing acute/gradual onset of sensory and/or motor loss,
often no recovery
❏ classified as motor, sensory, autonomic
❏ associated with motor neuron diseases, herpes zoster neuritis, paraneoplastic sensory neuronopathy

N42 – Neurology MCCQE 2002 Review Notes

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 FOCAL AND MULTIFOCAL NEUROPATHY . . . CONT.
DIABETIC POLYNEUROPATHIES
❏ 15% of diabetics have symptoms of mixed, focal, multifocal, or polyneuropathies
• ~ 50% will have abnormal NCS with > 20 years DM
• decreased incidence with close glucose control
Symmetric Polyneuropathy Syndromes
❏ most common is distal symmetrical sensory polyneuropathy
• feet/legs > hands
❏ symmetrical proximal motor weakness (lower limbs > upper limbs) without pain
Focal or Multifocal Neuropathy Syndromes
❏ focal limb and truncal neuropathy: femoral or sciatic most common, good potential for recovery
❏ ophthalmoplegia: CN III (pupil sparing, severe eye pain), CN VI, CN IV
❏ multifocal neuropathy: pain in low back or hip spreading to thigh and knee (deep ache with superimposed
lancinating jabs, worse at night, self-limited, recovery in months to years), pathology localized to
lumbosacral plexus
Other
❏ autonomic neuropathy: orthostatic hypotension, gastroparesis, impotence, diarrhea, bladder dysfunction
❏ acute painful neuropathy: weight loss, intense foot pain, good response to glucose control

NEUROMUSCULAR JUNCTION (NMJ) DISORDERS

MYASTHENIA GRAVIS
Epidemiology
❏ bimodal age of onset: 20’s female > males / 60’s males > females
❏ thymoma in 10% of patients (more often in those aged 30-60)
❏ associated with other autoimmune diseases: IDDM, thyroid disease, vitiligo
Pathophysiology
❏ an autoimmune disease: production of antibodies to acetylcholine (ACh)
receptors at nicotinic post-synaptic neuromuscular junction
❏ decreased number of ACh receptors
Signs and Symptoms
❏ fluctuating course of muscle weakness, chiefly in muscles innervated by cranial nerves, causing ptosis,
diplopia, dysphagia and characteristically improved by cholinesterase-inhibiting drugs
❏ fatigability with use, relief with rest
❏ remitting and exacerbating course
❏ fatigability is the hallmark of diseases affecting the neuromuscular junction,
weakness that worsens with activity and improves with rest
Classification
❏ based on distribution of weakness
• generalized: proximal weakness (neck flexors, deltoids, hip flexors)
• ocular: ptosis, ophthalmoplegia
• bulbar: dysphagia, dysarthria
Diagnosis
❏ EMG shows muscle fatigability with repetitive stimulation
❏ single fiber-EMG: increased jitter (variability in the firing of individual muscle fibers of a motor unit)
❏ Tensilon test (edrophonium) with transient reversal of weakness within 30-60 seconds
❏ anti-acetylcholine receptor (AChR) antibodies (90% of generalized myasthenia
compared to 50% of pure ocular myasthenia have detectable serum antibodies)
❏ thymic hyperplasia/thymomas visualized by CT or MRI
Treatment
❏ anti-ACh inhibitors (increased ACh at receptor site) Mestinon, pyridostigmine
❏ immunosuppressive drugs to attack underlying process:
• steroids, azathioprine
❏ for acute crisis
• IVIG, plasmapheresis
❏ thymectomy if indicated (after CT of chest to assess for thymoma)

MCCQE 2002 Review Notes Neurology – N43

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 SENSORY Pathology:
HEARING DISORDERS

Common hearing deficiencies:

- Ageing:
o Progressive loss of hearing receptors
- Acute Damage:
o Hair cells can be destroyed by a single explosive sound or continuous high-intensity sound tears
at the cilia
- Drugs:
o Some are ototoxic (damage the hair cells)
- Tinnitus:
o Ringing in the ear in the absence of auditory stimulus
o Symptom of nerve degeneration/inflammation of middle/inner ear.
o Can be caused by drugs. (Damage can be permanent)
Some antibiotics (Streptomycin, neomycin)
Loop diuretics (transient)
Salycilates
- Otitis Media:
o Inflammation of middle ear lining
o Is a common result of throat infections in infants & children (due to short Eustachian tube).
o Can be bacterial viral or bacterial
o Eardrum becomes inflamed and bulges – can perforate
o When large amounts of fluid - pus accumulate behind the eardrum, grommets may be inserted.
- Deafness:
o Conduction Deafness:
Problem with Soundwave Conduction (Ie. Mechanical Structures)
Eg. Earwax
Eg. Perforated Ear Drum
Eg. Fused Ossicles
o Sensorineural Deafness:
Problem with Soundwave Transduction (Ie. Neural Structures)
Eg. Damaged Hair Cells
Eg. Damaged Cochlear Nerve
Eg. Damaged Auditory Cortex

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 NEUROLOGICAL Pathology:
HERPETIC NEURALGIA (SHINGLES)

- Herpetic Ne ralgia Shingles

o Aetiology:
Herpes Zoster Virus Infection in Neural Ganglia
o Pathogenesis:
Trigger (Stress/Sunlight/Immunocompromise) Reactivation of Latent HZV Infection in
Neural Ganglia HZV Migrates down the Axons Painful Vesicular Lesions in the Sensory
Dermatome (Often Trigeminal Nerve)
o Morphology:
Vesicular Lesions over Sensory Dermatome
o Clinical Features:
Initially just paraesthesia & burning pain over Sensory Dermatome
Then Painful Vesicular Lesions over Sensory Dermatome
o Treatment:
Antivirals:
Famciclovir Shortens course of disease.
Pain Management:
Antidepressants / Anticonvulsants (For Neuropathic Pain)
Topical Anaesthetics (Lidocaine Patches / Capsaicin Lotion)
Opioid Analgesics

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 NEUROLOGICAL Pathology:
HUNTINGTONS DISEASE

- Huntingtons Disease:
o Aetiology:
Genetic - Autosomal Dominant
Defective Huntungton Gene (Chromosome 4) – Excess CAG Tandem Repeats
Onset Age & Severity depends on # of CAG Repeats in mutation.

o Pathogenesis:
Excess CAG Tandem Repeats in Huntington Gene Production of Mutant Huntingtin
Proteins in the Brain Increases Decay Rate of Certain Types of Neurons
Selective Marked Degeneration of the Basal Ganglia (incl. The Striatum
[Caudate + Putamen], Globus Pallidus & Substantia Nigra).
o NB: Loss of Basal Ganglia Dysfunctional Action Selection Chorea
Also loss of Cortical Tissue as well (Dementia as well as chorea)

o Morphology:
Macro:
Atrophy of Basal Ganglia (Striatum [Caudate & Putamen], Globus Pallidus &
Substantia Nigra)
Some Atrophy of Cortical Tissue as well.
Compensatory Hydrocephalus of Lateral Ventricles (Lateral Ventricular Dilatation)
o Clinical Features:
Onset in 40’s (NB: The more CAG repeats, the younger the onset & faster the progression)
H n ing n T iad
Dementia (Intellectual Decline)
Depression
Coreiform Movement (Involuntary Jerking) Unsteady Gait
Late Stages:
Slurred speech
Difficulty swallowing.
o Treatment:
Incurable
Tetrabenazine, Neuroleptics, Benzodiazepines Can Chorea
o Prognosis:
<20yr life expectancy after Symptoms Begin.

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 MOVEMENT DISORDERS . . . CONT.
Investigation of Tremor
❏ all patients < age of 45 exhibiting tremor should be screened for Wilson’s disease (serum and urine copper
high, ceruloplasmin low); and have TSH (postural tremor) and CT/MRI (if cerebellar disease suspected)
performed
CHOREA
Definition
❏ involuntary, irregular, jerky movements; affect head and neck, face, shoulders commonly
• other manifestations include grimacing and respiratory sounds
Huntington’s Disease
❏ AD transmission (single gene defect on chromosome 4p), usual onset 40-60 years, age of onset inversely
correlated with number of CAG trinucleotide repeats present in gene on chromosome 4p
(i.e. paternal inheritance associated with expansion ––> earlier and more severe presentation)
❏ no cure; fatal 10-20 years after clinical onset
❏ pathology: atrophy of head of caudate nucleus and putamen bilaterally, moderate gyral atrophy in
frontal and temporal regions
❏ associated with decreased levels of GABA and ACh and decreased activity of glutamic acid decarboxylase and
choline acetyltransferase
❏ chorea: initially hands and face involved, seem fidgety, restless
❏ chorea eventually progresses to gross involuntary movement that interrupts voluntary movements
❏ slight alterations in character are often the first signs: irritable, impulsive, eccentric
❏ emotional disturbances: depression, less communicative, more socially withdrawn
❏ subcortical dementia
❏ diminished work performance, inability to manage responsibilities, sleep disturbances
❏ reduced memory and attentiveness, loss of fine manual skills,
tongue cannot be held protruded, increased frequency of blinking
❏ dysarthric and explosive speech
❏ later appearance of akinetic-rigid states
❏ diagnosis: clinical plus family history, DNA testing available, CT
(atrophy of caudate), MRI (increased signal of caudate in T2)
❏ genetic counseling extremely important
❏ distinguish from benign hereditary chorea and senile chorea, which is a diagnosis of exclusion
❏ treatment: haloperidol most effective for suppressing movement disorder, but increased postural instability
Other Types of Chorea
❏ Wilson’s disease: AR disorder of copper metabolism that produces neurological and hepatic dysfunction
(corneal Kayser-Fleischer rings and copper deposition in liver)
❏ Sydenham’s chorea: primarily a complication of previous Group A ß-hemolytic Streptococcus infection,
acute onset and remits in weeks
❏ chorea gravidarum: acute onset during pregnancy
(many related to SLE and/or antiphospholipid antibody syndrome)
❏ SLE
❏ drugs (tardive chorea): L-dopa, amphetamine, oral contraceptives
❏ senile chorea: no dementia, older age of onset
❏ benign hereditary: AD with incomplete penetrance, childhood onset, intellect preserved, mild,
rarely progressive
Hemiballismus
❏ unilateral, large amplitude flinging of the limbs, especially in proximal limb muscles
❏ lesion in contralateral subthalamic nucleus or its neuronal projections
❏ usually self-limited, resolving in 6-8 weeks
❏ most common cause is stroke (PCA territory)
❏ neuroleptics are often effective for symptomatic treatment
DYSTONIA
❏ sustained co-contraction of agonist and antagonist muscles which distort the limbs, trunk or face
into characteristic postures
❏ focal dystonia - disturbance restricted to localized muscle groups, e.g. writer’s cramp
❏ spasmodic torticollis – unilateral deviation of the head
• geste antagoniste – patient uses finger pressure to turn head to neutral position
❏ idiopathic torsion dystonia
• childhood onset, sporadic or dominant inheritance
• initially intermittent progressing to constant disabling generalized dystonia
❏ abnormal movements are not present during sleep, and are enhanced by emotional stress
and voluntary activity
❏ perinatal anoxia, birth trauma and kernicterus are common causes
❏ treatment
• often unsatisfactory
• anticholinergics and botulinum toxin injection, surgery

N24 – Neurology MCCQE 2002 Review Notes

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 NEUROLOGICAL Pathology:
INTRACRANIAL HAEMORRHAGES

INTRACRANIAL HAEMORRHAGES:
- Aetiologies:
o Trauma:
Eg. Skull Fracture Extradural Haemorrhage (Arterial)
Eg. Low-Force Trauma Subdural Haemorrhage (Venous)
o Congenital Vascular Conditions:
Eg. Congenital Berry Aneurysms Rupture Subarachnoid Haemorrhage (Arterial)
Eg. Congenital AV Malformations Rupture Intracerebral Haemorrhage (Arterial)
o Hypertension:
Hypertensive Intracerebral Haemorrhage (Arterial)

EPIDURAL/EXTRADURAL HAEMORRHAGE:
- Aetiology:
o Trauma/Cranial Fracture Arterial Rupture Separation of Dura from the Skull Haematoma
- Pathogenesis:
o High pressure bleed Forced Splitting of the Dura Mata In ac anial P e e
- Morphology:
o Dura Mata gets separated from the skull
o Extent of Bleeding is Limited by Attached Dura, :. Clearly Defined Margin.
o Lens-shaped area
o Brain Underneath is Compressed
- Clinical Features:
o Severe headache, vomiting and altered consciousness.
o Course:
Rapid progression (due to arterial source of blood)
1. Acute Loss of Consciousness
2. Then Lucid Interval (Temporary Improvement)
3. Then Sudden Deterioration if Herniation (Vomiting, Ipsilateral Pupil Dilation, LOC)
o Signs:
Fixed & Dilated Pupil on side of injury.
Eye on side of injury may be down & out (CNIII Palsy)
Contralateral Weakness of Extremities
Contralateral Homonymous Hemianopsia (Loss of Contralateral Visual Field)
If ICP Cerebellar Tonsillar/Uncal Herniation Respiratory Arrest
- Investigations:
o Head CT (Biconvex Lens Appearance)
- Management:
o Good prognosis with Surgery (Burrhole Craniotomy Drainage)
Will C ne Die without surgery

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SUBDURAL HAEMATOMA (typically a venous bleed; happens slowly)
- Aetiology:
o Elderly + Low force trauma (Eg. Whiplash Injuries) Slow Venous Bleed
- Pathogenesis:
o Bleeding between the Dura Mata and the Arachnoid Mater
- Morphology:
o Wide distribution ie. Over the entire hemisphere
o Cerebral Oedema Flattening of the gyri, narrowing of sulci, shift of midline
o Abnormal Brain underneath (Ie. Pt. Is often demented, or alcoholic, or have severe cerebral
atrophy)
- Clinical Features:
o Acute Subdural Haematomas:
Typically Due to Mild Trauma
Acute Neurologic Dysfunction (Within Minutes)
High Mortality Rate
o Chronic Subdural Haematomas:
Typically a Spontaneous, Slow Venous bleed (Days Weeks)
Gradual headache, Somnolence, Confusion, Focal Deficits, Seizures.
Common in Elderly.
Signs/Symptoms within Days-Weeks.
o Signs/Symptoms:
Gradually Increasing Headache and Confusion
Dizziness/Tinnitus/Numbness
Blurred Vision
Disorientation/Amnesia
Weakness/Lethargy/Ataxia
Nausea/Vomiting/Anorexia
Irritability/Seizures
- Investigations:
o Head CT:
Acute Subdural = Crescent-Shaped Density.
Can compress lateral ventricle and cause midline shift
Chronic Subdural = Bleeding has Spread Throughout the Subdural Space Follows the
curve of the brain.
- Management:
o If Severe Bleed: D ill D ainage f Bl d ICP
o If Small Bleed: Conservative Management and Monitoring
- Prognosis:
o Brain is typically Abnormal Underneath, Therefore the prognosis is worse

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Subarachnoid Haemorrhage
- Aetiology:
o Berry Aneurysm Rupture in Circle of Willis
o Hypertension is a Contributing Factor
- Pathogenesis:
o #Congenital Berry Aneurysm (Rupture of Saccular Aneurysm on circle of willis)
MCA is Commonest, then ACA, then PCA rare

- Morphology:
o Blood in the sulci
o Blood pools around the Basal Cistern of the brain
- Clinical Features:
o (Pre-Rupture):
Fatigue, Loss of perception/balance, Dysphasia
o Post-Rupture:
Thunderclap Headache Sudden, Severe, Pulsating Headache
+ Vomiting
+ Meningism
+ Hemiparesis
+ Diplopia
Followed by Confusion Loss of Consciousness (+/- Seizures)
- Specific Investigations:
o Head CT/MRI: Blood Within the Sulci & Fissures
o Lumbar Puncture: Blood in CSF
o CT Angiography: To Identify Aneurysms
- Management:
o Stabilise Patient (Ie. Intubate/Ventilation, ICU Admission)
o Urgent Neurosurgical Consult & Intervention
o Prevent/Rx ICP
- Prognosis:
o <50% are Fatal.

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 STROKE
❏ a clinical syndrome characterized by sudden onset of a focal neurological deficit presumed to be on a
vascular basis; avoid ‘CVA’ (‘confused vascular assessment’)
CLASSIFICATION
Ischemic Stroke (80%)
❏ ischemic stroke results from focal ischemia leading to cerebral infarction. Mechanisms include embolism
from heart or proximal arteries, small vessel thrombosis, or hemodynamic from a drop in the local perfusion
pressure. Global ischemia (e.g. from cardiac arrest or hypotension) causes a diffuse encephalopathy.
❏ ischemic strokes vary according to their size, anatomical location in the brain, and temporal pattern
Hemorrhagic Stroke (20%)
❏ abrupt onset with focal neurological deficits, due to spontaneous (non-traumatic) bleeding into the brain
❏ includes ICH and SAH
❏ subdural and extradural hemorrhages are not usually classified as strokes as they are associated with trauma
❏ hemorrhage into an area of cerebral infarction (commonly following cardiogenic embolism) is a hemorrhagic
infarct which should be considered an ischemic stroke complicated by secondary hemorrhage;
not a hemorrhagic stroke
STROKE TERMINOLOGY
Transient Ischemic Attack (TIA)
❏ stroke syndrome with neurological symptoms lasting from a few minutes to as much as 24 hours,
followed by complete functional recovery
Amaurosis Fugax, Transient Monocular Blindness (TMB)
❏ due to episodic retinal ischemia, usually associated with ipsilateral carotid artery stenosis or embolism of the
retinal arteries resulting in a sudden, and frequently complete, transient loss of vision in one eye
Reversible Ischemic Neurological Deficit (RIND)/Minor Stroke
❏ neurological abnormalities similar to acute completed stroke, but the deficit disappears after 24 - 36 hours,
leaving few or no detectable neurological sequelae (a better term is minor stroke)
Completed Stroke (CS)
❏ stroke syndrome with a persisting neurological deficit suggesting cerebral infarction; the ensuing neurological
defect can last days, weeks, or permanently; even after maximal recovery, at least minimal neurological
difficulties often remain
Progressing Stroke (Stroke In Evolution)
❏ neurological deficits begin in a focal or restricted distribution but over the ensuing hours spread gradually in a
pattern reflecting involvement of more and more of the particular vascular territory

MAKING THE COMPLETE DIAGNOSIS: “THE FOUR QUESTIONS”

❏ 1. Has the patient had a stroke?
• not all acute focal neurological deficits are 2º to stroke
• temporal profile may differentiate between TIAs, progressing stroke,
and minor and severe completed stroke
❏ 2. Where is the lesion and what is the blood supply?
• vascular territory: carotid vs. vertebrobasilar
❏ 3. What is the lesion?
• ischemia/infarction (with or without 2º hemorrhage)
• hemorrhage
❏ 4. What is the pathogenesis? (i.e. mechanism of the stroke)
• it will guide acute and chronic therapy

DDx: IS IT A STROKE?
❏ focal seizures
❏ other focal lesions: tumours, abscesses, subdural hematoma, demyelination, focal encephalitis
(herpes simplex)
❏ LMN lesions: Bell's Palsy, plexopathies, mononeuropathy
❏ previous cerebral infarction (i.e. focal signs are old)
❏ confusion, dementia and coma (without focal signs) are rarely modes of presentation for strokes
and usually suggests diffuse disturbance of cerebral function

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 STROKE . . . CONT.
WHERE IS THE LESION?
❏ see Figure 17 for vascular territories of major cerebral arteries
Hemispheric (see Figure 17 and Table 18)
❏ carotid territory (ACA, MCA)
❏ posterior cerebral arteries (vertebrobasilar supplies)

anterior cerebral artery

anterior cerebral artery

middle cerebral artery
middle cerebral artery

lenticulostriate
lenticulostriate arteries
arteries

anterior choroidal artery

posterior cerebral artery anterior choroidal artery

posterior cerebral artery

Figure 17. Vascular Territories of Major Cerebral Arteries

Illustration by Dr. P. Stewart
Table 18. Anterior vs. Middle Cerebral Arteries vs. Posterior Cerebral Arteries
* UE = upper extremities, LE = lower extremities

Anterior Cerebral Artery Middle Cerebral Artery Posterior Cerebral Artery

• Hemiplegia of LE • Hemiplegia of UE and face • Homonymous hemianopia or cortical
• Hemianesthesia of LE • Hemianesthesia of UE and face blindness (if bilateral)
• Incontinence • Hemianopia • If dominant hemisphere
• Grasp, snout, palmomental reflexes • Aphasia (if dominant hemisphere) - alexia without agraphia
• Behavioural and memory disturbances • Neglect of contralateral limbs • If thalamus
and constructional apraxia (if non-dominant hemisphere) - contralateral hemisensory loss
(if non-dominant hemisphere) - spontaneous pain
• Gaze preference (away from hemiparesis) • If subthalamic
- hemiballismus
• If midbrain
- ipsilateral CN III palsy
- contralateral motor deficit

Brainstem
❏ vertebrobasilar territory
• cranial nerves
• diplopia, gaze palsies, nystagmus
• vertigo
• dysarthria and dysphagia (sometimes hemispheric if patient hemiplegic)
• other cranial nerve palsies (III-XII)
• cerebellum
• ataxia
• incoordination
• crossed sensory loss (face and opposite side of body)
• bilateral motor deficits
Indeterminate
❏ ‘hemisyndromes’: hemiparesis, hemisensory loss, dysarthria
WHAT IS THE LESION?
Table 19. Hemorrhagic vs. Ischemic Stroke
Hemorrhage Infarct
Hypertension Usually present Often present
Preceding TIA No 30% of cases
Onset Often with activity Often at night or no activity, on waking
Course Rapidly progressive Static (rarely stepwise)
Increased ICP Yes No
CT scan Shows blood Normal or changes of infarction
• CT (or MRI) is the only reliable way to rule out hemorrhage
N50 – Neurology MCCQE 2002 Review Notes

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 STROKE . . . CONT.
WHAT IS THE PATHOGENESIS?
Atherosclerotic Plaque
❏ inadequate perfusion of brain due to
• an embolus from an atherosclerotic plaque in a large vessel (artery to artery embolus) (most common)
• a large vessel thrombosis with low distal flow
❏ risk factors
• hypertension (HTN)
• diabetes mellitus (DM)
• cigarette smoking
• high cholesterol
❏ treatment
• control atherosclerotic risk factors
• carotid endarterectomy in selected patients (see below)
• antiplatelet agents: aspirin, ticlopidine, clopidogrel, aggrenox (dipyridamole and ASA)
Cardiogenic Origin
❏ an embolus of clot
• risk factors: A fib (commonest cause), LV aneurysm, LV dysfunction, increased age,
mitral annulus calcification
❏ air emboli - during surgery or diving
❏ valvular vegetations (infection, tumour)
❏ treatment
• risk of embolization can be decreased with anticoagulation (heparin and warfarin)
• increase risk of hemorrhagic infarction implying that it is imperative to exclude the
presence of bleeding
prior to starting anticoagulants (i.e. do CT scan at 48 hours post-bleed)
• if moderate sized infarct, delay anticoagulation 5-14 days
Lacunar Infarction
❏ small (< 2 cm) and deep infarcts (lacune means lake)
❏ most < 5 mm, only 1% > 10 mm = "giant lacunes"
❏ pathology
• lipohyalinosis of small penetrating arteries of basal ganglia and brain stem; microatheroma;
junctional plaques (atherosclerosis of parent vessel blocking orifices of penetrating vessels)
❏ sites
• putamen
• internal capsule - pure motor
• thalamic - pure sensory
• pons - brainstrm signs
❏ clinical syndromes
• pure motor or pure sensory
• clumsy hand dysarthria
• ataxic hemiparesis
❏ risk factors
• HTN
• DM
• increasing age
❏ treatment
• control HTN
• use antiplatelet drugs
Other Causes
❏ large artery diseases (Moya Moya, Takayasu’s arteritis)
❏ dissection, trauma, vasculitis (PAN, meningovascular syphilis)
❏ coagulation/viscosity problems (especially in younger patients)
❏ venous infarction (cortical vein or sinus thrombosis)
• seen in "hypercoagulable states" (e.g. pregnancy, dehydration) and results in cortical infarction,
often complicated by 2º hemorrhage and seizures
Risk Factors for Stroke
❏ hypertension (HTN)
❏ smoking
❏ myocardial infarction (MI)
❏ atrial fibrillation (A fib)
❏ diabetes mellitus (DM)
❏ alcohol abuse
❏ homocysteinemia
❏ obesity
❏ severe carotid stenosis
❏ parental stroke (family history)

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 STROKE . . . CONT.
Investigations
❏ laboratory
• CBC, ESR, PT, PTT, VDRL, glucose, lipids and hypercoagulability work-up (Protein C, Protein S,
Factor V Leiden, anti cardiolipin antibody, lupus anticoagulant, PT/INR, PTT, anti-phospholipid
antibody and heparin cofactor II)
❏ neuroimaging
• CT, MRI, functional imaging (SPECT, PET)
• for acute stroke, unenhanced CT head is imaging method of choice
❏ cardiac
• ECG, echocardiogram (transesophageal), Holter monitor
❏ non-invasive studies
• duplex doppler of carotids, transcranial doppler to look at intracranial vessels, MR angiography
❏ angiography
Management
❏ Asymptomatic Carotid Bruit
• suggests the presence of atherosclerotic stenosis and signifies increased risk for both
cerebral and myocardial infarction
• modify risk factors, +/– antiplatelet therapy
• if stenosis > 60%, risk of stroke is 2% per year
❏ TIA, Mild Stroke
• investigate to determine the vascular territory and etiology, then treat accordingly for
atherosclerotic pathogenesis: manage risk factors and use antiplatelet agents - ASA, ticlopidine (Ticlid),
clopidrogel (Plavix), Persantine with ASA (Aggrenox)
• for carotid territory event, consider carotid endarterectomy by a good experienced surgeon if there is
severe ipsilateral, extracranial carotid stenosis (> 70% by angiography)
• if angiography shows 50-69% stenosis refer to stroke neurologist to assess indication
for carotid endarterectomy
❏ Acute Cerebral Infarction
• management goals
• ensure medical stability
• limit or prevent neuronal death
• avoid secondary complication of immobilization (e.g. pneumonia, pulmonary embolus)
• prevent recurrent cerebral infarction
• practical guidelines
• ensure the ABC's
• patient with vertebrobasilar ischemia or bihemispheric ischemia can have decreased
respiratory drive or muscular airway obstruction
• consider heparin in patients who are not eligible for tPA who have a larger artery atherosclerotic
stroke, progressing thromboembolic stroke, or cardioembolic stroke and who do not have a
large infarct, uncontrolled hypertension or bleeding condtions
• make the correct etiological diagnosis so you have a rational
approach for secondary prevention of stroke
• remember that MI is an important cause of morbidity and mortality in these patients;
screen for and manage the patient’s CAD
• consider transfer to stroke center if patient seen in first few
hours for neuroprotective or thrombolytic therapy (both under evaluation by clinical trials)
• consider thrombolysis if early in course (< 3 hours from onset)
• IV tPA if severe deficit, < 3 hours from onset and no evidence of hemorrhage on CT
• ineligible for tPA if
• history of intracranial hemorrhage
• major surgery within 14 days
• GI bleed within 21 days, head trauma or stroke within 3 months
• LP within 7days, rapidly improving
• BP greater than 185/110, seizures at onset
• symptoms suggest SAH, post MI pericarditis, pregnant, evidence of hemorrhage on CT
• PTT greater than 15s on warfarin, increased INR on heparin,
• platelets less than 100,000/mm3
• glucose less than 50 or greater than 400mg/dl
• severe anemia
• BP: DO NOT LOWER THE BP, avoid acute administration of anti-hypertensive agents;
unless hypertension is extreme
• most patients with an acute cerebral infarct are initially hypertensive and their BP
will fall spontaneously within 1-2 days
• acutely elevated BP is necessary to maintain brain perfusion
• anytihypertensive therapy is withheld for at least 10days after thromboembolic stroke unless
there is cardiac failure, aortic dissection, or a systolic above 220mmHg or a diastolic BP
above 120mmHg
• IV labetalol is usually first line

N52 – Neurology MCCQE 2002 Review Notes

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 STROKE . . . CONT.
• avoid hyperglycemia which will increase the degree of lactic acidosisin ischemic tissue,
increase the infarct size
• keep patient well hydrated, this will keep blood viscosity low and maintain perfusion of
ischemic tissue
• keep patient NPO if there is any hint of abnormal swallowing due to the risk of aspiration
• aspirin 325mg should be started within 48 hours of stroke onset, optimal dose uncertain,
no compelling evidence that any specific dose more effective
• give antiplatelets clopidogrel if ASA not suitable (e.g. Anaphylaxis, ulcers)
• clopidogrel (75mg/d) preferred over ticlopidine because of risk of neutropenia with ticlopidine
• start ambulation early, and if not feasible, use subcutaneous heparin to avoid DVTs
• glycoprotein IIB/IIIA platelet receptor antagonists, most powerful antiplatelet agents,
have been used in cardiac revascularization and are currently being evaluated in
ischemic stroke patients

Clinical Pearl
❏ The leading causes of death during the first month following a stroke are pneumonia,
pulmonary embolus, cardiac disease and the stroke itself.
❏ If a patient survives beyond the first week following a stroke, the cause of death is not
directly related to the stroke.

MULTIPLE SCLEROSIS
❏ a relapsing or progressive disease of CNS myelin characterized by disseminated patches of demyelination in
the brain and spinal cord, resulting in multiple and varied neurological symptoms and signs usually
with exacerbations and remissions
❏ lesions separated in time and space
Epidemiology
❏ onset usually 20-40, but can be younger or older
❏ F:M = 3:2
❏ prevalence in North America 1/1,000; most common in European races and
in countries farther from the equator
❏ genetic predisposition: 3% risk for first degree relatives, 30% concordance for identical twins,
HLA DR2 and Dw2 association
Etiology
❏ unknown but immunological and viral theories
Pathology
❏ multiple discrete lesions of myelin destruction (plaques)
❏ common plaque sites include optic nerve, periventricular areas, corpus callosum, brainstem, spinal cord
Course of Illness
❏ 5 Types
1. relapsing remitting (80% present this way initially, F>M)
2. primary progressive (gradually progressive clinical course from presentation, F=M)
3. secondary progressive (starts with relapsing remitting becomes progressive)
4. clinically inactive disease
5. mixed pattern
Signs and Symptoms
❏ signs: hyperreflexia, ataxia, nystagmus, spasticity and limb weakness
❏ symptoms: parasthesia, gait disorder, weakness (e.g. hemiparesis, paraparesis (myelopathy))
and incoordination, visual loss (optic neuritis) and diplopia, incontinence, fatigue
Common Features
❏ internuclear ophthalmoplegia (lesion in MLF causing failure of adduction of the ipsilateral eye and nystagmus
of the abducting eye on attempted lateral gaze)
❏ optic neuritis
❏ Lhermitte's sign (forward flexion of the neck causes electric shock sensation down the back to limbs,
indicative of cervical cord lesion)
❏ Uhthoff's phenomenon (worsening of symptoms with heat e.g. hot bath, exercise)
❏ trigeminal neuralgia in young patient
MCCQE 2002 Review Notes Neurology – N53

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 NEUROLOGICAL Pathology:
ISCHAEMIC ENCEPHALOPATHY

- Global Ischaemia – (Ie. Hypoxemic Encephalopathy):

o Aetiology:
Impaired blood supply – Eg. Heart Failure, Hypotension, Shock, Carotid Thrombosis.
Impaired O2 carrying – Eg. Anemia, Hypoxia.
Impaired O2 utilization – Eg. Cyanide/Carbon-Monoxide Poisoning.
Excessive Neuronal Activity – Eg. Epilepsy/Seizure
o Pathogenesis:
Heart Failure, Anaemia, Hypotension, Hypoxia, Shock, Etc Global Brain Ischaemia
Sensitive Areas:
Adults:
o Watershed Zone (Between ACA & MCA Perfusion Zones)
o 3rd, 5th, 6th Layers of Cortex
o Hippocampus
o Purkinje Cells – Cerebellum Border Zone (watershed areas)
Infants:
o Brainstem Nuclei
o Morphology:
Watershed Zone Necrosis (Between ACA & MCA Perfusion Zones)
Laminar Necrosis – (Chronic – Short penetrating arteries) – the nuclear layers of the cortex
Hippocampus
Purkinje Cells of the Cerebellum
o Clinical Features:
Mild Transient Confusion Severe Irreversible Brain Death (Flat EEG = Vegetative = Coma)

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 NEUROLOGICAL Pathology:
MOTOR NEURONE DISEASES & POLIO

- Motor Neurone Disease (MND)/Amyelotrophic Lateral Sclerosis (ALS):

o *Remember:
Skeletal Muscles are Innervated by Lower Motor Neurons (Ventral Spinal Root Muscle)
LMN a e inne a ed b Uppe Mo o Ne one F om he Co ico pinal T ac
o Aetiology:
90% Sporadic; 10% Genetic
o Pathogenesis:
P og e i e Degene a ion of LMN UMN in he Spinal Co d
(+ Cranial Nerve Nuclei in the Spinal Cord)
UMN & LMN Degeneration Progressive Weakness, Muscle Wasting, Fasciculations,
Spasticity/Stiffness & Hyperreflexia.
Affects Voluntary Muscles (Ie. Walking, Speaking, Breathing, Swallowing)
o Morphology:
Macro:
Degeneration of the Ventral Horns of the Spinal Cord (Ie. LMN)
Degeneration of the Ventral Spinal Roots (Ie. LMN)
Degeneration of Ventral & Lateral Corticospinal Tracts in Spinal Cord (UMN)
Micro:
Neurons may show Spongiosis
Higher #s of Astrocytes
Neuronal Inclusions Skein-like Incl ion B nina Bodie Vac olisation.
o Clinical Features:
Highly Aggressive (Normal Severe within 1yr)
Voluntary Motor Only; Sensory System is Spared
LMN Signs:
o Progressive Muscle Weakness
o Am o oph No M cle G o h M cle A oph Wa ing
o Fasciculations & Cramps
o Hyporeflexia if Mo l LMN a e Affec ed Muscle Innervation)
UMN Signs:
o Spacticity/Stiffness/Rigidity
o Hyperreflexia - if Mo l UMN a e Affec ed D e o Co ical Inhibi ion
(+ Up-Going Plantars (Babinski Sign))
Clinical Diagnosis
o Treatment:
Supportive (Ventilation, Parenteral Nurtition)
o Prognosis:
Incurable
Death within 3yrs

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- Poliomyelitis:
o Aetiology:
Poliovirus Infection
o Epidemiology:
Non Existent in Aus (A single case would be an epidemic)
o Prevention:
Vaccination Available
Live Attenuated (Oral Polio Vaccine):
o Advantages:
Easy Administration - Given Orally
o Disadvantage:
Rarely causes paralysis (1 in 2.5million)
Inactivated Polio Vaccine (IPV):
o Advantages:
Carries NO risk of Vaccine-Associated Polio Paralysis
o Disadvantage:
Difficult Administration - Has to be injected
o Pathogenesis:
Transmission:
Faecal-Oral
or Respiratory
Initially Enteric Infection Spreads to Bloodstream Spinal Cord Preferentially Infect
& Destroy Motor Neurons
o Clinical Features:
90% Asymptomatic
<10% Minor Viral Illness:
Headache
Neck/Back pain
Abdominal Pain
Fever, Lethargy, Vomiting
1% CNS Infection Paralysis
Acute Asymmetrical Flaccid Paralysis + Areflexia
If Spinal Polio Paralysis of Legs(unilateral)
If B lba Polio Cranial Nerve Paralysis (eg. Dysphagia, Dysphasia, Dyspnoea)
Or Combination of Both.

o Treatment:
Self-Limiting, but Lasting Disability Only Supportive Rx (Eg. Ventilation, Physiotherapy)
But Vaccine Preventable

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 DISEASES OF THE SPINAL CORD
fasciculus cuneatus

fasciculus gracilis

spinothalamic tract

spinocerebellar tract
corticospinal tract

posterior spinal artery

anterior
spinal artery

Figure 15. Anatomy of the Spinal Cord

Illustration by Aimée Warrell

CLINICAL FEATURES
❏ paraplegia or quadriplegia
❏ deficit corresponding to a sensory level
• T4-nipple, T10-navel, L5-dorsum of foot, S1-lateral foot
❏ bladder and bowel incontinence
❏ LMN signs at the level of the lesion and UMN signs below the level of the lesion (see Table 15)
❏ radicular symptoms (sharp, shooting radicular pain, dermatomal sensory loss)
• causes
• extradural: disc, trauma, cervical spondylosis, bone, tumour (metastases), abscess
• intradural, extramedullary: meningioma, neurofibroma, arachnoid cyst
• intradural, intramedullary: demyelination, inflammatory (transverse myelitis) tumour, infarct,
hemorrhage (AVM), or degeneration (subacute combined degeneration (SACD)), syrinx

Table 15. Comparison of UMN and LMN Lesions

Upper Motor Neuron (UMN) Lower Motor Neuron (LMN)
Bulk Normal (unless disuse) Muscle wasting
Tone Increased (spastic) Decreased
Fasciculations Absent Present
Weakness Pyramidal pattern Specific to lesion
Upper extremity: extensors weakest i.e. root, nerve
Lower extremity: flexors weakest
Reflexes Increased Decreased —> absent
Plantar Reflex Extensor Flexor

Clinical Pearl
❏ Spinal cord diseases usually cause a triad of: parasthesia at a sensory level (hallmark
symptom); bilateral corticospinal (spastic) weakness; bowel and bladder problems.

SPINAL CORD SYNDROMES (see Neurosurgery Chapter)

Brown-Sequard Syndrome
❏ lateral compression of one half of spinal cord (hemisection)
❏ ipsilateral LMN signs at level of lesion
❏ ipsilateral hemiplegia or monoplegia below lesion & UMN signs
❏ ipsilateral loss of vibration and proprioception below lesion
❏ contralateral loss of pain and temperature below lesion
❏ common causes – tumour, radiation

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 DISEASES OF THE SPINAL CORD . . . CONT.
Central Cord Syndrome
❏ common causes are syringomyelia and intrinsic tumours
❏ suspended or cape sensory loss over shoulders from cervical lesion most common
❏ dissociated sensory loss
• loss of pain and temperature sensation with spared touch, joint position and vibration
❏ atrophy of intrinsic hand muscles (anterior horn cells),
Anterior Spinal Artery Syndrome
❏ sudden para/quadriplegia; initial flaccidity and areflexia (“spinal shock”);
within days develop UMN signs below lesion
❏ preserved vibration and position sense (spared posterior columns)
❏ due to occluded anterior spinal artery, usually at thoracic level
Subacute Combined Degeneration of the Spinal Cord
❏ corticospinal and posterior columns are affected e.g. vitamin B12 deficiency, HIV
❏ system degeneration therefore no distinct motor or sensory level
Conus Medullaris (Cauda Equina Syndrome)
❏ hypotonic bladder and rectal sphincters
❏ pain and loss of sensation in saddle distribution in perineum
❏ foot drop, absent ankle jerks
❏ causes: cord disc herniation, tumour, inflammatory
Foramen Magnum Syndrome
❏ quadriparesis, neck/head pain, hand atrophy +/– cerebellar signs
❏ cause: tumours
Transverse Myelitis
❏ back pain, rapid onset cord syndrome
❏ cause: post-infections, inflammatory, demyelination
Epidural Abscess
❏ severe back pain and tenderness, rapidly progressive cord syndrome
❏ may not have systemic signs/symptoms of infection
❏ surgical emergency!!!
Complete Transection of Cord
❏ trauma, tumour
❏ loss of all sensation and voluntary motor function below level (UMN)
MOTOR NEURON DISEASES
❏ diseases of cortical ––> anterior horn motor neurons
Spinal Muscular Atrophy (SMA)
❏ disorders beginning in infancy or childhood characterized by skeletal muscle wasting
due to progressive degeneration of cells in the anterior horn and medulla
❏ acute infantile SMA (Werdnig-Hoffmann disease)
❏ floppy baby, survival < 1 year
• childhood forms
• Wohlfart-Kugelberg-Welander disease
❏ adult forms
• proximal and distal muscles (may look like myopathy)
• slowly progressive, good prognosis
Amyotrophic Lateral Sclerosis (ALS)
❏ motor neuron disease of unknown etiology characterized by progressive degeneration
of corticospinal tracts and anterior horn cells or bulbar efferent neurons
❏ 10% familial
❏ onset age = 40-60 (very rare < 20)
❏ progressive, fatal 2-6 years (50% at 3 years)
❏ signs and symptoms
• no sensory findings
• fasciculations, muscle cramps
• segmental, asymmetrical weakness and atrophy
• upper and lower motor neuron signs (tonic atrophy)
• bulbar palsy, atrophy and tongue fasciculations
• dysarthria, dysphagia
• sparing of bowel and bladder function, and ocular muscles
❏ investigations
• EMG: diffuse denervation, fibrillation with normal nerve conduction
• Don’t miss high spinal cord lesion!
❏ treatment
• Riluzole in bulbar causes prolongs survival
• supportive treatment
SPINAL ROOT(see Neurosurgery Chapter)

MCCQE 2002 Review Notes Neurology – N39

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 NEUROLOGICAL Pathology:
MULTIPLE SCLEROSIS & LEUKODYSTROPHIA

Multiple Sclerosis – (Demyelinating Disorder):

- (Formed Meylin gets Destroyed)
- Aetiology:
o Chronic Autoimmune Inflammatory Disease of the CNS
o Some Genetic Concordance
- Pathogenesis:
o Precise Mechanism Unknown.
o Autoimmune Demyelination within the Brain Spinal Cord (Stripping of Wire’s Insulation
Defective Impulse Transmission & Short-Circuits.
o Affects the CNS ONLY!!
- Morphology:
o Macro:
Patches/islands of Grey-matter-like material in the white matter (Demyelination)
Multiple Soft Pink plaques of Demyleination (Periventricular) (Seen as white periventricular
patches on MRI)
o Micro:
Areas of Loss of Myelin Firstly Around the blood vessels, extending outwards.
Perivascular Inflammation: T-Lymphocytes, Macrophages & Plasma Cells
Reactive Gliosis
- Clinical Features:
o Onset @ 20-40yrs
o Affects White Matter Only
Limb weakness
Ataxia
Paraesthesia
Optic Neuropathy
Vertigo + Nystagmus, but Without Tinnitus or Deafness.
o Relapsing & Remitting
o Progressive Spastic Quadraparesis Death in years (Typically due to paralysis of chest muscles
pneumonia)
- Diagnosis:
o MRI – Visible Plaques around the Ventricles in the White Matter.
o CSF Examination – Oligoclonal IgG
- Treatment:
o Currently Incurable
o Home Care & Supportive Therapy

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Leukodystrophies – (Myelin Production Disorders):
- (Insufficient Production of Myelin)
- Many, Many Types
- Aetiology:
o Genetic Defect
- Pathogenesis:
o Insufficient Production of Myelin (Insufficient Insulation around Wires) Defective Impulse
Transmission & Short-Circuits.
- Morphology:
o Macro:
o Micro:
- Clinical Features:
o Gradual Decline in a Previously-Well Infant/Child:
Body Tone
Movements
Gait
Dysphasia
Dysphagia
Sensory Impairment (Vision/Hearing)
Behaviour
Slowed Mental/Physical Development
- Treatment:
o Incurable

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 STROKE . . . CONT.
• avoid hyperglycemia which will increase the degree of lactic acidosisin ischemic tissue,
increase the infarct size
• keep patient well hydrated, this will keep blood viscosity low and maintain perfusion of
ischemic tissue
• keep patient NPO if there is any hint of abnormal swallowing due to the risk of aspiration
• aspirin 325mg should be started within 48 hours of stroke onset, optimal dose uncertain,
no compelling evidence that any specific dose more effective
• give antiplatelets clopidogrel if ASA not suitable (e.g. Anaphylaxis, ulcers)
• clopidogrel (75mg/d) preferred over ticlopidine because of risk of neutropenia with ticlopidine
• start ambulation early, and if not feasible, use subcutaneous heparin to avoid DVTs
• glycoprotein IIB/IIIA platelet receptor antagonists, most powerful antiplatelet agents,
have been used in cardiac revascularization and are currently being evaluated in
ischemic stroke patients

Clinical Pearl
❏ The leading causes of death during the first month following a stroke are pneumonia,
pulmonary embolus, cardiac disease and the stroke itself.
❏ If a patient survives beyond the first week following a stroke, the cause of death is not
directly related to the stroke.

MULTIPLE SCLEROSIS
❏ a relapsing or progressive disease of CNS myelin characterized by disseminated patches of demyelination in
the brain and spinal cord, resulting in multiple and varied neurological symptoms and signs usually
with exacerbations and remissions
❏ lesions separated in time and space
Epidemiology
❏ onset usually 20-40, but can be younger or older
❏ F:M = 3:2
❏ prevalence in North America 1/1,000; most common in European races and
in countries farther from the equator
❏ genetic predisposition: 3% risk for first degree relatives, 30% concordance for identical twins,
HLA DR2 and Dw2 association
Etiology
❏ unknown but immunological and viral theories
Pathology
❏ multiple discrete lesions of myelin destruction (plaques)
❏ common plaque sites include optic nerve, periventricular areas, corpus callosum, brainstem, spinal cord
Course of Illness
❏ 5 Types
1. relapsing remitting (80% present this way initially, F>M)
2. primary progressive (gradually progressive clinical course from presentation, F=M)
3. secondary progressive (starts with relapsing remitting becomes progressive)
4. clinically inactive disease
5. mixed pattern
Signs and Symptoms
❏ signs: hyperreflexia, ataxia, nystagmus, spasticity and limb weakness
❏ symptoms: parasthesia, gait disorder, weakness (e.g. hemiparesis, paraparesis (myelopathy))
and incoordination, visual loss (optic neuritis) and diplopia, incontinence, fatigue
Common Features
❏ internuclear ophthalmoplegia (lesion in MLF causing failure of adduction of the ipsilateral eye and nystagmus
of the abducting eye on attempted lateral gaze)
❏ optic neuritis
❏ Lhermitte's sign (forward flexion of the neck causes electric shock sensation down the back to limbs,
indicative of cervical cord lesion)
❏ Uhthoff's phenomenon (worsening of symptoms with heat e.g. hot bath, exercise)
❏ trigeminal neuralgia in young patient
MCCQE 2002 Review Notes Neurology – N53

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 MULTIPLE SCLEROSIS . . . CONT.

Clinical Pearl
❏ MS is a common cause of internuclear ophthalmoplegia.

Diagnosis
❏ evidence from history and examination of multiple lesions disseminated in both time and space
❏ slowing of evoked potentials (visual/auditory/somatosensory)
❏ CSF (oligoclonal Ig bands in 90%, increased IgG concentration, mild lymphocytosis and increased protein)
❏ MRI (plaques show as hyperintense lesions on T2 MRI in periventricular distribution)
Management
❏ patient education and counseling (disclosure, prognosis, future expectations, support groups,
psychosocial issues: divorce, depression, suicide not uncommon)
❏ acute treatment
• corticosteroids are the most commonly used treatment for acute attacks
• current recommendation is to treat disabling attacks with 500 to 1,000 mg of IV methylprednisolone
for 3-5 days with or without short oral tapering dose of prednisone
❏ symptomatic treatment
• symptomatic treatment for spasticity (baclofen), painful symptoms, bladder dysfunction (ditropan),
fatigue (amantadine), depression
• monitor closely for infection especially UTI
• physiotherapy, speech therapy, occupational therapy, nutrition, social work
❏ disease suppressing agents
• interferons, copolymers are being used to suppress disease activity
• mechanism uncertain
• disease suppressing medications are indicated for ambulating patients with frequent relapses
• ß-interferon (beta 1b, Betaseron) shown to decrease relapse rate, decreased rogression of disability
in patients with relapsing/remitting and progressive disease
• ß-interferon (beta 1a, Avonex) appears promising with similar efficacy to betaseron
• Copolymer also decreases relapse rate in relapsing remitting disease
and is currently under investigation for use in secondary progressive
• trials under way for chronic and primarily progressive

CNS INFECTIONS
❏ see Infectious Diseases Chapter
MENINGITIS
❏ inflammation of the meninges
Predisposing Factors
❏ systemic (especially respiratory) or parameningeal (otitis media, odontogenic, sinusitis) infections
❏ head trauma
❏ anatomical meningeal defects
❏ previous neurosurgical procedures
❏ cancer, alcoholism, and other immunodeficiency states
Etiology
❏ bacterial
• neonates: E. coli, Group B Streptococcus, Listeria monocytogenes
• infants and children: H. influenzae, S. pneumoniae, N. meningitidis
• adolescents and adults: S. pneumoniae, N. meningitidis
• elderly: S. pneumoniae, N. meningitidis, Gram negatives
• CSF leak: S. aureus, Gram negatives
• immunocompromised: Listeria monocytogenes
❏ viral (“aseptic”)
• Enteroviruses, H. influenzae, HIV, HSV, Adenovirus
❏ fungal
• cryptococcus
❏ other
• Treponema pallidum (meningeal neurosyphillis)
• Borrelia burgdorferi (Lyme disease)
• TB

N54 – Neurology MCCQE 2002 Review Notes

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 NEUROLOGICAL Pathology:
MYAESTHENIA GRAVIS

Myasthenia Gravis
- Se ere M scle Weakness
o Myo(Muscle)
o Asthenia (Weakness)
o Gravis (Grave/Severe)
- Pathophysiology:
o MG is an Antibody-Mediated Autoimmune Disease which attacks the ACh-Receptors @ the NMJ,
leading to Failure of Neuromuscular Transmission.
o How? By 3 Mechanisms:
A) Complement Binding & Activation @ the NMJ:
Ab-Binding to the AChR activates the Complement Cascade.
Receptor Density Changed Physical Architecture of the Muscle
o Failure of Neuromuscular Transmission.
B) Antigenic Mod lation
Ab-Binding Cross-Links AChRs on NMJ.
Causes Endocytosis & Destruction of the Cross-Linked AChRs.
Leads to a Reduced Number of AChRs on the NMJ.
o Failure of Neuromuscular Transmission.
C) Functional AChR-Block by Antibodies: (Relatively Rare)
Ab-Binding to AChR @ the ACh-Binding Sites.
Causes functional block of the AChR by preventing ACh binding @ the NMJ.
o Failure of Neuromuscular Transmission.

- 2 Types of Myasthenia Gravis:

o Ocular MG:
Fatigable Muscle Weakness limited to Extrinsic Ocular Muscles of the Eye.
In 75% of Cases, Ocular MG is the Initial Manifestation of Generalised MG.
Ocular Muscles are susceptible due to constant rapid neuronal stimulation.
Symptoms:
Double Vision (Diplopia)
Drooping Eyelids
o Generalised (Whole Body) MG:
Fatigable Muscle Weakness involving Other Muscle Groups Including the Eye Muscles.
As mentioned above, Generalised-MG typically begins as Ocular-MG, and then progresses
to the rest of the body.

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 Symptoms (In addition to Ocular MG):
Unstable/Waddling Gait
Weakness in Arms/Hands/Fingers
Weakness in Legs
Weakness in Facial Muscle
Difficulty Swallowing
Shortness of Breath

- Diagnosis:
o Clinical (Pharmacological) Test:
Edrophonium (Short-Acting ACh-Esterase Inhibitor):
Repeated stimulation of remaining receptors.
If symptoms improve, then it s MG
o Serology Testing:
Test for Antibodies Against:
AChR (Acetylcholine Receptors)
MuSK (MuSK = Muscle-Specific Tyrosine Kinase)
Anti-Muscle (eg. Actin) Antibodies
o Electrophysiological Tests:
Repetitive Stimulation of Peripheral Nerves:
Single-Fibre Electromyography:
- Treatment:
o Acetyl-Cholinesterase Inhibitors:
Drugs that Inhibit the Cholinesterase Enzyme from degrading ACh in the Synapse.
Eg. Physostigmine/Organophosphates
Mechanism of Action:
Prolonged Action of ACh in the Synapse
ACh in the Synapse
Side Effects:
Short Term Excessive ACh-Signalling:
o Increases Mainly Parasympathetic Activity:
Increased Secretions (Salivary/Lacrimal/Bronchial/Intestinal)
Increased Peristaltic Activity
Bronchoconstriction
Bradycardia & Hypotension
Pupillary Constriction ( Fall in Intraocular Pressure)
Long Term Desensitisation of AChRs:
o Decreased Parasympathetic Activity.
o In Cholinergic Crisis Muscle & Respiratory Paralysis:
(Cholinergic Crisis = AChR Desensitisation due to ACh-
Stimulation)
ACh-E-Inhibitors Potentiate further Desensitisation.
In Myasthenic Crisis Original Dose is Ineffective:
o (Myasthenic Crisis = Disease Progression Number of ACh-Rs )
o Requires higher Dose for Therapeutic Effect.
o Immunomodulation:
Goal To Remove the Source of the Antibodies or the Antibodies Themselves.
Variety of Options:
Thymectomy
Plasma Exchange
Intravenous-Ig
Immunoadsorption.
o General Immunosuppression:
Goal To dial down the whole immune system slow progression of disease.
Immunosuppressive Drugs:
*Prednisone:

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 o Prednisone = A Corticosteroid = Powerful Anti-Inflammatory
*Azathioprine:
o A Purine-Analogue DNA Synthesis in Clonally Dividing Cells (B/T-
Cells).
- Complications:
o Cholinergic & Myasthenic Crises:
Cholinergic Crisis:
Rapid Desensitisation of the AChRs due to ACh-Stimulation.
Requires Instant Cessation of Acetyl-Cholinesterase Inhibitors.
Myasthenic Crisis:
The point at which the Disease Progression has rendered the current ACh-E-
Inhibitor Useless
Often causes Paralysis of Respiratory Muscles Pt. Requires Assisted Ventilation.
Ie. Requires a higher dose of ACh-E-Inhibitor to Maintain Therapeutic Effects.
o Rectifying these Complications:
Cholinergic Crisis:
Cessation of ACh-E-Inhibitors.
Myasthenic Crisis:
Dose of ACh-E-Inhibitors.

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 NEUROLOGICAL Pathology:
NEUROSYPHILIS

NEUROSYPHILIS:
- Aetiology:
o Infection of the Brain/Spinal Cord by Bacteria: Treponema pallidum.
- Pathogenesis:
o Chronic, Untreated Syphilis – Usually after 10-20yrs Tertiary Syphilis Brain/Spinal Cord
- Clinical Features:
o Weakness, Abnormal Gait
o Blindness, Argyll-Robertson Pupils (Bilateral Miosis, responsive to accommodation, but not to light)
o Confusion, Dementia, Irritability
o Depression
o Headache
o Paraeshtesia in Toes/Feet/Legs
o Seizures
- Treatment:
o 2wk Course of IV/IM Penicillin.

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 Week 11
Neuroscience Synthesizing Session Notes
Disorders of the Special Senses:

Visual Disorders
- Visual Acuity:
o The ability to discriminate fine detail in a visual image.
o Refers to the ability of one cone to be stimulated without its neighbour being stimulated
o Disorders are typically due to imperfections in lens/eyeball shape.
o Measured Using Snellen’s Charts @ 6m distance (See lecture notes for full description)
§ 20/20 (20ft) (6m) = Normal Acuity
§ 20/15 = Better than Normal Acuity
§ 20/60 = Less than Normal Acuity

- Glaucoma:
o A group of diseases characterised by raised intraocular pressure due to an imbalance between
Aqueous Humour Production & Drainage (via Canal of Schlemm)
o IOP produces ocular tissue damage
o One of the leading causes of blindness
o 3 Categories:
§ Angle-closure
• Aqueous humour produced by the ciliary body normally is emptied through the canal
of schlemm.
• Angle-Closure is an imbalance between production & drainage à ↑IOP
• “Angle” = the angle between the inside of the cornea & the iris.
• “Closure” = the angle is too small à blocks off the canal of schlemm.

§ Open angle
• The ‘angle’ is fine.
• But the part of the canal of schlemm is blocked à obstruction to drainage à ↑IOP
§ Congenital/juvenile
• Hereditary
• Infections can do it too.
o IOP à pressure on nerve fibres à axonal necrosis
§ à pressure on the blood vessels à ↓Blood flow

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 - Diabetic Retinopathy:
o Change in basement membranes of the retinal capillaries:
§ Microaneurysms
§ Microvascular obstruction
§ Non-perfusion of capillaries
§ Narrowing of arterial walls
§ Increase in Retinal Vein Calibre
- Cataracts:
o Opacity in the crystalline lens
o Causes are multifactorial:
§ Metabolic disease (eg. Diabetes)
§ UV Light
§ Smoking
§ Ocular diseases (eg. Glaucoma)
§ Skin diseases (eg. Dermatitis)
§ Drug induced (Eg. Corticosteroids)
§ Ageing (Idiopathic – unknown cause)
o Prevention is important –
§ Improving nutrition
§ Reducing diarrhoea
§ Wearing sunglasses with UV filters.
Common hearing deficiencies:
- Ageing:
o Progressive loss of hearing receptors
- Acute Damage:
o Hair cells can be destroyed by a single explosive sound or continuous high-intensity sound à tears at
the cilia
- Drugs:
o Some are ototoxic (damage the hair cells)
- Tinnitus:
o Ringing in the ear in the absence of auditory stimulus
o Symptom of nerve degeneration/inflammation of middle/inner ear.
o Can be caused by drugs. (Damage can be permanent)
§ Some antibiotics (Streptomycin, neomycin)
§ Loop diuretics (transient)
§ Salycilates
- Otitis Media:
o Inflammation of middle ear lining
o Is a common result of throat infections in infants & children (due to short Eustachian tube).
o Can be bacterial viral or bacterial
o Eardrum becomes inflamed and bulges – can perforate
o When large amounts of fluid - pus accumulate behind the eardrum, grommets may be inserted.
- Vertigo:
o Hallucinatory sensation of movement (Referred to dizziness)
o Labyrinthitis or vestibular neuronitis (subsequent to viral/bacterial infection/metabolic disturbance –
eg. hypoglycaemia)
o Elderly patients – due to reduced blood supply to the labyrinth.
- Meniere’s Syndrome:
o Labyrinthine disorder – affects both semicircular canals & cochlea:
§ Repeated attacks of vertigo, nausea & vomiting
§ Tinnitus is common & hearing is impaired
- Positional Vertigo:
o May often follow trauma
o May follow drug overdose
o Anxiety & depression may contribute (psychogenic)
- Motion Sickness:
o Mismatch between visual & vestibular information.

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 NEUROLOGICAL Pathology:
PARKINSON'S DISEASE

PARKINSON S DISEASE Shaking Pals

- Aetiologies:
o Parkinsonism Shaking Pals The Clinical S ndrome
(Drugs/Toxins/Post Encephalitis)
o Parkinson s Disease Primar Atroph of Substantia Nigra Dopaminergic System)
(Idiopathic)
- Pathogenesis:
o Loss of Substantia Nigra (Dopamine makers) Neurons Dopamine Deficiency in the Basal
Ganglia Basal Ganglia Dysfunction.
Dopamine is required by the basal ganglia to coordinate complex movements, therefore, a
lo of he ba al ganglia f nc ion m om of Pa kin on
o More Specifically:
S lemen a Mo o A ea i n ac i a ed o Fine-Tune Movement.
- Morphology:
o Macro:
Loss of Pigment Neurons (Melanin) in the Striatum

- Clinical Features:
o Onset: In second half of life (mean age of onset = 55yrs)
o Parkinson s Triad
1. Resting Tremor Ma be a Pill-Rolling emo
2. Rigidity (Hypertonia Re i ance o Pa i e Join Mo emen
Ma be Lead-Pi e Rigidi Con an o Cog-Wheel Rigidi Fl c a ing
3. Brady/Akinesia (Slowness/Inability to Initiate/Execute Movement)
o Other Symptoms:
+Diminished Facial Expressions
+Stooped Posture
+Shuffling/Hurried Gait
+Declined intellectual function
+Depression
+ Inability to pick up small objects, cups, do up buttons, write in small font etc.

- Treatment:
o Aim: To Do amine Le el in he B ain o o mimic he effec of Do amine
o Oral Levodopa: (L-Dopa can cross the bbb Converted to Dopamine in the Brain)
o Dopamine Agonist: Drugs that mimic Dopamine, binding to Dopamine receptors.
o Side effects:
B Do amine in he Ba al Ganglia Do amine i Globall Side Effects
On Off Phenomenon Symptomatic Relief is Random & Fluctuating
O e ime Le odo a Efficac Dec ea e B Side Effec Inc ea e

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 MOVEMENT DISORDERS . . . CONT.

Table 9. Comparison of Corticospinal vs. Extrapyramidal Lesions

Corticospinal Extrapyramidal
Muscle Tone Clasp-knife spasticity Rigidity (lead-pipe or cogwheel)
Hypotonia (cerebellar)
Distribution of Arm flexors and leg extensors Flexors and extensors of all limbs
Increased Tone
Involuntary Absent Tremor, chorea, athetosis, dystonia
Movements
Tendon Reflexes Increased Normal
Plantar Reflex Extensor Flexor
Paralysis or Present Absent
Weakness

PARKINSON’S DISEASE (PD)

❏ an idiopathic, slowly progressive, degenerative CNS disorder
❏ insidious onset between 40-70 years
Clinical Features
❏ characteristic symptoms and signs (usually asymmetric onset)
• Tremor (rest, pill-rolling, 4-7 Hz, can be suppressed by voluntary movement)
• Rigidity (lead pipe and cogwheeling)
• Akinesia/Bradykinesia
• Postural instability (festinating gait, retropulsion, falls)
❏ other features
• D - dysphagia, drooling, decreased voice
• G - gait: start hesitation, small shuffling steps, loss of arm swing
• E - eye: blepharoclonus (fluttering of closed eyelids), lack of blinking
• M - micrographia
• M - mask like face (hypomimia)
• S - subcortical dementia (apathy, forgetful, poor ability to use knowledge)
DDx
❏ therapeutic drugs: neuroleptics, metoclopramide
❏ toxins: MPTP (drug abusers), manganese, carbon disulfide, CO
❏ “Parkinson Plus” disorders
❏ Vascular Parkinson’s
❏ post-infectious: 1914 flu epidemic (encephalitis lethargica “Awakenings”)
❏ metabolic: Wilson’s
Pathology
❏ loss of dopaminergic nigrostriatal neurons in substantia nigra’s zona compacta
❏ dopamine neurons degenerate, upsetting normal balance between dopaminergic inhibition and cholinergic
excitation of striatal output (GABA) neurons, results in relative acetylcholine (ACh) excess
❏ loss of neurons in multiple other selected areas
❏ result is relative increase in GABAergic output from striatum
❏ Lewy bodies (eosinophilic intraneural inclusion granules)
• not specific to PD
Treatment
❏ deprenyl (MAO-B inhibitor) acts by blocking dopamine breakdown and may slow progressive course
❏ levodopa + peripheral decarboxylase inhibitor (i.e. Sinemet)
❏ dopamine agonists (bromocriptine, pergolide, ropinirole, promipexole) can be used as 1st line or as add on
therapy when levodopa responsiveness diminishes
❏ anticholinergics (benztropine, trihexyphenidyl) for tremor
❏ NMDA antagonists (amantadine)
❏ neurosurgical options (lesions or stimulators)
❏ therapeutic problems: orthostatic hypotension, sudden loss of therapeutic effect, wearing off, dyskinesia,
freezing, psychiatric (psychosis, paranoia)
Clinical Pearl
❏ In Parkinson’s disease, postural instability generally appears later in the course of
the disease. If postural instability occurs earlier consider alternative diagnosis
e.g. Parkinson’s plus, Vascular Parkinson’s.

N22 – Neurology MCCQE 2002 Review Notes

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 MOVEMENT DISORDERS . . . CONT.
Vascular (pseudo) Parkinsonism
❏ parkinson’s-like state due to multiple strokes
❏ imaging usually shows basal ganglia lacunae or white matter change
❏ generally occurs in elderly; associated with hypertension
❏ often have signs and symptoms of Parkinson’s below the waist (short stepped gait, start hesitation, freezing,
postural instability) but relative sparing above the waist (normal voice and facial expression, absence of
rest tremor and bradykinesia)
❏ poor response to L-dopa
❏ patients with true PD may also have cerebrovascular disease in addition their PD
❏ CT/MRI helpful in distinguishing vascular parkinsonsim from true PD
“PARKINSON PLUS” DISORDERS
Progressive Supranuclear Palsy (PSP)
(Steele, Richardson, Olszewski Syndrome)
❏ age of onset 50 to 70 years
❏ onset characterized by difficulty in balance, abrupt falls, ocular disturbances, slurred speech,
dysphagia, vague personality changes, depression, abnormal facial expression
❏ may take years for characteristic syndrome to fully develop
• supranuclear ophthalmoplegia - abnormality of vertical gaze
(if eyes are fixated on a target and neck is flexed and extended, full or 8 movements can be obtained)
• pseudobulbar palsy - UMN spastic weakness of pharyngeal musculature, slurred speech,
mouth held open, swallowing difficulties, exaggerated jaw jerk
• axial dystonia - gradual stiffening and extension of the neck
(contrast to Parkinson’s where neck is flexed)
• affected neurons in subthalamus, thalamus, basal ganglia, and peri-aqueductal grey
• pathology: neurofibrillary tangles (like in Alzheimer’s)
• L-dopa not very effective in PSP
• fatal within 2-5 years
• may have prominent dementia or normal cognition
Multiple System Atrophy
❏ includes striatonigral degeneration, sporadic olivopontocerebellar atrophy, Shy-Drager Syndrome
❏ Parkinsonian features
❏ early dysautonomia (orthostatic hypotension, impotence, bladder dysfunction, mottled cold hands)
❏ other features are cerebellar dyfunction, pyramidal tract signs, stimulus sensitive myoclonus of
hands and feet, extreme forward neck flexion, inspiratory stridor, dysarthria
❏ 20% respond to L-dopa initially, 13% sustained response
TREMOR
Definition
❏ rhythmic oscillatory involuntary movement about an axis
Rest Tremor
❏ slow (3-7 Hz), coarse, distal
❏ the characteristic tremor of Parkinsonism
❏ hands - pill rolling, alternating flexion/extension of fingers or hands,
alternating pronation/supination of forearms
❏ best examined with hands resting in lap, can be brought out by tasks of concentration
Postural and Action (Kinetic) Tremor
❏ fast (6-12 Hz), fine, usually upper limbs, head (titubation)
❏ seen best with arms and hands outstretched
❏ physiological
• always present, imperceptible to the eye
❏ exaggerated physiological
• anxiety, sleep deprivation
❏ drugs (e.g. theophylline, lithium, caffeine, amphetamines, decongestants)
❏ drug withdrawal (e.g. EtOH)
❏ hyperthyroidism, hypoglycemia
❏ essential tremor
• AD inheritance
• patient complains of shaking when carrying teacup, putting a glass to the mouth, or trying to drink soup
• affects handwriting, and voice
• head titubation is seen
• tremor diminishes with alcohol
❏ treatment: propranolol, nadolol; primidone if ß-blocker contraindicated (diabetes, asthma), surgery
Intention Tremor (Cerebellar Tremor)
❏ seen in diseases of cerebellar outflow (worsens with alcohol)
• coarse tremor of limbs or head; absent at rest
• intention tremor worse at end point of movement
• may be associated with dysarthria, nystagmus and ataxia
❏ examination maneuvers: finger to nose testing, heel to shin testing

MCCQE 2002 Review Notes Neurology – N23

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 Week 12
Neuroscience Notes
Parkinson’s Disease (“Shaking Palsy”)

What is it?
- Onset:
o Insidious (Menacing/Unstoppable)
o In second half of life (mean age of onset = 55yrs)
o Progression is variable.
- Characterised by:
o Slowly progressive akinesia
o Rigidity (stiffness)
o Postural abnormality (Leaning forward, stiff, difficult to move, weight loss – difficult to swallow
without aspirating)
o Tremor
- 2nd most common neuro-degenerate disease after Alzheimer’s
- Prevalence:
o 1/1000 non-elderly
o 1/200 in elderly

Biochemistry – Basal Ganglia Dysfunction due to:
- Loss of Substantia Nigra (Dopamine makers) Neurons à Dopamine Deficiency in the Basal Ganglia.
o These neurons project to the striatum (Caudate & Putamen) – Part of the Basal Nuclei – Normally
involved in coordinating movements.
o 50-60% loss of Nigral neurons are required for symptoms.
o Dopamine is required by the basal ganglia to coordinate complex movements, therefore, a loss of
the basal ganglia’s function à symptoms of Parkinson’s.
- Unknown Aetiology
- Secondary Causes – eg. Illicit drug
- More Specifically:
o Without input from the Substantia Nigra, there’s no activation of Inhibitory Neurons of the Putamen,
meaning there’s No Inhibition of Inhibitory Neurons of the Globus Pallidus, Meaning Inhibitory
Neurons of the Globus Pallidus are unopposed in inhibiting Neurons of the VL-Thalamus that usually
activate the SMA (Supplementary Motor Area).
o à Supplementary Motor Area isn’t activated to Fine-Tune Movement.
- NB: Loss of Dopamine release in Striatum creates a NT Imbalance that favours ACh :. Anticholinergic Drugs
are used to treat Parkinson’s Disease.

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Symptoms:
- Most common presenting feature = Tremor:
o Usually unilaterally
o Present at rest
o Increased by emotion & stress
o ‘Pill rolling’ tremor (with fingers)
- Rigidity:
o Stiff muscles
o Cogwheel phenomenon (ratchet-like feeling during passive pronation)
- Slowness of movement
- Postural Chances
- Decline in intellectual function
- Depression
- à Inability to pick up small objects, cups, do up buttons, write in small font etc.

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Treatment:
- Aim: to try to increase the level of Dopamine in the brain, or to mimic the effect of Dopamine, or to relieve
competition of dopamine receptors by anticholinergics.
- Oral Levodopa (Dopamine – NB: L-dopa can cross the bbb, but dopamine can’t)
o Converted to dopamine in the brain by dopa-decarboxylase.
o However, if taken orally, less than 5 % reaches the brain as most is converted to dopamine
systemically. Therefore, it is usually administered with decarboxylase inhibitors to prevent
conversion in systemic.
- Dopamine Agonist:
o Drugs that mimic Dopamine, binding to Dopamine receptors.

NB: Remember, The Dopamine Deficit is only in the Striatum. Other Dopamine pathways are Unaffected. Hence
this poses a problem of side-effects with Dopamine supplementation & Dopamine Agonist drugs.

- Anticholinergic drugs – to prevent competition in the brain. (Loss of Dopamine release in Striatum creates a
NT Imbalance that favours ACh :. Anticholinergic Drugs are used to treat Parkinson’s Disease)
- Surgery
o Sterotactic – using instruments to make a lesion to the brain resulting in decreased symptoms.
o Today – rather than making a physical lesion, an electrical probe is inserted into the brain à ‘lesions’
the brain à stimulates response.
o However, the aim is to 1. Find the area of the brain you want to target, & 2. Find a way to get there
safely while minimising the damage to the brain.
o Benefits – Good control of tremor and on/off phenomenon – stimulator inhibits the sub-thalamic
nuclei (can also be used in some severe OCD’s and other psychiatric disorders)
o Disadvantage – dangerous, and only beneficial for a small subgroup of parkinsons patients.
- Physical Therapy
- Side effects:
o On off phenomenon – Symptoms range from either well controlled or poorly controlled at random
times
o Over time, Levodopa decreases in Efficacy & Side effects Increase.
o Increased severity of symptoms after a while of DOPA administration
o Therefore, treatment is delayed until symptoms are sufficient to impact on the person’s life.

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 Other Facets of Parkinson’s: By Speech pathologist

Psychosocial Impact:
- Myths:
o Only old people get it. – wrong - +10% of diagnoses are in people under 40yrs.
o They only get shaking – wrong – people have postural, balance, speech, swallowing, breathing
problems.
o Sufferers are unemotional and non-feeling – wrong – this is often due to inability to show facial
expression
- Personal Impact:
o Tremor
o Stooped posture
o Masklike face
o Rigidity
o Arms flexed at elbows & wrist
o Bradykinesia
o Postural/balance instability
o Short shuffling steps
o Other CNS symptoms:
§ Speech difficulties (Disarthria of speech
muscles)
§ Swallowing difficulties (dysphagia)
§ Masklike facial expression
§ Constipation/urinary problems
§ Sweating
§ Pain & sensory disturbances.
o Behavioural/Psychological:
§ Sleep disturbances
§ Fatigue (Fatigue very quickly)
§ Mood/depression
§ Anxiety & obsessiveness
§ Thought & memory disturbances (dementia in the end-stage)
§ Psychosis.
- Impact on Carers:
o Depression
o Financial stress
o Strained relationships
o Frustration
o Anger
o Change in roles
o Changed plans for the future
- Impact on families:
o Helplessness
o Financial stress
o Change in roles
o Frustration
o Resentment
- Impact on community:
o Loss of social capital
o Increased healthcare costs
o Increased social benefits
o Loss of productivity
o Ageing population.

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Role of Health Professionals:
- Role of the GP:
o Coordinate lifetime, holistic care
o Plan of management
o Give patient an idea of what the future may hold
o Aim of management:
§ Encourage healthy lifestyle
§ Help the family & patient
§ Manage symptoms as they arise
- Allied Health:
o Specialist (Neurologist)
o Geriatrician
o Physiotherapy
o Occupational therapy
o Speech path
o Social work
o Psychologist
- Other management issues:
o Conselling
o Continuing to work
o Continuing to drive
o Help for carers.

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 MOVEMENT DISORDERS . . . CONT.

Table 9. Comparison of Corticospinal vs. Extrapyramidal Lesions

Corticospinal Extrapyramidal
Muscle Tone Clasp-knife spasticity Rigidity (lead-pipe or cogwheel)
Hypotonia (cerebellar)
Distribution of Arm flexors and leg extensors Flexors and extensors of all limbs
Increased Tone
Involuntary Absent Tremor, chorea, athetosis, dystonia
Movements
Tendon Reflexes Increased Normal
Plantar Reflex Extensor Flexor
Paralysis or Present Absent
Weakness

PARKINSON’S DISEASE (PD)

❏ an idiopathic, slowly progressive, degenerative CNS disorder
❏ insidious onset between 40-70 years
Clinical Features
❏ characteristic symptoms and signs (usually asymmetric onset)
• Tremor (rest, pill-rolling, 4-7 Hz, can be suppressed by voluntary movement)
• Rigidity (lead pipe and cogwheeling)
• Akinesia/Bradykinesia
• Postural instability (festinating gait, retropulsion, falls)
❏ other features
• D - dysphagia, drooling, decreased voice
• G - gait: start hesitation, small shuffling steps, loss of arm swing
• E - eye: blepharoclonus (fluttering of closed eyelids), lack of blinking
• M - micrographia
• M - mask like face (hypomimia)
• S - subcortical dementia (apathy, forgetful, poor ability to use knowledge)
DDx
❏ therapeutic drugs: neuroleptics, metoclopramide
❏ toxins: MPTP (drug abusers), manganese, carbon disulfide, CO
❏ “Parkinson Plus” disorders
❏ Vascular Parkinson’s
❏ post-infectious: 1914 flu epidemic (encephalitis lethargica “Awakenings”)
❏ metabolic: Wilson’s
Pathology
❏ loss of dopaminergic nigrostriatal neurons in substantia nigra’s zona compacta
❏ dopamine neurons degenerate, upsetting normal balance between dopaminergic inhibition and cholinergic
excitation of striatal output (GABA) neurons, results in relative acetylcholine (ACh) excess
❏ loss of neurons in multiple other selected areas
❏ result is relative increase in GABAergic output from striatum
❏ Lewy bodies (eosinophilic intraneural inclusion granules)
• not specific to PD
Treatment
❏ deprenyl (MAO-B inhibitor) acts by blocking dopamine breakdown and may slow progressive course
❏ levodopa + peripheral decarboxylase inhibitor (i.e. Sinemet)
❏ dopamine agonists (bromocriptine, pergolide, ropinirole, promipexole) can be used as 1st line or as add on
therapy when levodopa responsiveness diminishes
❏ anticholinergics (benztropine, trihexyphenidyl) for tremor
❏ NMDA antagonists (amantadine)
❏ neurosurgical options (lesions or stimulators)
❏ therapeutic problems: orthostatic hypotension, sudden loss of therapeutic effect, wearing off, dyskinesia,
freezing, psychiatric (psychosis, paranoia)
Clinical Pearl
❏ In Parkinson’s disease, postural instability generally appears later in the course of
the disease. If postural instability occurs earlier consider alternative diagnosis
e.g. Parkinson’s plus, Vascular Parkinson’s.

N22 – Neurology MCCQE 2002 Review Notes

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 MOVEMENT DISORDERS . . . CONT.
Vascular (pseudo) Parkinsonism
❏ parkinson’s-like state due to multiple strokes
❏ imaging usually shows basal ganglia lacunae or white matter change
❏ generally occurs in elderly; associated with hypertension
❏ often have signs and symptoms of Parkinson’s below the waist (short stepped gait, start hesitation, freezing,
postural instability) but relative sparing above the waist (normal voice and facial expression, absence of
rest tremor and bradykinesia)
❏ poor response to L-dopa
❏ patients with true PD may also have cerebrovascular disease in addition their PD
❏ CT/MRI helpful in distinguishing vascular parkinsonsim from true PD
“PARKINSON PLUS” DISORDERS
Progressive Supranuclear Palsy (PSP)
(Steele, Richardson, Olszewski Syndrome)
❏ age of onset 50 to 70 years
❏ onset characterized by difficulty in balance, abrupt falls, ocular disturbances, slurred speech,
dysphagia, vague personality changes, depression, abnormal facial expression
❏ may take years for characteristic syndrome to fully develop
• supranuclear ophthalmoplegia - abnormality of vertical gaze
(if eyes are fixated on a target and neck is flexed and extended, full or 8 movements can be obtained)
• pseudobulbar palsy - UMN spastic weakness of pharyngeal musculature, slurred speech,
mouth held open, swallowing difficulties, exaggerated jaw jerk
• axial dystonia - gradual stiffening and extension of the neck
(contrast to Parkinson’s where neck is flexed)
• affected neurons in subthalamus, thalamus, basal ganglia, and peri-aqueductal grey
• pathology: neurofibrillary tangles (like in Alzheimer’s)
• L-dopa not very effective in PSP
• fatal within 2-5 years
• may have prominent dementia or normal cognition
Multiple System Atrophy
❏ includes striatonigral degeneration, sporadic olivopontocerebellar atrophy, Shy-Drager Syndrome
❏ Parkinsonian features
❏ early dysautonomia (orthostatic hypotension, impotence, bladder dysfunction, mottled cold hands)
❏ other features are cerebellar dyfunction, pyramidal tract signs, stimulus sensitive myoclonus of
hands and feet, extreme forward neck flexion, inspiratory stridor, dysarthria
❏ 20% respond to L-dopa initially, 13% sustained response
TREMOR
Definition
❏ rhythmic oscillatory involuntary movement about an axis
Rest Tremor
❏ slow (3-7 Hz), coarse, distal
❏ the characteristic tremor of Parkinsonism
❏ hands - pill rolling, alternating flexion/extension of fingers or hands,
alternating pronation/supination of forearms
❏ best examined with hands resting in lap, can be brought out by tasks of concentration
Postural and Action (Kinetic) Tremor
❏ fast (6-12 Hz), fine, usually upper limbs, head (titubation)
❏ seen best with arms and hands outstretched
❏ physiological
• always present, imperceptible to the eye
❏ exaggerated physiological
• anxiety, sleep deprivation
❏ drugs (e.g. theophylline, lithium, caffeine, amphetamines, decongestants)
❏ drug withdrawal (e.g. EtOH)
❏ hyperthyroidism, hypoglycemia
❏ essential tremor
• AD inheritance
• patient complains of shaking when carrying teacup, putting a glass to the mouth, or trying to drink soup
• affects handwriting, and voice
• head titubation is seen
• tremor diminishes with alcohol
❏ treatment: propranolol, nadolol; primidone if ß-blocker contraindicated (diabetes, asthma), surgery
Intention Tremor (Cerebellar Tremor)
❏ seen in diseases of cerebellar outflow (worsens with alcohol)
• coarse tremor of limbs or head; absent at rest
• intention tremor worse at end point of movement
• may be associated with dysarthria, nystagmus and ataxia
❏ examination maneuvers: finger to nose testing, heel to shin testing

MCCQE 2002 Review Notes Neurology – N23

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 NEUROLOGICAL Pathology:
PERIPHERAL NEUROPATHIES

- Diabetic Neuropathy:
o Aetiology:
Chronic Hyperglycaemia
Demyelination
& Arteriolosclerosis
o Pathology:
1. Hyperglycaemia Focal Osmotic Demyelination of Axons Exposure of axon
Affects SENSORY nerves first because they are the ones covered with myelin.
Remember Motor ne rons aren t co ered in m elin
2. Hyperglycaemia Arteriolosclerosis in Vasa-Nervorum (Nerve Blood Supply)
Ischaemic Neuropathy
o Morphology:
Arteriolosclerosis (Amyloid Thickening of the Basement Membrane of Capillaries)
Myelin Loss in Nerve Seen on Myelin Stain (Myelin Stains Black)
Diabetic Neuropathy Normal

o Clinical Features:
Distal, Symmetric Sensory Neuropathy (Paraesthesia, Loss of Sensation)
Autonomic Neuropathy
Progression:
o 1. Sensory Neuropathy
Glo e Stocking Paraesthesia Pain Night-Time Pain
Loss of Proprioception
Risk of Ulcers due to Chronic Painless Injuries.
NB: Bilateral, Symmetrical
o 2. Motor Neuropathy
Muscle Atrophy
3rd Nerve Palsy (Eye is Down & Out)
o 3. Autonomic Neuropathy
Postural Hypotension
GI Motilit Constipation Diarrhoea
Urine Retention/Urgency/Incontinence
Erectile Dysfunction

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- B12 Deficiency:
o Aetiology:
B12 Deficiency Due to:
Dietar Intake Eggs meat, milk, shellfish Ie. Vegetarian/Vegan)
Malabsorption Eg Pernicio s Anaemia GI S rger Coeliac Crohn s
Loss
o Pathogenesis:
B12 is necessary for Maintenance of the CNS
:. B12 Deficiency Demyelination, Axonal Oedema, Neuronal Sclerosis
Particularly Affects Spinal Cord:
(1) Dorsal Column ML Pathway (Sensory Paraesthesia)
o Vibration Proprioception fine to ch
(3) Corticospinal Pathway (Motor Weakness)

o Clinical Features:
Weakness and Paraesthesiae in the Lower Limbs
Loss of Balance
Megaloblastic Anaemia
o Treatment:
Supplemental B12

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 PERIPHERAL NEUROPATHIES
APPROACH TO PERIPHERAL NEUROPATHIES
❏ signs and symptoms
• onset, progression (acute, subacute, chronic)
• determine if motor, sensory, autonomic dysfunction
• if chronic motor, see muscle atrophy and absent reflexes
• if sensory, may have paresthesias in addition to sensory loss
• autonomic dysfunction includes anhydrosis, GI dysmotility, orthostatic hypotension,
impotence, bladder/bowel dysfunction, impairment of pupillary responses
• mononeuropathy vs. polyneuropathy
• proximal vs. distal
• upper vs. lower extremities
• fiber size selectivity
• large: motor weakness, loss of joint position, vibration, and touch/pressure
• small: pain and temperature loss, autonomic dysfunction
• relationships to systemic illness (DM, RA, EtOH)
• family history, especially with unexplained polyneuropathy from childhood (hereditary);
later life, paraneoplastic or paraproteinemic causation is more likely
• pes cavus, thickened nerves in hereditary causes
❏ lab investigations (based on clinical suspicion)
❏ electrodiagnostic studies (NCS, EMG, quantitative sensitivity testing)
• confirm neuropathy and elimination of non-neuropathic disorder
• localization of focal lesion, prediction of pathology
❏ nerve biopsy has limited use
• vasculitides, sarcoid, amyloid, inherited storage disorders

presentation

clinical/labs/EDS*

disorders mimicking PNS disease true PNS disease

- hyperventilation
- myopathy focal/multifocal neuropathies diffuse disease
- myasthenia gravis
- spinal muscular atrophy
- psychogenic demyelinating axonal
- cervical cord pathology polyneuropathy polyneuropathy
- other

mononeuropathy mononeuropathy acquired ––– uniform –––> hereditary acquired

- compression multiplex
- injury - ischemic nonuniform - systemic
(vasculitis) - inflammatory i.e. GBS - drugs/toxins
- infiltrative - non-inflammatory - nutritional
*EDS - electrodiagnostic studies

Figure 17. Assessment of Peripheral Neuropathies

Clinical Pearl
❏ Distal and asymmetric weakness with denervation and sensory
changes are characteristic of peripheral neuropathies.

N40 – Neurology MCCQE 2002 Review Notes

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 NEUROLOGICAL Pathology:
PRION DISEASES

- Eg. Creutzfeldt Jackob Disease, Gertsmann-Straussler Syndrome, Fatal Familial Insomnia, Kuru Kuru:
o Aetiology:
Prion Infection of the Brain
Prions Proteinaceous, Infectio s on
= Abnormally folded Host-Proteins that accumulate in the brain
NO DNA or RNA!! (Important for Exams)
Prion Proteins (PrP):
Normal Form = PrPc (Cellular)
o Normal -Helix form. (Functional & Denaturable)
o Found throughout the body (Also in mammals).
Abnormal Form = PrPsc (Scrapie)
o Abnormal -Sheet form. (Non-Functional & Non-Denaturable)
o Accumulates in plaques in the brain Tissue Damage & Cell Death.
o EXTREMELY STABLE – Resists denaturation :. Difficult disposal.
o Pathogenesis:
Prions cause Neurodegenerative Disease by aggregating Extra-Cellularly in the CNS form
amyloid plaques Plaques are Internalised Vacuole formation in Neurons Spongy
Architecture.
Propagation Conversion of Normal Proteins -helix -sheet):
Prions propagate by transmitting a Mis-Folded Protein State, not replicating.
Ie. They convert Pre-Existing, Normal forms of the protein to the Abnormal Form.
o Morphology:
Macro:
Empty cystic lesions in the brain Spongiform Encephalopathy
Micro:
Neuronal Vacuolation & Plaque Formation
o Clinical Features:
Initially Subtle Memory & Behavioural Changes Then Rapidly Progressive Dementia
Convulsions (Myoclonus)
Dementia
Ataxia, Dysarthria, Dysphagia, Nystagmus
Behavioural/Personality Changes
o Prognosis:
All known Prion Diseases affect the Brain and are currently Untreatable & Universally
Fatal
7mths life expectancy

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 NEUROLOGICAL Pathology:
RAISED INTRACRANIAL PRESSURE

Intracranial Pressure:
Normal ICP:
o 10mmHg
ICP leads to :
o Cerebral blood flow (Due to reduced perfusion pressure)
(Perfusion Pressure = Sys.BP Intracranial Pressure)
(NB: Perfusion only occurs when Perfusion Pressure is Positive)
o ICP may = Arterial Pressure?
If Arterial Pressure ICP then Perfusion Pressure
Nil Perfusion
Signs of Raised Intracranial Pressure:
o C hing Re pon e Refle C hing Triad
Hypertension
Bradycardia
Irregular Breathing
Treating Raised ICP:
o Osmotic Diuretics (Eg. Mannitol)
o Hyperventilation Hypocapnia Vasoconstriction of Cerebral Vessels
o Continuous CSF Drainage/Surgical CSF Shunt

NB DON T do a L mbar P nc re if Intracranial Pressure is High:

o If ICP is high, and you drain CSF Can ca se Coning
Aka Cerebral Herniation Aka Cistern Obliteration
Brain can herniated through the foramen magnum Puts extreme pressure on parts of
the brain and thereby cuts off their blood supply.
Is often Fatal
o Signs:
ALOC (GCS 3-5)
Vomiting (Compression of Emetic Centre in Medulla)
Can cause 3rd Nerve (Oculomotor) Palsy:
Ptosis = Unable to Open Eyelid (Levator Palpebrae Superioris)
Blo n P pils Dila ed Unresponsi e o Light.
Eye faces Downwards & Outwards
Decerebra e Pos ring (Abnormal Extension to Pain)

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CEREBRAL OEDEMA:
- Cerebral Oedema = Fluid Accumulation in the Intracellular and/or Extracellular Spaces of the Brain
- Aetiologies & Pathogeneses 4 Types:
o Vasogenic - Cap Permeabilit (Ie. Trauma, Ischaemia/Infarction, Infection/Inflammation)
o Cytotoxic Na & H2O Retention in Injured Neurons (Eg. From Hypoxia or Neurotoxin)
o Osmotic CSF Osmolality > Plasma Osmolality (Eg. Overhydration, Hyponatraemia)
o Interstitial Obstructive Hydrocephalus
- Clinical Features:
o (Features of Aetiology Fever if Meningitis, Concussion if Trauma, Stroke if Infarction, etc)
o Fea res of ICP
C shing s Triad - (Hypertension, Bradycardia, Cheyne-Stokes Respiration)
Headache
ALOC
Vomiting
Pupil Dilation
- Management:
o Osmotic Diuretics (Eg. Mannitol)
o Hyperventilation Hypocapnia Vasoconstriction of Cerebral Vessels
o Continuous CSF Drainage/Surgical CSF Shunt

Chronic In racranial Press re Due to Space-Occupying Lesions (See Wk 3 for Cerebral Oedema & Herniation):
- Aetiologies:
o *Space-Occupying Tumours
- Clinical Features of Raised Intracranial Pressure:
o Signs (Cushings Response/Reflex/Triad):
1. Hypertension
2. Bradycardia
3. Cheyne-Stokes Respiration
o Symptoms:
Headache; Drowsiness; Altered level of Consciousness (GCS 3-5)
Vomiting
Seizures
- Treating Raised ICP:
o Elevate Head 30-40o
o Hyperventilate Hypercapnia Vasoconstriction of Cerebral Vessels
o Neurosurgery (if Trauma Haemorrhage)
o Continuous CSF Drainage/Surgical CSF Shunt
o Osmotic Diuretics (Eg. Mannitol) Plasma Osmolarit Extracts Water from Brain Tissue.

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BRAIN HERNIATIONS:
- Aetiology An hing ha Ca ses ICP
o Eg. Cerebral Haemorrhage
o Eg. Cerebral Oedema
o Eg. Obstructive Hydrocephalus
o Eg. Space-Occupying Lesions

- Cerebellar Tonsil Hernia ion Coning

o Pathogenesis:
General ICP Herniation of Cerebellar Tonsils through Foramen Magnum
Compresses the Brainstem Brainstem Ischaemia Cardio/Respiratory-Centre
Dysfunction (Death)
o Specific Signs/Symptoms:
Decerebrate Posturing Abnormal E tension to Pain

ALOC (GCS 3-5)

One Both Pupils Blo n Dilated Unresponsi e to Light
Vomiting (Compression of Emetic Centre in Medulla)
- Uncal Hernia ion
o Pathogenesis:
Unilateral Lesion Lateral Herniation of the Uncus Inferomedial Temporal Lobe against
the midbrain Then, Inferior Uncal Herniation below the Tentorium Cerebelli.
o Specific Signs/Symptoms:
May compress the Occulomotor Nerve Third Ner e Pals
Ipsilateral Pupil Dilation & Unresponsive to Light
Ptosis = Unable to Open Eyelid (Levator Palpebrae Superioris)
Eye Down & Out
o NB: May Progress to Cerebellar Tonsil Herniation Coning Death

- S bfalcine Hernia ion

o Pathogenesis:
Frontal Lesion Posterio-Lateral Herniation of the Cingulate Gyrus (Medial Frontal Lobe)
under the Rigid Falx Cerebri.
o Specific Signs/Symptoms:
May compress the ACA Stroke Contralateral Para-Hemiplegia
Abulia (Frontal Dysexecutive Symptoms)

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 NEUROLOGICAL Pathology:
SPINAL CORD SYNDROMES

Revision of Spinal Cord Tracts:

Changes in Motor Reflexes with Spinal Cord Injury:

o Immediate Consequences:
Reflexes are conserved since they aren’t mediated by the brain.
(NB: Reflexes are only lost if the lesion is @ the level of that reflex)
o Consequences Over Time:
Muscle movement diminishes over a period of time
Due to Progressive Muscle Atrophy (not Nerve Atrophy)

Different Spinal Cord Lesions (Pathways Affected & Clinical Consequences):

o B o n-Sequard S nd ome
(seen if someone is stabbed in the back with a knife or shot with a handgun causing a hemi-
transection of the spinal cord)
Pathways Affected & Clinical Consequences:
Dorsal Column Medial Lemniscal Pathway
o Loss of Discriminative Touch & Proprioception
o (Ipsilateral to & below the level of the lesion)
Spinothalamic Tract
o Loss of somatosensation (Touch, Pain, Temperature)
o (Contralateral to & below the level of the lesion)
Lateral Corticospinal:
o Loss of voluntary movements
o (Ipsilateral to the side of the lesion. Because it decussates in the pyramidal
tracts)

o Anterior Spinal Artery Syndrome:

(Due to Lesion of the Anterior Spinal Artery. Eg. Diving injury)
Pathways Affected & Clinical Consequences:
Doesn’t affect the Dorsal Column Medial Lemniscal pathway.
o No loss of Discriminitive Touch & Proprioception
Doesn’t Affect the Corticospinal Tract
o Conserves Motor Function)
Affects Spinothalamic Tract
o Loss of Somaosensation Contralateral to & below the level of the lesion.

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o Central Cord Syndrome:
(Usually secondary to spinal trauma and, affects the centre of the spinal cord.)
Pathways Affected & Clinical Consequences:
Mainly Corticospinal Tracts
o Motor Impairment (Mostly in Upper Extremities)
o (Why? Motor Fibres supplying Upper limbs tend to be more Central than
those supplying the lower limbs)
Dorsal Column & Spinothalamic Tracts:
o Variable sensory losses below the Lesion.

o Dorsal Column Syndrome:

(Very Unusual)
Pathways Affected & Clinical Consequences:
Dorsal Column Medial Lemniscal Tracts:
o Ipsilateral Loss of Discriminitive Touch & Proprioception Below the Lesion
Doesn’t Affect Spinothalamic Tract:
o Somatosensation (Touch, Pain, Temperature) Unaffected.
Doesn’t Affect Corticospinal Tract:
o Motor Functions Conserved

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 DISEASES OF THE SPINAL CORD
fasciculus cuneatus

fasciculus gracilis

spinothalamic tract

spinocerebellar tract
corticospinal tract

posterior spinal artery

anterior
spinal artery

Figure 15. Anatomy of the Spinal Cord

Illustration by Aimée Warrell

CLINICAL FEATURES
❏ paraplegia or quadriplegia
❏ deficit corresponding to a sensory level
• T4-nipple, T10-navel, L5-dorsum of foot, S1-lateral foot
❏ bladder and bowel incontinence
❏ LMN signs at the level of the lesion and UMN signs below the level of the lesion (see Table 15)
❏ radicular symptoms (sharp, shooting radicular pain, dermatomal sensory loss)
• causes
• extradural: disc, trauma, cervical spondylosis, bone, tumour (metastases), abscess
• intradural, extramedullary: meningioma, neurofibroma, arachnoid cyst
• intradural, intramedullary: demyelination, inflammatory (transverse myelitis) tumour, infarct,
hemorrhage (AVM), or degeneration (subacute combined degeneration (SACD)), syrinx

Table 15. Comparison of UMN and LMN Lesions

Upper Motor Neuron (UMN) Lower Motor Neuron (LMN)
Bulk Normal (unless disuse) Muscle wasting
Tone Increased (spastic) Decreased
Fasciculations Absent Present
Weakness Pyramidal pattern Specific to lesion
Upper extremity: extensors weakest i.e. root, nerve
Lower extremity: flexors weakest
Reflexes Increased Decreased —> absent
Plantar Reflex Extensor Flexor

Clinical Pearl
❏ Spinal cord diseases usually cause a triad of: parasthesia at a sensory level (hallmark
symptom); bilateral corticospinal (spastic) weakness; bowel and bladder problems.

SPINAL CORD SYNDROMES (see Neurosurgery Chapter)

Brown-Sequard Syndrome
❏ lateral compression of one half of spinal cord (hemisection)
❏ ipsilateral LMN signs at level of lesion
❏ ipsilateral hemiplegia or monoplegia below lesion & UMN signs
❏ ipsilateral loss of vibration and proprioception below lesion
❏ contralateral loss of pain and temperature below lesion
❏ common causes – tumour, radiation

N38 – Neurology MCCQE 2002 Review Notes

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 DISEASES OF THE SPINAL CORD . . . CONT.
Central Cord Syndrome
❏ common causes are syringomyelia and intrinsic tumours
❏ suspended or cape sensory loss over shoulders from cervical lesion most common
❏ dissociated sensory loss
• loss of pain and temperature sensation with spared touch, joint position and vibration
❏ atrophy of intrinsic hand muscles (anterior horn cells),
Anterior Spinal Artery Syndrome
❏ sudden para/quadriplegia; initial flaccidity and areflexia (“spinal shock”);
within days develop UMN signs below lesion
❏ preserved vibration and position sense (spared posterior columns)
❏ due to occluded anterior spinal artery, usually at thoracic level
Subacute Combined Degeneration of the Spinal Cord
❏ corticospinal and posterior columns are affected e.g. vitamin B12 deficiency, HIV
❏ system degeneration therefore no distinct motor or sensory level
Conus Medullaris (Cauda Equina Syndrome)
❏ hypotonic bladder and rectal sphincters
❏ pain and loss of sensation in saddle distribution in perineum
❏ foot drop, absent ankle jerks
❏ causes: cord disc herniation, tumour, inflammatory
Foramen Magnum Syndrome
❏ quadriparesis, neck/head pain, hand atrophy +/– cerebellar signs
❏ cause: tumours
Transverse Myelitis
❏ back pain, rapid onset cord syndrome
❏ cause: post-infections, inflammatory, demyelination
Epidural Abscess
❏ severe back pain and tenderness, rapidly progressive cord syndrome
❏ may not have systemic signs/symptoms of infection
❏ surgical emergency!!!
Complete Transection of Cord
❏ trauma, tumour
❏ loss of all sensation and voluntary motor function below level (UMN)
MOTOR NEURON DISEASES
❏ diseases of cortical ––> anterior horn motor neurons
Spinal Muscular Atrophy (SMA)
❏ disorders beginning in infancy or childhood characterized by skeletal muscle wasting
due to progressive degeneration of cells in the anterior horn and medulla
❏ acute infantile SMA (Werdnig-Hoffmann disease)
❏ floppy baby, survival < 1 year
• childhood forms
• Wohlfart-Kugelberg-Welander disease
❏ adult forms
• proximal and distal muscles (may look like myopathy)
• slowly progressive, good prognosis
Amyotrophic Lateral Sclerosis (ALS)
❏ motor neuron disease of unknown etiology characterized by progressive degeneration
of corticospinal tracts and anterior horn cells or bulbar efferent neurons
❏ 10% familial
❏ onset age = 40-60 (very rare < 20)
❏ progressive, fatal 2-6 years (50% at 3 years)
❏ signs and symptoms
• no sensory findings
• fasciculations, muscle cramps
• segmental, asymmetrical weakness and atrophy
• upper and lower motor neuron signs (tonic atrophy)
• bulbar palsy, atrophy and tongue fasciculations
• dysarthria, dysphagia
• sparing of bowel and bladder function, and ocular muscles
❏ investigations
• EMG: diffuse denervation, fibrillation with normal nerve conduction
• Don’t miss high spinal cord lesion!
❏ treatment
• Riluzole in bulbar causes prolongs survival
• supportive treatment
SPINAL ROOT(see Neurosurgery Chapter)

MCCQE 2002 Review Notes Neurology – N39

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 NEUROLOGICAL Pathology:
STROKES

Strokes:
- TIA vs. CVA vs. Stroke:
o TIA = TRANSIENT ISCHAEMIC ATTACK Mini-Strokes = A brief stroke (<24hrs)(episode of
neurologic dysfunction) due to a temporary focal cerebral ischemia NOT associated with cerebral
infarction.
Typically Thromboembolic
Clinical Course:
Temporary Ischaemia, Resolves within 24hrs
o CVA = CEREBRO-VASCULAR ACCIDENT = Any cerebro-vascular pathology that leads to lack of blood
supply to the brain Stroke >24hrs
Clinical Course:
Evolving CVA = Increasing Ischaemia, Longer than 24hrs, Typically Thrombosis
Completed CVA= Complete Ischaemia, No Change, Typically Embolism
o Stroke = Rapid Loss of Brain Function s due to Disturbance in the Blood Supply to the Brain.
(Stroke is the clinical syndrome of a CVA)

- Common Causes of Stroke:

o 1. Ischaemic Strokes (Focal, Thrombo/Embolic):
Atherosclerosis
Rupture Thrombosis Cerebral Ischaemia Stroke.
Or Athero-Emboli in a Cerebral Artery Cerebral ischaemia Stroke
Heart Disease
Eg. Atrial Fibrillation Thrombo-Emboli Cerebral Ischaemia Stroke
o 2. Haemorrhagic Strokes Global ICP Hypoperfusion):
Hypertension
Blood vessel bursts under pressure Bleeding ICP Compresses other
Cerebral Arteries Blood Flo Cerebral Ischaemia Stroke.
Congenital Vascular Conditions
Eg. Congenital Berry Aneurysms Rupture Subarachnoid Haemorrhage
(Arterial) ICP Compresses other Cerebral Arteries Blood Flo
Cerebral Ischaemia Stroke.
Eg. Congenital AV Malformations Rupture Intracerebral Haemorrhage
(Arterial) ICP Compresses other Cerebral Arteries Blood Flo
Cerebral Ischaemia Stroke.
Trauma
Eg. Skull Fracture Extradural Haemorrhage (Arterial)
Eg. Low-Force Trauma Subdural Haemorrhage (Venous)

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 CAUSES OF ALOC VASCULAR:

TRANSIENT ISCHAEMIC ATTACK Mini-Strokes

- Aetiology Typically Transient Embolism:
o Thrombo-Embolism from Carotids
o Cardioembolism from a mural thrombus (Post-MI, AF, Valve disease, Prosthetic Valve)
- Pathogenesis:
o Thrombo-Emboli Lodged in Cerebral Artery Ischaemia Temporary Neurological Deficit (TIA)
When Blood Flow Returns, Neurons are still Functional Recovery
NB: Prolonged Ischaemia Infarction Stroke.
- Morphology:
o No Physical Changes Only Metabolic.
- Clinical Features:
o Signs/Symptoms:
Mimic those of stroke (But <24hrs)
Cerebral hemisphere Contralateral Hemiplegia
Brainstem Quadriplegia, Vision Disturbances etc
Lacunar (Basal Ganglia) Pure Motor, Pure Sensory etc
Emboli in Retinal Artery Amaurosis Fugax (Unilateral Descending Curtain of Blindness)
o DDX:
Hypoglycemia
Migraine Aura
Focal Epilepsy
Hyperventilation
Retinal Bleeds
- Investigations:
o Physical Examination:
Causes? Carotid Bruit, HT, Murmur, ECG (AF?), Fundoscopy
o Lab:
FBC, ESR, U&Es, Glucose, Lipids,
o Imaging:
CT Brain (Rule out Haemorrhagic Since Rx Contradict Each Other)
Carotid Doppler +/- Angiography
- Management:
o Control CV risk factors
Lower lipids (Simvastatin / Atorvastatin)
Stop smoking (Champix)
Lower BP (Perindopril/Candesartan +/- Atenolol)
Antiplatelet (Aspirin)
o Anticoagulation (Warfarin with Heparin Cover)
o +/- Carotid Endarterectomy

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80% ISCHAEMIC STROKE (Thrombo/Embolic Infarction):
- Aetiologies:
o 50% Thrombotic Infarct (Sudden Onset At Rest)
Eg. Rupture of Atherosclerotic Plaque
Eg. Hypercoagulable Syndromes (Eg. Oral Contraceptives, Clotting Disorders)
o 30% Embolic Infarct (Sudden Onset Following Exercise)
Eg. Embolus from Atherosclerosis (eg. From internal carotid)
Eg. AF Blood Stasis in Atria Thrombus Formation
Eg. Paradoxical Embolism (Embolus from DVT Through ASD CVA)
- Pathogenesis:
o Thrombosis/Embolism Focal Ischaemia Infarction Focal Neurology
- Locations:
o MCA (Most Common)
o ACA (Common)
o PCA (Rare - 4% clinically)
- Morphology:
o Early: Oedema (Narrow Sulci, Flattened Gyri)
o NB: Thrombolytic Therapy can Pin-point Haemorrhages around Capillaries
o 1wk: Liquefactive Necrosis & Cavitation
- Clinical Features (Depend on which arteries/functional areas are affected/occluded):
o Middle CA Stroke (#1 Most Common):
Contralateral Whole Body Hemiplegia (Primary Motor Cortex) +/- Dysarthria
Generalised Reduced Sensation (Primary Somatosensory Cortex)
Homonymous Hemianopia (Or sometimes Homonymous Quadrantonopia)
Expressive Aphasia If on LEFT (Dominant) Side Left Broca s Area

o Anterior CA Stroke (#2 Most Common)

Contralateral Lower-Limb Hemiplegia (Primary Motor Cortex)
Lower-Limb Numbness (Primary Somatosensory Cortex)
D se ecutive S ndrome Abulia Slo ness Prolonged Dela s to Perform Acts
Cognitive Impairment (Frontal)
Flat Affect (Limbic System)

o Posterior CA Stroke (#3 - Rare - 4% clinically)

Primarily Visual Defects
Memory Deficits (Short Term)

- Investigations:
o Clinical Examination
o FCB, Coags, Lipids
o CT Brain (Rule out Haemorrhagic)
- Treatment:
o Supportive (O2, Fluids)
o Rapid Reperfusion (Thrombolysis [Tissue Plasminogen Activator] +/- Thrombectomy)
o Anticoagulation (Clopidogrel/Aspirin + Warfarin with Heparin Cover)
o Stroke Rehabilitation (Speech Therapy, OT, Physio)
- Prognosis/Complications:
o 40% Mortality; 75% Mobidity (Eg. Hemiplegia, Aphasia, Dementia, Epilepsy, Mental Dysfunction)

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20% HAEMORRHAGIC STROKES (Bleeds):
INTRACEREBRAL HAEMORRHAGE ICH Haemorrhagic Stroke CVA :
o Aetiologies:
Head Trauma.
Congenital Arteriovenous Malformations
= Tufts of Blood Vessels where they shouldn’t be
Highly Susceptible to Rupture Intracerebral Haemorrhage & Cystic Change

Hypertension
Hypertension Vessels Burst Bleeding ICP Compresses other Cerebral
Arteries Blood Flo Cerebral Ischaemia Stroke.
Morphology:
o Slit Haemorrhages Microhaemorrhages heal as slits with pigment
o Lacunar Infarcts in the Brainstem Small cavity-like areas of pale infarcts

o Clinical Features:
Sudden onset Headache/Vomiting/Meningism
Anisocoria (Uneven Pupils), Nystagmus
Signs of ICP (Hypertension, Bradycardia & Cheyne-Stokes Respiration)
+ Potentially Fatal Herniation Syndromes (Cerebellar Tonsillar/Uncal/Subfalcine).
ALOC
+Focal Neurological Deficits:
o Investigations:
Head CT/MRI (Bleeding within the Brain or Ventricles)
Transcranial Doppler
o Management:
Supportive (Intubation, IV Fluids)
Medical:
Antihypertensives (B-Blocker, ACEi/ARB, Ca-Ch-Blocker)
Coagulation Factor VIIa
Mannitol (Osmotic Diuretic) ICP
Paracetamol Hyperthermia
FFP, Vit.K, Platelets (if Coagulopathy)
Corticosteroids Swelling
Surgical (If Haematoma >3cm)
o Prognosis:
>40% Mortality
75% of Survivors are Disabled

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 STROKE
❏ a clinical syndrome characterized by sudden onset of a focal neurological deficit presumed to be on a
vascular basis; avoid ‘CVA’ (‘confused vascular assessment’)
CLASSIFICATION
Ischemic Stroke (80%)
❏ ischemic stroke results from focal ischemia leading to cerebral infarction. Mechanisms include embolism
from heart or proximal arteries, small vessel thrombosis, or hemodynamic from a drop in the local perfusion
pressure. Global ischemia (e.g. from cardiac arrest or hypotension) causes a diffuse encephalopathy.
❏ ischemic strokes vary according to their size, anatomical location in the brain, and temporal pattern
Hemorrhagic Stroke (20%)
❏ abrupt onset with focal neurological deficits, due to spontaneous (non-traumatic) bleeding into the brain
❏ includes ICH and SAH
❏ subdural and extradural hemorrhages are not usually classified as strokes as they are associated with trauma
❏ hemorrhage into an area of cerebral infarction (commonly following cardiogenic embolism) is a hemorrhagic
infarct which should be considered an ischemic stroke complicated by secondary hemorrhage;
not a hemorrhagic stroke
STROKE TERMINOLOGY
Transient Ischemic Attack (TIA)
❏ stroke syndrome with neurological symptoms lasting from a few minutes to as much as 24 hours,
followed by complete functional recovery
Amaurosis Fugax, Transient Monocular Blindness (TMB)
❏ due to episodic retinal ischemia, usually associated with ipsilateral carotid artery stenosis or embolism of the
retinal arteries resulting in a sudden, and frequently complete, transient loss of vision in one eye
Reversible Ischemic Neurological Deficit (RIND)/Minor Stroke
❏ neurological abnormalities similar to acute completed stroke, but the deficit disappears after 24 - 36 hours,
leaving few or no detectable neurological sequelae (a better term is minor stroke)
Completed Stroke (CS)
❏ stroke syndrome with a persisting neurological deficit suggesting cerebral infarction; the ensuing neurological
defect can last days, weeks, or permanently; even after maximal recovery, at least minimal neurological
difficulties often remain
Progressing Stroke (Stroke In Evolution)
❏ neurological deficits begin in a focal or restricted distribution but over the ensuing hours spread gradually in a
pattern reflecting involvement of more and more of the particular vascular territory

MAKING THE COMPLETE DIAGNOSIS: “THE FOUR QUESTIONS”

❏ 1. Has the patient had a stroke?
• not all acute focal neurological deficits are 2º to stroke
• temporal profile may differentiate between TIAs, progressing stroke,
and minor and severe completed stroke
❏ 2. Where is the lesion and what is the blood supply?
• vascular territory: carotid vs. vertebrobasilar
❏ 3. What is the lesion?
• ischemia/infarction (with or without 2º hemorrhage)
• hemorrhage
❏ 4. What is the pathogenesis? (i.e. mechanism of the stroke)
• it will guide acute and chronic therapy

DDx: IS IT A STROKE?
❏ focal seizures
❏ other focal lesions: tumours, abscesses, subdural hematoma, demyelination, focal encephalitis
(herpes simplex)
❏ LMN lesions: Bell's Palsy, plexopathies, mononeuropathy
❏ previous cerebral infarction (i.e. focal signs are old)
❏ confusion, dementia and coma (without focal signs) are rarely modes of presentation for strokes
and usually suggests diffuse disturbance of cerebral function

MCCQE 2002 Review Notes Neurology – N49

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 STROKE . . . CONT.
WHERE IS THE LESION?
❏ see Figure 17 for vascular territories of major cerebral arteries
Hemispheric (see Figure 17 and Table 18)
❏ carotid territory (ACA, MCA)
❏ posterior cerebral arteries (vertebrobasilar supplies)

anterior cerebral artery

anterior cerebral artery

middle cerebral artery
middle cerebral artery

lenticulostriate
lenticulostriate arteries
arteries

anterior choroidal artery

posterior cerebral artery anterior choroidal artery

posterior cerebral artery

Figure 17. Vascular Territories of Major Cerebral Arteries

Illustration by Dr. P. Stewart
Table 18. Anterior vs. Middle Cerebral Arteries vs. Posterior Cerebral Arteries
* UE = upper extremities, LE = lower extremities

Anterior Cerebral Artery Middle Cerebral Artery Posterior Cerebral Artery

• Hemiplegia of LE • Hemiplegia of UE and face • Homonymous hemianopia or cortical
• Hemianesthesia of LE • Hemianesthesia of UE and face blindness (if bilateral)
• Incontinence • Hemianopia • If dominant hemisphere
• Grasp, snout, palmomental reflexes • Aphasia (if dominant hemisphere) - alexia without agraphia
• Behavioural and memory disturbances • Neglect of contralateral limbs • If thalamus
and constructional apraxia (if non-dominant hemisphere) - contralateral hemisensory loss
(if non-dominant hemisphere) - spontaneous pain
• Gaze preference (away from hemiparesis) • If subthalamic
- hemiballismus
• If midbrain
- ipsilateral CN III palsy
- contralateral motor deficit

Brainstem
❏ vertebrobasilar territory
• cranial nerves
• diplopia, gaze palsies, nystagmus
• vertigo
• dysarthria and dysphagia (sometimes hemispheric if patient hemiplegic)
• other cranial nerve palsies (III-XII)
• cerebellum
• ataxia
• incoordination
• crossed sensory loss (face and opposite side of body)
• bilateral motor deficits
Indeterminate
❏ ‘hemisyndromes’: hemiparesis, hemisensory loss, dysarthria
WHAT IS THE LESION?
Table 19. Hemorrhagic vs. Ischemic Stroke
Hemorrhage Infarct
Hypertension Usually present Often present
Preceding TIA No 30% of cases
Onset Often with activity Often at night or no activity, on waking
Course Rapidly progressive Static (rarely stepwise)
Increased ICP Yes No
CT scan Shows blood Normal or changes of infarction
• CT (or MRI) is the only reliable way to rule out hemorrhage
N50 – Neurology MCCQE 2002 Review Notes

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 STROKE . . . CONT.
WHAT IS THE PATHOGENESIS?
Atherosclerotic Plaque
❏ inadequate perfusion of brain due to
• an embolus from an atherosclerotic plaque in a large vessel (artery to artery embolus) (most common)
• a large vessel thrombosis with low distal flow
❏ risk factors
• hypertension (HTN)
• diabetes mellitus (DM)
• cigarette smoking
• high cholesterol
❏ treatment
• control atherosclerotic risk factors
• carotid endarterectomy in selected patients (see below)
• antiplatelet agents: aspirin, ticlopidine, clopidogrel, aggrenox (dipyridamole and ASA)
Cardiogenic Origin
❏ an embolus of clot
• risk factors: A fib (commonest cause), LV aneurysm, LV dysfunction, increased age,
mitral annulus calcification
❏ air emboli - during surgery or diving
❏ valvular vegetations (infection, tumour)
❏ treatment
• risk of embolization can be decreased with anticoagulation (heparin and warfarin)
• increase risk of hemorrhagic infarction implying that it is imperative to exclude the
presence of bleeding
prior to starting anticoagulants (i.e. do CT scan at 48 hours post-bleed)
• if moderate sized infarct, delay anticoagulation 5-14 days
Lacunar Infarction
❏ small (< 2 cm) and deep infarcts (lacune means lake)
❏ most < 5 mm, only 1% > 10 mm = "giant lacunes"
❏ pathology
• lipohyalinosis of small penetrating arteries of basal ganglia and brain stem; microatheroma;
junctional plaques (atherosclerosis of parent vessel blocking orifices of penetrating vessels)
❏ sites
• putamen
• internal capsule - pure motor
• thalamic - pure sensory
• pons - brainstrm signs
❏ clinical syndromes
• pure motor or pure sensory
• clumsy hand dysarthria
• ataxic hemiparesis
❏ risk factors
• HTN
• DM
• increasing age
❏ treatment
• control HTN
• use antiplatelet drugs
Other Causes
❏ large artery diseases (Moya Moya, Takayasu’s arteritis)
❏ dissection, trauma, vasculitis (PAN, meningovascular syphilis)
❏ coagulation/viscosity problems (especially in younger patients)
❏ venous infarction (cortical vein or sinus thrombosis)
• seen in "hypercoagulable states" (e.g. pregnancy, dehydration) and results in cortical infarction,
often complicated by 2º hemorrhage and seizures
Risk Factors for Stroke
❏ hypertension (HTN)
❏ smoking
❏ myocardial infarction (MI)
❏ atrial fibrillation (A fib)
❏ diabetes mellitus (DM)
❏ alcohol abuse
❏ homocysteinemia
❏ obesity
❏ severe carotid stenosis
❏ parental stroke (family history)

MCCQE 2002 Review Notes Neurology – N51

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 STROKE . . . CONT.
Investigations
❏ laboratory
• CBC, ESR, PT, PTT, VDRL, glucose, lipids and hypercoagulability work-up (Protein C, Protein S,
Factor V Leiden, anti cardiolipin antibody, lupus anticoagulant, PT/INR, PTT, anti-phospholipid
antibody and heparin cofactor II)
❏ neuroimaging
• CT, MRI, functional imaging (SPECT, PET)
• for acute stroke, unenhanced CT head is imaging method of choice
❏ cardiac
• ECG, echocardiogram (transesophageal), Holter monitor
❏ non-invasive studies
• duplex doppler of carotids, transcranial doppler to look at intracranial vessels, MR angiography
❏ angiography
Management
❏ Asymptomatic Carotid Bruit
• suggests the presence of atherosclerotic stenosis and signifies increased risk for both
cerebral and myocardial infarction
• modify risk factors, +/– antiplatelet therapy
• if stenosis > 60%, risk of stroke is 2% per year
❏ TIA, Mild Stroke
• investigate to determine the vascular territory and etiology, then treat accordingly for
atherosclerotic pathogenesis: manage risk factors and use antiplatelet agents - ASA, ticlopidine (Ticlid),
clopidrogel (Plavix), Persantine with ASA (Aggrenox)
• for carotid territory event, consider carotid endarterectomy by a good experienced surgeon if there is
severe ipsilateral, extracranial carotid stenosis (> 70% by angiography)
• if angiography shows 50-69% stenosis refer to stroke neurologist to assess indication
for carotid endarterectomy
❏ Acute Cerebral Infarction
• management goals
• ensure medical stability
• limit or prevent neuronal death
• avoid secondary complication of immobilization (e.g. pneumonia, pulmonary embolus)
• prevent recurrent cerebral infarction
• practical guidelines
• ensure the ABC's
• patient with vertebrobasilar ischemia or bihemispheric ischemia can have decreased
respiratory drive or muscular airway obstruction
• consider heparin in patients who are not eligible for tPA who have a larger artery atherosclerotic
stroke, progressing thromboembolic stroke, or cardioembolic stroke and who do not have a
large infarct, uncontrolled hypertension or bleeding condtions
• make the correct etiological diagnosis so you have a rational
approach for secondary prevention of stroke
• remember that MI is an important cause of morbidity and mortality in these patients;
screen for and manage the patient’s CAD
• consider transfer to stroke center if patient seen in first few
hours for neuroprotective or thrombolytic therapy (both under evaluation by clinical trials)
• consider thrombolysis if early in course (< 3 hours from onset)
• IV tPA if severe deficit, < 3 hours from onset and no evidence of hemorrhage on CT
• ineligible for tPA if
• history of intracranial hemorrhage
• major surgery within 14 days
• GI bleed within 21 days, head trauma or stroke within 3 months
• LP within 7days, rapidly improving
• BP greater than 185/110, seizures at onset
• symptoms suggest SAH, post MI pericarditis, pregnant, evidence of hemorrhage on CT
• PTT greater than 15s on warfarin, increased INR on heparin,
• platelets less than 100,000/mm3
• glucose less than 50 or greater than 400mg/dl
• severe anemia
• BP: DO NOT LOWER THE BP, avoid acute administration of anti-hypertensive agents;
unless hypertension is extreme
• most patients with an acute cerebral infarct are initially hypertensive and their BP
will fall spontaneously within 1-2 days
• acutely elevated BP is necessary to maintain brain perfusion
• anytihypertensive therapy is withheld for at least 10days after thromboembolic stroke unless
there is cardiac failure, aortic dissection, or a systolic above 220mmHg or a diastolic BP
above 120mmHg
• IV labetalol is usually first line

N52 – Neurology MCCQE 2002 Review Notes

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 STROKE . . . CONT.
• avoid hyperglycemia which will increase the degree of lactic acidosisin ischemic tissue,
increase the infarct size
• keep patient well hydrated, this will keep blood viscosity low and maintain perfusion of
ischemic tissue
• keep patient NPO if there is any hint of abnormal swallowing due to the risk of aspiration
• aspirin 325mg should be started within 48 hours of stroke onset, optimal dose uncertain,
no compelling evidence that any specific dose more effective
• give antiplatelets clopidogrel if ASA not suitable (e.g. Anaphylaxis, ulcers)
• clopidogrel (75mg/d) preferred over ticlopidine because of risk of neutropenia with ticlopidine
• start ambulation early, and if not feasible, use subcutaneous heparin to avoid DVTs
• glycoprotein IIB/IIIA platelet receptor antagonists, most powerful antiplatelet agents,
have been used in cardiac revascularization and are currently being evaluated in
ischemic stroke patients

Clinical Pearl
❏ The leading causes of death during the first month following a stroke are pneumonia,
pulmonary embolus, cardiac disease and the stroke itself.
❏ If a patient survives beyond the first week following a stroke, the cause of death is not
directly related to the stroke.

MULTIPLE SCLEROSIS
❏ a relapsing or progressive disease of CNS myelin characterized by disseminated patches of demyelination in
the brain and spinal cord, resulting in multiple and varied neurological symptoms and signs usually
with exacerbations and remissions
❏ lesions separated in time and space
Epidemiology
❏ onset usually 20-40, but can be younger or older
❏ F:M = 3:2
❏ prevalence in North America 1/1,000; most common in European races and
in countries farther from the equator
❏ genetic predisposition: 3% risk for first degree relatives, 30% concordance for identical twins,
HLA DR2 and Dw2 association
Etiology
❏ unknown but immunological and viral theories
Pathology
❏ multiple discrete lesions of myelin destruction (plaques)
❏ common plaque sites include optic nerve, periventricular areas, corpus callosum, brainstem, spinal cord
Course of Illness
❏ 5 Types
1. relapsing remitting (80% present this way initially, F>M)
2. primary progressive (gradually progressive clinical course from presentation, F=M)
3. secondary progressive (starts with relapsing remitting becomes progressive)
4. clinically inactive disease
5. mixed pattern
Signs and Symptoms
❏ signs: hyperreflexia, ataxia, nystagmus, spasticity and limb weakness
❏ symptoms: parasthesia, gait disorder, weakness (e.g. hemiparesis, paraparesis (myelopathy))
and incoordination, visual loss (optic neuritis) and diplopia, incontinence, fatigue
Common Features
❏ internuclear ophthalmoplegia (lesion in MLF causing failure of adduction of the ipsilateral eye and nystagmus
of the abducting eye on attempted lateral gaze)
❏ optic neuritis
❏ Lhermitte's sign (forward flexion of the neck causes electric shock sensation down the back to limbs,
indicative of cervical cord lesion)
❏ Uhthoff's phenomenon (worsening of symptoms with heat e.g. hot bath, exercise)
❏ trigeminal neuralgia in young patient
MCCQE 2002 Review Notes Neurology – N53

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 NEUROLOGICAL Pathology:
TRAUMATIC BRAIN INJURIES

Focal Primary Injury:

- CONCUSSION:
o Aetiology:
Moderate-Force Blunt Trauma to Head
o Pathogenesis:
Brain Trauma Metabolic/Ionic/Neurotransmitter Disruption Impaired
Neurotransmission
o Morphology:
Macro:
No structural damage
No visible Bleed
o Clinical Features:
Course = Acute, Temporary Unconsciousness (Secs-Mins) Normal Arousal
Symptoms:
Temporary Loss of function
Likely to fully recover (unless secondary injury)
Anterograde & Retrograde Amnesia Memor before after Injur
Headache
“Post Concussion Syndrome” 3wks Post injury
Memory Problems
Dizziness/Loss of Balance
Visual Disturbances/Photophobia
Tiredness
Sickness
Depression/Irritability/Restlessness
Rarely, Post-Traumatic Seizures
o Investigations:
History (Mechanism & Duration of LOC)
Concussion Grading Systems:
Grade I: Confusion, No LOC
Grade II: Confusion, Amnesia, No LOC
Grade III: Any LOC
Physical Examination
Neurological Examination
Including GCS
If GCS is <14 CT
o Management:
Usually Benign :. Just Supportive Treatment (Analgesics, Rest, Sleep, Avoid Drugs/Alcohol)
Avoid Further Head Trauma to Prevent “Second-Impact Syndrome” (Dangerous cerebral
oedema following second impact. Occurs days-weeks after an initial concussion)

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- CONTUSION:
o Aetiology:
Higher-Force Blunt Trauma to Head
(Often a “Contre-Coup injury” Brain Injury on the Opposite Side of Impact Due to
Rebound of the Brain)
(NB: “Coup Injuries” Brain Injury on the Side of Impact)
o Pathogenesis:
Higher-Force Trauma Coup &/or Contre-Coup Injury Bruising & Swelling of the Brain.
o Morphology:
Macro:
Contusion = Local Injury + haemorrhage
Some damage
Localised, Visible Injury with Bleeding (Bruising)
o Clinical Features:
Headache
Confusion/Sleepiness/Loss of Consciousness
Dizziness/Nausea/Vomiting
Cognitive Impairment
Sensory Impairment
Seizures
Ataxia
o Specific Investigations:
CT/MRI:
Focal Cerebral Oedema and often Surrounding Brain tissue
Transtentorial Herniation
o Management:
ICU management
Goal Treat ICP
Prevent Hypotension, Hyponatraemia, Hypercapnia.
May require surgical Intervention
Usually heal without other treatments.
o Prognosis:
Expect a reasonable recovery (but decreased memory, concentration; but still retain
normal function)

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- LACERATION:
o Aetiology:
Penetrating Head Trauma
An incised wound of brain tissue (Eg. Bullet/knife/etc)
o Pathogenesis:
Mechanical Destruction of Brain Matter due to Invading Object
Usually SEVERE damage
o Morphology:
Macro:
Visible tear in the tissue
Haemorrhage
o Clinical Features:
High Velocity:
Instant Death due to Blast Effect Immediate Supratentorial Pressure
Brainstem Herniation through Foramen Magnum.
Low Velocity:
May have Lucid Interval and No LOC
May have LOC as the laceration bleeds into the skull ICP
o Specific Investigations:
CT:
Frequently Associated with Skull Fractures &/or Diffuse Axonal Injury
Cerebral Laceration
Large amounts of Blood
o Management:
Pre ent ICP
o Prognosis:
Typically a Poor Prognosis.

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Diffuse Primary Injury:
- DIFFUSE AXONAL INJURY:
o Aetiology:
High-Force Blunt Trauma to Head
o Pathogenesis:
Shearing of neurons.
(Grey matter of whole areas of the brain have been sheared right off)
o Morphology:
Macro:
Small Haemorrhagic Lesions In the Corpus Callosum and Dorsolateral Brainstem
o Clinical Features:
Unconsciousness
Persistent Vegetative State (Coma) NB: 90% Never regain constiousness.
10% Regain Constiousness BUT significant mental impairment.
o Specific Investigations:
Difficult Doesn t sho up ell on CT/MRI.
CT may appear normal initially
May see small bleeds in Basal Ganglia/Corpus Callosum/Cerebral Cortex on MRI.
o Management:
No Specific Treatment Exists
(Stabilise Patient, & Control ICP)
o Prognosis:
Poor (Brain Damage GCS 3 Organ donor)

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 SENSORY Pathology:
VISION DISORDERS

Focal Disorders:
- Myopia (Short Sighted) Eye is too long
- Hyperopia (Far Sighted) Eye is too short

- Astigmatism:
o The lenses are not perfectly round (more football shaped)
o C ec ed b a T c (Football shaped) lens which is oriented in the opposite direction

- Presbyopia:
o As you get older, the ability to Accommodate gets less Presbyopia
Ie. A progressively diminished ability to focus on near objects with age.
Why? Because the lens loses its elasticity with age.
o Inability to focus on Near Objects (Similar to Hyperopia, but different aeitiology)
o Corrected by Corrected by Plus lenses.

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Glaucoma:
- = A State of Increased Intraocular Pressure
- Aetiology:
o Imbalance between Aqueous Humour Production & Drainage (via Canal of Schlemm)
- Pathogenesis:
o Imbalance between Production & Drainage IOP Damages Optic Nerve & Causes Retinal
Ischaemia Loss of Vision (Typically Peripheral Vision First)
- 3 Categories:
o De e d he A g e be ee he C ea The Iris Which determines patency of the Canal
of Schlemn)
o Open Angle Glaucoma:
The a g e f e, But the Canal of Schlemm is Blocked for another reason obstruction
to drainage IOP
NB: Progresses slower and vision loss may be insidious until the disease has progressed
significantly.
o Closed Angle Glaucoma:
The Angle is too acute Blocks Canal of Schlemn Drainage IOP
NB: Acute onset - Often Painful; visual loss can progress quickly but the discomfort often
leads patients to seek medical attention before permanent damage occurs.
o Congenital/juvenile
Hereditary
Infections can do it too.
- Acute Glaucoma Symptoms:
o Sudden onset headache, nausea, vomiting
o Loss of vision
o Red Eye
o Commonly Unilateral
o Pupils are Dilated
- Treatment:
o Pupillary Constrictors
o NB: Pupillary Dilation will make glaucoma worse; or precipitate glaucoma in predisposed
individuals. (Eg. At night)

Production of aqueous humour & drainage through canal of schlemn

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“Red Eye”
- Common Causes:
o Foreign bodies
o Conjunctivitis(baterial,viral,allergic)
o Sub-conjunctival haemorrhage
o Corneal abrasion
o Corneal ulcer(bacterial/viral)
o Uveitis
o Acute Glaucoma
- Questions to ask:
o Ascertain History of Injury:
Have you been welding
Have you been using contact lenses (notorious for causing problems)
Have you been handling acids/alkalines?
o Is it Uniocular or Binocular?:
If Binocular Probably conjunctivitis
If Monocular These are the ones to be concerned about:
Uveitis
Glaucoma
o Watery or Sticky?
If watery More concerning Eg. Uveitis, Glaucoma, corneal ulcer
If Sticky - Less concerning Prob Conjunctivitis
o Painful/Sensitive to light OR Just uncomfortable?
o (If Vision is Blurred & Painful, Flourescein is used to determine corneal staining)
- Red Flags:
o Unilateral
o Blurred vision
o Severe pain
o Photophobia
o Haloes.
- Common Causes of Red-Eye:
o Foreign Body:
Must know the mechanism of injury

o Subconjunctival haemorrhage:
Just a simple bruise
Common Causes
Coughing fit
Hypertensive
Anticoagulants
Requires no treatment but check BP hether the re on anticoagulants

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o Conjunctivitis:
Inflammation of conjunctiva
Sticky eyes common due to exudates (sometimes purulent)
NB: The cornea is nice and clear
Common Causative Organisms
Staph epiderm
Staph aureus
Strep
adenovirus

o Corneal Abrasion:
Diagnosed by Flourescine + Blue Light
Stains areas of epithelial loss
Must be able to distinguish between a simple corneal abrasion & a corneal ulcer
S perficial abra ion Heal i hin hr and don car

o Corneal Ulcers:
Require Urgent Specialist Care
Viral:
Commonest is Herpetic (Herpes Simplex Virus)
Characteristic feature = has branches :. A Dendritic Ulcer.

Bacterial:
More concerning
Most dangerous organism is Gonorrhea (Can penetrate the eye even without break
in the epithelium)
Pseudomonas Spreads Very Quickly Opaque (Most sight-threatening)

o Uveitis:
Very common cause of Red Eye
Inflammation of the entire uveal tract
Different from iritis
Very sensitive to light
Watery Eyes
NB: There is pus collecting in the anterior chamber.

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Diabetic Retinopathy:
- Change in basement membranes of the retinal capillaries:
o Microaneurysms
o Microvascular obstruction
o Non-perfusion of capillaries
o Narrowing of arterial walls

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Cataracts:
- Opacity in the crystalline lens
- Causes are multifactorial:
o Metabolic disease (eg. Diabetes)
o UV Light
o Smoking
o Ocular diseases (eg. Glaucoma)
o Skin diseases (eg. Dermatitis)
o Drug induced (Eg. Corticosteroids)
o Ageing (Idiopathic unknown cause)

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- Chalazion:
o A cyst in the eyelid that is caused by inflammation of a blocked meibomian gland, usually on the
upper eyelid.

- Strabismus:
o Where eyes are not properly aligned with each other.
o Very important to treat in children because it can cause Amblyopia (Where the brain ignores input
from the deviated eye. Can also be a cosmetic problem)

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SS Quiz Cases: - What is Most Likely in Each Patient?

1.A 23 yr old male presented to A&E with a red left eye .He was grinding metal at work.
- Foreign Body

2.A 13 yr old girl presented with complaints of a red right eye and blurred vision. She has also noticed some cold
sores around her lips.
- Herpetic ulcer

3.A 65yr old male presents with a painless red right eye following a coughing fit. He happens to be on
anticoagulants for a heart condition.
- Subconjunctival Haemorrhage

4.A 35yr old lady who wears contact lenses comes with a 2 day history of a painful red left eye. Her vision is
blurred and her eye is watery.
- Acanthomeba or Pseudomonas infection

5.A 27 yr old male comes with a 2 day history of red eyes. Both eyes are affected. He tells you that his vision
appears OK but they are sticky. He has recently had a sore throat.
- Conjunctivitis (Probably Bacterial)

6. 78 yr male presents with sudden loss of vision in the right eye.Over the past few months he has episodes of
loss of vision lasting for a couple of minutes. He has raised cholesterol.
- transient ischaemic attacks due to embolic atherosclerotic lesions lodging in the eye.

7. 80 yr old woman presents with blurring of central vision.She complains that whem she looks at your face she
cant see anything clearly but can see around it.She tries to read but all the letters are wavy and distorted.
- Age Related Macular Degenration

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 COMMON COMPLAINTS
Persistent Loss of Vision
❏ gradual (weeks to months)
• refractive error
• cataracts
• diabetes
• macular degeneration
• glaucoma (chronic)
• intracranial compressive lesion
• tumour infiltration
• toxic degeneration
❏ acute (minutes to days)
• vascular/ischemia
• cortical blindness
• central retinal vein/artery occlusion (CRVO/CRAO)
• vitreous hemorrhage
• optic neuropathy
• giant cell (temporal) arteritis
• anterior ischemic optic neuropathy (AION)
• optic neuritis
• multiple sclerosis (MS)
• papillitis or retrobulbar neuritis
• retinal detachment
• acute glaucoma
• acute iritis
Transient Loss of Vision (Amaurosis Fugax)
❏ transient ischemic attack (TIA), microemboli
❏ migrainous spasm of artery
❏ hypertension
Floaters
❏ physiologic; vitreous syneresis
❏ vitreous hemorrhage
❏ retinal detachment
Flashing Lights
❏ vitreous traction
❏ retinal tear/detachment
❏ migraine
Ocular Pain
❏ corneal abrasion, corneal ulcer, foreign body
❏ acute angle closure glaucoma
❏ acute uveitis
❏ scleritis, episcleritis
❏ optic neuritis
❏ differentiate from ocular ache: eye fatigue/asthenopia
Photophobia (Light Sensitivity)
❏ iritis
❏ meningitis, encephalitis
❏ light dispersion by mucus, lens, corneal opacities
❏ retinal degeneration
❏ acute glaucoma
Diplopia (Double Vision)
❏ binocular diplopia: strabismus, CN paresis (III,IV,VI), muscle entrapment
❏ monocular diplopia: dislocated lens, cataract, corneal scar

MCCQE 2002 Review Notes Ophthalmology – OP3

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 COMMON COMPLAINTS . . . CONT.

Red Eye
Table 1. Differential Diagnosis of Red Eye
Conjunctivitis Acute Iritis Acute Glaucoma Angle Closure Keratitis

Discharge Bacteria: pus No No Profuse tearing

Virus: serous
Allergy: mucous
Pain No ++ ++++ +++
(tender globe) With nausea With blinking
Photophobia No ++++ + ++
Vision Normal Reduced Reduced Varies with
(cloudy (corneal edema) site of lesion
aqueous)
Pupil Normal Smaller Fixed Same or smaller
in mid dilation

Clinical Pearl
❏ All red eyes are not necessarily conjunctivitis.

Table 2. Additional Features of Red Eye

Conjunctivitis Acute Iritis Acute Angle Closure Glaucoma

Injection Palpebral+bulbar Ciliary flush Diffuse

(limbal palor)

Intraocular Pressure (IOP) Normal Lower Increased

Ant.chamber Normal Cells/flare Shallow

Cornea Normal Keratic Steamy

precipitates

Other Preauricular node Synechiae Nausea/vomiting

(if viral)

❏ other causes of red eye

• adnexal and lacrimal system
• hordeolum/stye
• chalazion
• blepharitis
• dacryocystitis
• canaliculitis
• dacryoadenitis
• preseptal cellulitis
• orbital cellulitis
• conjunctiva
• subconjunctival hemorrhage
• pterygium, pinguecula
• sclera
• episcleritis
• scleritis
• cornea
• abrasion
• ulcer
• foreign body
• endophthalmitis

OP4 – Ophthalmology MCCQE 2002 Review Notes

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 COMMON COMPLAINTS . . . CONT.

COMMON OCULAR PROBLEMS

IN THE ELDERLY
❏ cataracts
❏ age-related macular degeneration (ARMD)
❏ epiphora
• overflow of tears
• due to dry eyes, entropion, ectropion, trichiasis
❏ neoplasia
❏ vascular disease: CRVO, CRAO, giant cell arteritis
❏ dry eyes
❏ ptosis
COMMON OCULAR PROBLEMS
IN CONTACT LENS WEARER
❏ corneal abrasion
❏ superficial punctate keratitis
❏ giant papillary conjunctivitis
❏ sterile infiltrates (immunologic)
❏ infected ulcers (Pseudomonas, S. aureus)

THE OCULAR EXAMINATION

suprachoroidal space
sinus venosus sclerae choroid
(canal of schlemm)
sclera
vitreous body visual part of retina
lens
fovea centralis
aqueous outflow hyaloid canal
sheath of optic nerve
anterior chamber
cornea posterior chamber central artery and vein of retina
iris
suspensory ligament optic nerve
anterior chamber angle of lens
lamina cribrosa of sclera
ciliary part of retina ciliary body and
conjunctiva ciliary muscle intervaginal space
ora serrata bulbar fascia (tendon’s capsule)
tendon of medial rectus muscle

Figure 1. Diagram of the Eye

Drawing by Luke Itani

VISION ASSESSMENT
❏ always note best corrected vision first (e.g. with glasses if possible), especially in emergency room
❏ test both near and distance vision
❏ pinhole test will improve vision with most refractive errors
Visual Acuity (VA) Far
❏ Snellen Fraction = testing distance (usually 6 metres or 20 feet)
smallest line patient can read on the chart
e.g. 20/40 -2 (missed two letters of 20/40 line)
❏ OD = RIGHT EYE, OS = LEFT EYE, OU = BOTH EYES
❏ Heirarchy for low vision - Snellen acuity – count fingers – hand motion – light perception – no light perception
❏ Legal blindness is 20/200 or < 10 degress of visual field in better eye
❏ Minimum visual acuity to operate an automobile is 20/40 in the better eye
Visual Acuity (VA) Near
❏ use pocket vision chart (e.g. Rosenbaum)
❏ record number and testing distance (usually 30 cm)
e.g. J2 @ 30cm
MCCQE 2002 Review Notes Ophthalmology – OP5

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 THE OCULAR EXAMINATION . . . CONT.

Visual Acuity for Children,Illiterate Adults or Dysphasics with CVA

❏ Sheridan-Gardiner matching test (most 4 year olds able to do it)
❏ illiterate "E" chart (patient states direction of “E”)
Infant Visual Acuity
❏ 6-12 months - 20/120
❏ 1-2 years - 20/80
❏ 2-4 years - 20/20
VISUAL FIELDS
❏ test visual field quadrants grossly by confrontation
❏ automated field testing (e.g. Humphrey, Goldmann), or Tangent Screen
PUPILS
❏ examine pupils with respect to
• equality, size, shape
• reactivity to light (both direct and consensual)
❏ test for relative afferent pupillary defect (RAPD)
• use "swinging flashlight test" (use ophthalmoscope with “+4” setting especially with dark brown iris)
❏ test pupillary constriction portion of near reflex
• bringing object from far to near results in
• lens accommodation
• eye convergence
• pupil constriction
• must use reduced illumination or pupillary constriction will be produced
ANTERIOR CHAMBER DEPTH
❏ shine light tangentially from temporal side
❏ shallow = > 2/3 of nasal iris in shadow
EXTRAOCULAR MUSCLES
Alignment
❏ examine in primary position of gaze (e.g. straight ahead)
❏ Hirschberg test (shine light into patient's eyes from 30 cm away)
• corneal light reflex should be symmetric and near centre of each cornea
❏ strabismus testing as indicated - see Strabismus section
Movement
❏ examine movement of eyeball through the nine diagnostic positions of gaze
(with six muscles responsible for extra-ocular movement (EOM))
❏ determine if diplopia is present in any position of gaze
❏ observe for nystagmus (horizontal and vertical)
EXTERNAL EXAMINATION
❏ the four L’s
• lymph nodes
• lacrimal apparatus
• lids
• lashes
SLIT-LAMP EXAMINATION
❏ systematically examine all structures of the anterior segment
• lids and lashes, including upper lid eversion if necessary
• conjunctiva and sclera
• cornea
• anterior chamber
• iris
• lens
❏ also examine with
• fluorescein staining: (water-soluble dye stains de-epithelialized cornea green),
with cobalt blue filter
• Rose Bengal dye (stains devitalized corneal epithelium)
❏ can examine structures in the posterior segment with special lenses (78D, 90D)

OP6 – Ophthalmology MCCQE 2002 Review Notes

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 THE OCULAR EXAMINATION . . . CONT.

TONOMETRY
❏ measurement of intraocular pressure
❏ normal range is 8-21 mm Hg
❏ commonly measured by
• indentation (Schiotz or Tonopen)
• applanation (Goldmann) – gold standard
• non-contact (air puff)
❏ use topical anesthetic for Schiotz, Goldmann, Tonopen
OPHTHALMOSCOPY/FUNDOSCOPY
❏ examination of the anterior segment
• fluorescein and colbat blue filter and +20 lens
• corneal opacity
• cataract
• the red reflex
❏ examination of the posterior segment of the eye
• vitreous
• optic disc (colour, cup, margins, cup/disc ratio)
• nasal retinal vessels
• retina, macula (temporal)
❏ best with pupillary dilatation (e.g. tropicamide) (see Table 6)
❏ contraindications to dilatation
• narrow anterior chamber angles
• neurologic abnormality requiring pupillary evaluation
• iris supported anterior chamber lens implant (square pupil)

OPTICS
❏ main refractive mechanisms are: cornea (2/3), lens (1/3)
EMMETROPIA
❏ no refractive error
❏ image of distant objects focused on the retina without accommodation
REFRACTIVE ERRORS (see Figure 2 and Figure 3)
❏ distant light is not focused on retina, without accommodation
❏ three types: myopia, hyperopia, and astigmatism
MYOPIA
❏ "nearsightedness"
❏ to remember: LMN (Long eyeball is Myopic, requiring negative/concave lens, and is Nearsighted)
Pathophysiology
❏ globe too long relative to refractive mechanisms or refractive mechanisms too strong
❏ image of distant object falls in front of retina without accommodation ––> blurring of distant vision
Presentation
❏ usually presents in 1st or 2nd decade, stabilizes in 2nd and 3rd decade;
rarely begins after 25 years except in diabetes or cataracts
❏ blurring of distance vision
❏ near vision usually unaffected
Complications
❏ retinal degeneration and detachment
❏ chronic open angle glaucoma
❏ complications not prevented with refractive correction
Management
❏ correct with concave negative (–) spectacles or contact lenses, which diverge light rays
❏ refractive eye surgery - see below
HYPEROPIA
❏ "farsightedness"
❏ hyperopia may be developmental, or may be due to any cause which shortens the eyeball
❏ to quantitate hyperopia, cycloplegic drops are used to prevent accommodation
Pathophysiology
❏ globe too short or refractive mechanisms too weak
❏ image of distant object falls behind retina without accommodation
❏ person will accommodate to try to bring image onto retina
MCCQE 2002 Review Notes Ophthalmology – OP7

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 OPTICS . . . CONT.

Presentation
❏ youth: usually do not require glasses (still have sufficient accommodative ability to focus image on retina),
+/– accommodative esotropia (see Strabismus section)
❏ 30s: blurring of near vision due to decreased accommodation, may need reading glasses
❏ > 50s: blurring of distance vision due to severely decreased accommodation
Complications
❏ angle closure glaucoma, particularly in later life as lens enlarges
Management
❏ when symptomatic, correct with convex positive (+) lenses, which converge light rays
❏ refractive eye surgery - see below
ASTIGMATISM
❏ light rays not refracted uniformly in all meridians
❏ due to non-spherical surface of cornea or non-spherical lens (e.g. football shaped)
❏ regular astigmatism: curvature is uniformly different in meridians at right angles to each other
❏ irregular astigmatism: distorted cornea, due to injury or keratoconus (cone-shaped cornea)
Management
❏ correct with cylindrical lens, toric contact lens, arcuate keratotomy or refractive eye surgery (see below)
PRESBYOPIA
❏ decreased ability of eye to accommodate with aging (decrease in lens elasticity, NOT a refractive error)
❏ experienced by emmetropes as well as patients with refractive errors
❏ normal decline in near vision with age (> 40 years) with distance spectacles in place
Presentation
❏ if initially emmetropic, starts holding things further away to read, but distance vision unaffected
❏ if initially myopic, remove distance glasses to read
❏ if initially hyperopic, symptoms of presbyopia occurs earlier;
the hyperope needs distance glasses in later decades
Management
❏ correct vision with convex positive (+) lens for reading
❏ reading lens will blur distance vision; options are half-glasses or bifocals
ANISOMETROPIA
❏ difference in refractive error between eyes
• second most common cause of amblyopia in children

Emmetropia Converges
Hyperopia corrected with positive lens

Myopia Hyperopia
Diverges
Figure 2. Refractive Errors and Emmetropia Myopia corrected with negative lens
Figure 3. Correction of
Refractory Errors

OP8 – Ophthalmology MCCQE 2002 Review Notes

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 COMMON COMPLAINTS
Persistent Loss of Vision
❏ gradual (weeks to months)
• refractive error
• cataracts
• diabetes
• macular degeneration
• glaucoma (chronic)
• intracranial compressive lesion
• tumour infiltration
• toxic degeneration
❏ acute (minutes to days)
• vascular/ischemia
• cortical blindness
• central retinal vein/artery occlusion (CRVO/CRAO)
• vitreous hemorrhage
• optic neuropathy
• giant cell (temporal) arteritis
• anterior ischemic optic neuropathy (AION)
• optic neuritis
• multiple sclerosis (MS)
• papillitis or retrobulbar neuritis
• retinal detachment
• acute glaucoma
• acute iritis
Transient Loss of Vision (Amaurosis Fugax)
❏ transient ischemic attack (TIA), microemboli
❏ migrainous spasm of artery
❏ hypertension
Floaters
❏ physiologic; vitreous syneresis
❏ vitreous hemorrhage
❏ retinal detachment
Flashing Lights
❏ vitreous traction
❏ retinal tear/detachment
❏ migraine
Ocular Pain
❏ corneal abrasion, corneal ulcer, foreign body
❏ acute angle closure glaucoma
❏ acute uveitis
❏ scleritis, episcleritis
❏ optic neuritis
❏ differentiate from ocular ache: eye fatigue/asthenopia
Photophobia (Light Sensitivity)
❏ iritis
❏ meningitis, encephalitis
❏ light dispersion by mucus, lens, corneal opacities
❏ retinal degeneration
❏ acute glaucoma
Diplopia (Double Vision)
❏ binocular diplopia: strabismus, CN paresis (III,IV,VI), muscle entrapment
❏ monocular diplopia: dislocated lens, cataract, corneal scar

MCCQE 2002 Review Notes Ophthalmology – OP3

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 COMMON COMPLAINTS . . . CONT.

Red Eye
Table 1. Differential Diagnosis of Red Eye
Conjunctivitis Acute Iritis Acute Glaucoma Angle Closure Keratitis

Discharge Bacteria: pus No No Profuse tearing

Virus: serous
Allergy: mucous
Pain No ++ ++++ +++
(tender globe) With nausea With blinking
Photophobia No ++++ + ++
Vision Normal Reduced Reduced Varies with
(cloudy (corneal edema) site of lesion
aqueous)
Pupil Normal Smaller Fixed Same or smaller
in mid dilation

Clinical Pearl
❏ All red eyes are not necessarily conjunctivitis.

Table 2. Additional Features of Red Eye

Conjunctivitis Acute Iritis Acute Angle Closure Glaucoma

Injection Palpebral+bulbar Ciliary flush Diffuse

(limbal palor)

Intraocular Pressure (IOP) Normal Lower Increased

Ant.chamber Normal Cells/flare Shallow

Cornea Normal Keratic Steamy

precipitates

Other Preauricular node Synechiae Nausea/vomiting

(if viral)

❏ other causes of red eye

• adnexal and lacrimal system
• hordeolum/stye
• chalazion
• blepharitis
• dacryocystitis
• canaliculitis
• dacryoadenitis
• preseptal cellulitis
• orbital cellulitis
• conjunctiva
• subconjunctival hemorrhage
• pterygium, pinguecula
• sclera
• episcleritis
• scleritis
• cornea
• abrasion
• ulcer
• foreign body
• endophthalmitis

OP4 – Ophthalmology MCCQE 2002 Review Notes

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 COMMON COMPLAINTS . . . CONT.

COMMON OCULAR PROBLEMS

IN THE ELDERLY
❏ cataracts
❏ age-related macular degeneration (ARMD)
❏ epiphora
• overflow of tears
• due to dry eyes, entropion, ectropion, trichiasis
❏ neoplasia
❏ vascular disease: CRVO, CRAO, giant cell arteritis
❏ dry eyes
❏ ptosis
COMMON OCULAR PROBLEMS
IN CONTACT LENS WEARER
❏ corneal abrasion
❏ superficial punctate keratitis
❏ giant papillary conjunctivitis
❏ sterile infiltrates (immunologic)
❏ infected ulcers (Pseudomonas, S. aureus)

THE OCULAR EXAMINATION

suprachoroidal space
sinus venosus sclerae choroid
(canal of schlemm)
sclera
vitreous body visual part of retina
lens
fovea centralis
aqueous outflow hyaloid canal
sheath of optic nerve
anterior chamber
cornea posterior chamber central artery and vein of retina
iris
suspensory ligament optic nerve
anterior chamber angle of lens
lamina cribrosa of sclera
ciliary part of retina ciliary body and
conjunctiva ciliary muscle intervaginal space
ora serrata bulbar fascia (tendon’s capsule)
tendon of medial rectus muscle

Figure 1. Diagram of the Eye

Drawing by Luke Itani

VISION ASSESSMENT
❏ always note best corrected vision first (e.g. with glasses if possible), especially in emergency room
❏ test both near and distance vision
❏ pinhole test will improve vision with most refractive errors
Visual Acuity (VA) Far
❏ Snellen Fraction = testing distance (usually 6 metres or 20 feet)
smallest line patient can read on the chart
e.g. 20/40 -2 (missed two letters of 20/40 line)
❏ OD = RIGHT EYE, OS = LEFT EYE, OU = BOTH EYES
❏ Heirarchy for low vision - Snellen acuity – count fingers – hand motion – light perception – no light perception
❏ Legal blindness is 20/200 or < 10 degress of visual field in better eye
❏ Minimum visual acuity to operate an automobile is 20/40 in the better eye
Visual Acuity (VA) Near
❏ use pocket vision chart (e.g. Rosenbaum)
❏ record number and testing distance (usually 30 cm)
e.g. J2 @ 30cm
MCCQE 2002 Review Notes Ophthalmology – OP5

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 THE OCULAR EXAMINATION . . . CONT.

Visual Acuity for Children,Illiterate Adults or Dysphasics with CVA

❏ Sheridan-Gardiner matching test (most 4 year olds able to do it)
❏ illiterate "E" chart (patient states direction of “E”)
Infant Visual Acuity
❏ 6-12 months - 20/120
❏ 1-2 years - 20/80
❏ 2-4 years - 20/20
VISUAL FIELDS
❏ test visual field quadrants grossly by confrontation
❏ automated field testing (e.g. Humphrey, Goldmann), or Tangent Screen
PUPILS
❏ examine pupils with respect to
• equality, size, shape
• reactivity to light (both direct and consensual)
❏ test for relative afferent pupillary defect (RAPD)
• use "swinging flashlight test" (use ophthalmoscope with “+4” setting especially with dark brown iris)
❏ test pupillary constriction portion of near reflex
• bringing object from far to near results in
• lens accommodation
• eye convergence
• pupil constriction
• must use reduced illumination or pupillary constriction will be produced
ANTERIOR CHAMBER DEPTH
❏ shine light tangentially from temporal side
❏ shallow = > 2/3 of nasal iris in shadow
EXTRAOCULAR MUSCLES
Alignment
❏ examine in primary position of gaze (e.g. straight ahead)
❏ Hirschberg test (shine light into patient's eyes from 30 cm away)
• corneal light reflex should be symmetric and near centre of each cornea
❏ strabismus testing as indicated - see Strabismus section
Movement
❏ examine movement of eyeball through the nine diagnostic positions of gaze
(with six muscles responsible for extra-ocular movement (EOM))
❏ determine if diplopia is present in any position of gaze
❏ observe for nystagmus (horizontal and vertical)
EXTERNAL EXAMINATION
❏ the four L’s
• lymph nodes
• lacrimal apparatus
• lids
• lashes
SLIT-LAMP EXAMINATION
❏ systematically examine all structures of the anterior segment
• lids and lashes, including upper lid eversion if necessary
• conjunctiva and sclera
• cornea
• anterior chamber
• iris
• lens
❏ also examine with
• fluorescein staining: (water-soluble dye stains de-epithelialized cornea green),
with cobalt blue filter
• Rose Bengal dye (stains devitalized corneal epithelium)
❏ can examine structures in the posterior segment with special lenses (78D, 90D)

OP6 – Ophthalmology MCCQE 2002 Review Notes

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 THE OCULAR EXAMINATION . . . CONT.

TONOMETRY
❏ measurement of intraocular pressure
❏ normal range is 8-21 mm Hg
❏ commonly measured by
• indentation (Schiotz or Tonopen)
• applanation (Goldmann) – gold standard
• non-contact (air puff)
❏ use topical anesthetic for Schiotz, Goldmann, Tonopen
OPHTHALMOSCOPY/FUNDOSCOPY
❏ examination of the anterior segment
• fluorescein and colbat blue filter and +20 lens
• corneal opacity
• cataract
• the red reflex
❏ examination of the posterior segment of the eye
• vitreous
• optic disc (colour, cup, margins, cup/disc ratio)
• nasal retinal vessels
• retina, macula (temporal)
❏ best with pupillary dilatation (e.g. tropicamide) (see Table 6)
❏ contraindications to dilatation
• narrow anterior chamber angles
• neurologic abnormality requiring pupillary evaluation
• iris supported anterior chamber lens implant (square pupil)

OPTICS
❏ main refractive mechanisms are: cornea (2/3), lens (1/3)
EMMETROPIA
❏ no refractive error
❏ image of distant objects focused on the retina without accommodation
REFRACTIVE ERRORS (see Figure 2 and Figure 3)
❏ distant light is not focused on retina, without accommodation
❏ three types: myopia, hyperopia, and astigmatism
MYOPIA
❏ "nearsightedness"
❏ to remember: LMN (Long eyeball is Myopic, requiring negative/concave lens, and is Nearsighted)
Pathophysiology
❏ globe too long relative to refractive mechanisms or refractive mechanisms too strong
❏ image of distant object falls in front of retina without accommodation ––> blurring of distant vision
Presentation
❏ usually presents in 1st or 2nd decade, stabilizes in 2nd and 3rd decade;
rarely begins after 25 years except in diabetes or cataracts
❏ blurring of distance vision
❏ near vision usually unaffected
Complications
❏ retinal degeneration and detachment
❏ chronic open angle glaucoma
❏ complications not prevented with refractive correction
Management
❏ correct with concave negative (–) spectacles or contact lenses, which diverge light rays
❏ refractive eye surgery - see below
HYPEROPIA
❏ "farsightedness"
❏ hyperopia may be developmental, or may be due to any cause which shortens the eyeball
❏ to quantitate hyperopia, cycloplegic drops are used to prevent accommodation
Pathophysiology
❏ globe too short or refractive mechanisms too weak
❏ image of distant object falls behind retina without accommodation
❏ person will accommodate to try to bring image onto retina
MCCQE 2002 Review Notes Ophthalmology – OP7

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 OPTICS . . . CONT.

Presentation
❏ youth: usually do not require glasses (still have sufficient accommodative ability to focus image on retina),
+/– accommodative esotropia (see Strabismus section)
❏ 30s: blurring of near vision due to decreased accommodation, may need reading glasses
❏ > 50s: blurring of distance vision due to severely decreased accommodation
Complications
❏ angle closure glaucoma, particularly in later life as lens enlarges
Management
❏ when symptomatic, correct with convex positive (+) lenses, which converge light rays
❏ refractive eye surgery - see below
ASTIGMATISM
❏ light rays not refracted uniformly in all meridians
❏ due to non-spherical surface of cornea or non-spherical lens (e.g. football shaped)
❏ regular astigmatism: curvature is uniformly different in meridians at right angles to each other
❏ irregular astigmatism: distorted cornea, due to injury or keratoconus (cone-shaped cornea)
Management
❏ correct with cylindrical lens, toric contact lens, arcuate keratotomy or refractive eye surgery (see below)
PRESBYOPIA
❏ decreased ability of eye to accommodate with aging (decrease in lens elasticity, NOT a refractive error)
❏ experienced by emmetropes as well as patients with refractive errors
❏ normal decline in near vision with age (> 40 years) with distance spectacles in place
Presentation
❏ if initially emmetropic, starts holding things further away to read, but distance vision unaffected
❏ if initially myopic, remove distance glasses to read
❏ if initially hyperopic, symptoms of presbyopia occurs earlier;
the hyperope needs distance glasses in later decades
Management
❏ correct vision with convex positive (+) lens for reading
❏ reading lens will blur distance vision; options are half-glasses or bifocals
ANISOMETROPIA
❏ difference in refractive error between eyes
• second most common cause of amblyopia in children

Emmetropia Converges
Hyperopia corrected with positive lens

Myopia Hyperopia
Diverges
Figure 2. Refractive Errors and Emmetropia Myopia corrected with negative lens
Figure 3. Correction of
Refractory Errors

OP8 – Ophthalmology MCCQE 2002 Review Notes

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 OPTICS . . . CONT.

REFRACTION
❏ technique of determining the lenses needed to correct the optical defects of the eye (ametropia)
❏ two techniques used
1) Flash/Streak Retinoscopy
• refractive error determined objectively by use of retinoscope and lenses
2) Manifest (Acceptance)
• subjective trial of lenses used to refine retinoscopy findings
• a typical lens prescription would contain
• sphere power in D (diopters)
• cylinder power in D to correct astigmatism
• axis of cylinder (in degrees)
• add (reading lens) for presbyopes
• e.g. –1.50 + 1.00 x 120 degrees, add +2.00
REFRACTIVE EYE SURGERY
❏ permanently altering the refractive properties of the cornea
❏ used for correction of myopia, hyperopia, and astigmatism
❏ most commonly using excimer laser system
❏ ablation of the corneal surface - photorefractive keratectomy (PRK)
❏ ablation of the stroma below a flap of corneal tissue (LASIK)

THE ORBIT
EXOPHTHALMOS (PROPTOSIS)
❏ eyeball protrusion
Etiology
❏ hyperthyroidism: Graves' disease (unilateral or bilateral, the most common cause in adults)
❏ orbital cellulitis (unilateral, most common cause in children)
❏ primary or secondary orbital tumours
❏ orbital/retrobulbar hemorrhage
❏ cavernous sinus thrombosis or fistula
❏ sinus mucoceles
Diagnosis
❏ exophthalmometer (Hertel): measure at lateral canthi
❏ CT head
ENOPHTHALMOS
❏ retracted globe
❏ often due to "blow-out" fracture (see Ocular Trauma section)
PRESEPTAL CELLULITIS
❏ infection of soft tissue anterior to orbital septum
Etiology
❏ stye, chalazion, acute meibomitis
❏ insect bite
❏ secondary to lid laceration or lacrimal system inflammation
Symptoms and Signs
❏ tender and erythematous lids
❏ may have adenopathy and fever
❏ normal VA, pupil, EOMs
❏ no exophthalmos or RAPD
Management
❏ topical and systemic antibiotics
❏ warm compress
ORBITAL CELLULITIS
❏ inflammation of orbital contents posterior to orbital septum
❏ common in children, but also in the aged and immunocomprimised
Etiology
❏ secondary to sinusitis, facial and/or tooth infections and trauma

MCCQE 2002 Review Notes Ophthalmology – OP9

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 GLAUCOMA
Definition
❏ progressive optic neuropathy involving characteristic structural changes to
optic nerve head with associated visual field changes
❏ commonly associated with high intraocular pressure (IOP)
Background
❏ aqueous is produced by the ciliary body and flows from the posterior chamber to the anterior chamber
through the pupil, and drains into the episcleral veins via the trabecular meshwork and the
canal of Schlemm (see Figure 1)
❏ an isolated increase in IOP is termed ocular hypertension (or glaucoma suspect) and these patients
should be followed because ~10% if 20-30 mmHg; 40% if 30-40 mmHg; most if > 40 mm Hg (or more)
will develop glaucoma
❏ normal, average IOP is 16 +/– 2 mm Hg (diurnal variation, higher in a.m.)
❏ pressures > 21 more likely to be associated with glaucoma
❏ normal C/D (cup/disc) ratio < 0.4
❏ suspect if glaucoma if C/D ratio > 0.6, difference between eyes > 0.2 or cup approaches disc margin
❏ loss of peripheral vision most commonly precedes central loss
❏ sequence of events: gradual pressure rise, followed by increased in C/D ratio, followed by visual field loss
as cup approaches disc margin (usually asymptomatic)
❏ screening tests should include
• medical and family history
• visual acuity testing
• slit lamp exam: to assess anterior chamber depth
• ophthalmoscopy: to assess the disc features
• tonometry by applanation or indentation: to measure the IOP
• Humphrey visual field testing
PRIMARY OPEN ANGLE GLAUCOMA
❏ most common form, > 55% of all glaucoma cases
❏ due to obstruction to aqueous drainage within the trabecular meshwork and its drainage
into the canal of Schlemm
❏ screening is critical for early detection
Risk Factors
❏ elevated intraocular pressure (> 21 mm Hg)
❏ age > 40
❏ higher incidence in blacks
❏ myopes
❏ familial, polygenic (10x increased risk)
❏ diabetes
❏ chronic topical steroid use on eyes in steroid responders
❏ previous ocular trauma
❏ anemia/hemodynamic crisis (ask about blood transfusions in past)
❏ hypertension
Symptoms and Signs (see Colour Atlas OP26)
❏ asymptomatic initially
❏ insidious, painless, gradual rise in IOP due to restriction of aqueous outflow
❏ bilateral, but usually asymmetric
❏ visual field loss
• slow, progressive, irreversible loss of peripheral vision
• arcuate scotoma and nasal step are characteristic
❏ late loss of central vision if untreated
❏ earliest signs are optic disc changes
• increased cupping of disc with a cup/disc ratio > 0.4 (the cup is usually enlarged asymmetrically
(i.e. eye more affected initially) and cup approaches disc margin)
• large vessels become nasally displaced
❏ may have hemorrhage at disc margin
❏ safe to dilate pupil
Management
❏ principles: increase the drainage of aqueous and/or decrease the production of aqueous
❏ medical treatment: see Ocular Medications section
• increases aqueous outflow
• topical cholinergics
• topical adrenergics
• topical prostaglandin agonist
• decreases aqueous outflow
• topical beta-blockers
• topical and oral carbonic anhydrase inhibitor
• topical adrenergics
❏ surgical treatment
• laser: trabeculoplasty, selective destruction of ciliary body
• microsurgery: trabeculectomy, tube shunt placement
❏ visual field testing to monitor course of disease
OP26 – Ophthalmology MCCQE 2002 Review Notes

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 GLAUCOMA . . . CONT.

PRIMARY ANGLE CLOSURE GLAUCOMA

❏ 12% of all glaucoma cases
❏ peripheral iris bows forward in an already susceptible eye with a shallow
anterior chamber obstructing aqueous access to the trabecular meshwork
Risk Factors
❏ hyperopia: small eye, big lens - large lens crowds the angle
❏ age > 70, female
❏ family history, more common in Asians and Inuit
❏ mature cataracts
❏ shallow anterior chamber
❏ pupil dilation (topical and systemic anticholinergics, stress, darkness)
Symptoms
❏ unilateral, but other eye predisposed
❏ RED FLAG: red, painful eye
❏ decreased visual acuity, vision acutely blurred from corneal edema
❏ halo around lights
❏ nausea and vomiting
❏ abdominal pain
Signs
❏ fixed mid-dilated pupil
❏ corneal edema with conjunctival injection
❏ marked increase in IOP even to palpation (> 40 mm Hg)
❏ shallow anterior chamber, +/– cells in anterior chamber
Complications
❏ irreversible loss of vision if untreated, within hours to days
❏ permanent peripheral anterior synechiae
Management
❏ immediate treatment important to
• preserve vision
• prevent adhesions of peripheral iris to trabecular meshwork (peripheral anterior synechiae)
resulting in permanent closure of angle
❏ medical treatment: see Ocular Medications section
• miotic drops (pilocarpine)
• topical beta-blockers
• systemic carbonic anhydrase inhibitors
• systemic hyperosmotic agents (oral glycerine; IV hypertonic mannitol)
• topical steroids (not in primary care)
❏ surgical treatment (for acute angle closure glaucoma)
• laser iridotomy or surgical iridectomy
SECONDARY OPEN ANGLE GLAUCOMA
❏ increased IOP secondary to ocular/systemic disorders which clog the trabecular meshwork
❏ steroid-induced glaucoma
• due to topical/systemic corticosteroid use
• develop in 25% (higher in extended use) of general population (responders) after 4 weeks (or less)
of QID topical steroid use
• 5% of population are super-responders
❏ traumatic glaucoma
• hyphema-induced increase in IOP
• angle recession glaucoma occurs with blunt, non-penetrating trauma to globe and orbit,
causing tears in trabecular meshwork and ciliary body with secondary scarring
❏ pigmentary dispersion syndrome
• iris pigment clogs trabecular meshwork
• typically seen in younger myopes
❏ pseudoexfoliation syndrome
• abnormal basement membrane-like material clogs trabecular meshwork
• seen mostly in the elderly
❏ neovascular glaucoma
• abnormal blood vessels develop on surface of iris (rubeosis iridis)
• due to retinal ischemia associated with proliferative diabetic retinopathy and CRVO
• treatment with laser therapy to retina, to reduce neovascular stimulus to iris vessels

MCCQE 2002 Review Notes Ophthalmology – OP27

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 GLAUCOMA . . . CONT.

SECONDARY ANGLE CLOSURE GLAUCOMA

❏ uveitis
• inflamed iris adheres to lens (posterior synechiae)
NORMAL PRESSURE GLAUCOMA
❏ IOP in normal range but cupping and field loss typical of glaucoma are present
❏ often found in women > 60 but may occur earlier
❏ may see splinter hemorrhages of disc margin
❏ damage to optic nerve may be due to vascular insufficiency
Management
❏ treat any underlying medical condition and lower the IOP still further
CONGENITAL GLAUCOMA
❏ due to inadequate development of the filtering mechanism of the anterior chamber angle
Symptoms and Signs
❏ cloudy cornea, increased IOP
❏ photophobia, tearing
❏ buphthalmos (large eye), blepharospasm
Management
❏ filtration surgery is required soon after birth to prevent blindness

PUPILS
PUPILLARY LIGHT REFLEX (see Figure 4)
❏ light shone directly into eye travels along optic nerve (1) to optic tracts (2) to both sides of midbrain
❏ impulses enter both sides of midbrain via pretectal area (3) and Edinger-Westphal nuclei (4)
❏ nerve impulses then travel down both CNs III (5) to reach the ciliary ganglia (6),
and finally to the iris sphincter muscle, which results in direct and consensual light reflex

1. optic nerve
2. optic tracts
3. pretectal area
4. Edinger-Wesphal nuclei
5. cranial nerve III
6. ciliary ganglia
LGB = lateral geniculate body
1
6 6

5
2 2

4 4
3 3

LGB LGB

Figure 4. Pathway of direct light reflex from left eye and

consensual light reflex from right eye

Illustration by Aimée Worrell

OP28 – Ophthalmology MCCQE 2002 Review Notes

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 LENS
❏ consists of an outer capsule surrounding a soft cortex and a firm inner nucleus
CATARACTS
❏ lens opacity
❏ most common cause of reversible blindness
Etiology
❏ increased age is the most common cause
• includes nuclear sclerosis, cuneiform (spoke-like), cortical and posterior subcapsular cataracts
❏ congenital
• presents with leukocoria
• treat promptly to prevent amblyopia
❏ juvenile onset: diabetes mellitus, metabolic abnormalities
(e.g. Wilson's disease, galactosemia, homocystinuria)
❏ medication toxicity
• steroids: posterior subcapsular cataracts
• phenothiazines: anterior subcapsular cataracts
• hypocalcemia: zonular cataracts (rare)
❏ inflammatory: uveitis
❏ radiation, UV light
❏ traumatic: typically leaf-shaped or rosette cataracts, may see subcapsular cataracts
Symptoms and Signs (see Colour Atlas OP7)
❏ gradual, progressive, painless decrease in visual acuity
• “second sight” phenomenon: only in cataracts due to nuclear sclerosis
• cataract increases power of lens causing artificial myopia - patient may read without reading glasses
❏ halos around lights at night, double and triple images
❏ diagnose by slit-lamp exam and by eliciting red reflex with direct ophthalmoscope
❏ if severe enough may not be able to see fundus on ophthalmic exam
Management
❏ surgical extraction of the cataract
❏ phacoemulsification
• more common
• remove cataract nucleus and cortex, leaving the posterior capsule and a
peripheral rim of anterior capsule
• less commonly by extracapsular nuclear expression and leaving the
posterior capsule behind
• leaving an intact posterior capsule confines the vitreous to the posterior
segment, thereby reducing the incidence of retinal detachments and macular edema
• allows for posterior chamber intraocular lens, lowering risk of hyphema,
glaucoma and corneal endothelial damage
• approximately 5-30% get an "after-cataract" (opacification of posterior capsule)
which is treated with YAG laser capsulotomy
❏ intracapsular: (now rarely used)
• remove whole lens with the cataract still in the capsule
• use with anterior chamber intraocular lens, glasses, or contact lenses
❏ post-operative complications: retinal detachment, endophthalmitis, opacified
posterior capsule, corneal edema secondary to endothelial damage, macular edema
Indication for Surgery
❏ absolute indications: trauma, congenital cataracts
❏ relative indications: age related (elective surgery when cataract interferes with daily living)
DISLOCATED LENS ‘ECTOPIA LENTIS’
Etiology
❏ associated with Marfan's Syndrome, Ehlers-Danlos type VI, homocystinuria, syphilis,
lens coloboma (congenital cleft due to failure of ocular adnexa to complete growth)
❏ traumatic
Symptoms and Signs
❏ decreased visual acuity
❏ may get unilateral diplopia
❏ iridodenesis (quivering of iris with movement)
❏ direct ophthalmoscopy may elicit abnormal red reflex
Complications
❏ cataract, glaucoma, uveitis
Management
❏ surgical correction +/– lens replacement

MCCQE 2002 Review Notes Ophthalmology – OP21

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 OPTICS . . . CONT.

REFRACTION
❏ technique of determining the lenses needed to correct the optical defects of the eye (ametropia)
❏ two techniques used
1) Flash/Streak Retinoscopy
• refractive error determined objectively by use of retinoscope and lenses
2) Manifest (Acceptance)
• subjective trial of lenses used to refine retinoscopy findings
• a typical lens prescription would contain
• sphere power in D (diopters)
• cylinder power in D to correct astigmatism
• axis of cylinder (in degrees)
• add (reading lens) for presbyopes
• e.g. –1.50 + 1.00 x 120 degrees, add +2.00
REFRACTIVE EYE SURGERY
❏ permanently altering the refractive properties of the cornea
❏ used for correction of myopia, hyperopia, and astigmatism
❏ most commonly using excimer laser system
❏ ablation of the corneal surface - photorefractive keratectomy (PRK)
❏ ablation of the stroma below a flap of corneal tissue (LASIK)

THE ORBIT
EXOPHTHALMOS (PROPTOSIS)
❏ eyeball protrusion
Etiology
❏ hyperthyroidism: Graves' disease (unilateral or bilateral, the most common cause in adults)
❏ orbital cellulitis (unilateral, most common cause in children)
❏ primary or secondary orbital tumours
❏ orbital/retrobulbar hemorrhage
❏ cavernous sinus thrombosis or fistula
❏ sinus mucoceles
Diagnosis
❏ exophthalmometer (Hertel): measure at lateral canthi
❏ CT head
ENOPHTHALMOS
❏ retracted globe
❏ often due to "blow-out" fracture (see Ocular Trauma section)
PRESEPTAL CELLULITIS
❏ infection of soft tissue anterior to orbital septum
Etiology
❏ stye, chalazion, acute meibomitis
❏ insect bite
❏ secondary to lid laceration or lacrimal system inflammation
Symptoms and Signs
❏ tender and erythematous lids
❏ may have adenopathy and fever
❏ normal VA, pupil, EOMs
❏ no exophthalmos or RAPD
Management
❏ topical and systemic antibiotics
❏ warm compress
ORBITAL CELLULITIS
❏ inflammation of orbital contents posterior to orbital septum
❏ common in children, but also in the aged and immunocomprimised
Etiology
❏ secondary to sinusitis, facial and/or tooth infections and trauma

MCCQE 2002 Review Notes Ophthalmology – OP9

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