DEFINISI DAN KLASIFIKASI
TERBARU EPILEPSI
dr. Endang Kustiowati, Sp.S (K) MSi.Med
Bagian / SMF Neurologi Universitas Diponegoro
RSUP Dr. Kariadi- Semarang
Definisi ILAE
Epilepsi Bangkitan Epilepsi
Bangkitan Non
Status Epileptikus
Epilepsi
ILAE OFFICIAL RE
PORT
Epilepsy is a disease of the brain defined by any of the following conditions
1. A least two unprovoked (or reflex) seizures occurring >24 h apart
2. One unprovoked (or reflex) seizure and a probability of further seizures similar to
the general recurrence risk (at least 60%) after two unprovoked seizures, occurring
over the next 10 years
3. Diagnosis of an epilepsy syndrome
Epilepsy is considered to be resolved for individuals who had an age-dependent epilepsy s
yndrome but are now past the applicable age or those who have remained seizure-free for t
he last 10 years, with no seizure medicines for the last 5 years.
AAN Guideline
Conclusion:
Adults with an unprovoked first seizure should be informed
that seizure recurrence risk is greatest early within the first 2
years (21%–45%) (Level A), and
clinical variables associated with increased risk may include:
– a prior brain insult (Level A),
– an epileptiform EEG (Level A),
– an abnormal CT/MRI(Level B)
– a nocturnal seizure (Level B).
Courtesy of Jacqueline French
AAN Guideline
• Immediate antiepileptic drug (AED) therapy, as compared wi
th delay of treatment pending a second seizure, is likely to red
uce recurrence risk within the first 2 years (Level B)
• Clinicians’ recommendations whether to initiate immediat
e AED treatment after a first seizure should be based on i
ndividualized assessments that weigh the risk of recurrenc
e against the adverse events of AED therapy.
Courtesy of Jacqueline French
DEFINISI
Bangkitan epileptik / epileptic
seizure :
Manifestasi klinis yang disebabkan oleh aktifitas listrik
sel-sel otak yang abnormal dan berlebihan
Bangkitan non epileptik :
Manifestasi klinis yang TIDAK disebabkan oleh aktifitas
listrik sel - sel otak yang abnormal dan berlebihan
Bangkitan refleks
Kondisi dimana kejang/bangkitan dapat dipresipitasi oleh
stimulus eksternal maupun internal yang selalu serupa.
Kilatan cahaya, membaca buku, mendengar lagu, makan,
sikat gigi, buang air kecil. Dll
Reflex Epilepsies (ILAE)
• Despite the fact that seizures are “provoked” in reflex epilepsies, these are
considered epilepsy, because…
• If the seizure threshold were not altered, these precipitants would typically
not cause seizures
– e.g., photosensitive epilepsy, eating epilepsy
These are not Epilepsy because there is small risk of a
seizure in the absence of a precipitating factor
• Febrile seizures in children age 0.5 – 6 years old
• Alcohol-withdrawal seizures
• Metabolic seizures (sodium, calcium, magnesium, glucose, oxygen)
• Toxic seizures (drug reactions or withdrawal, renal failure)
• Convulsive syncope
• Acute concussive convulsion
• Seizures within first week after brain trauma, infection or stroke
Manifestasi Klinis Bangkitan Epileptik
Gangguan kesadaran
•
• Gerakan Motorik
Area otak yan
• Sensoris g mengalami
• Emosi gangguan
• Perilaku atau persepsi
Purpose of ILAE Classification of Seizures and
Epilepsies
• To Provide common terminology and common class
ifications schemes throughout he world
• Largely for clinical purpose
• To organize items according to their fundamental
relationships
Klasifikasi Bangkitan Epilepsi
Bangkitan Parsial Bangkitan Umum Bangkitan Tidak
Terklasifikasi
Bangkitan Parsial
Sederhana Bangkitan lena
(absence seizures)
Epilepsia 1981: 22 : 489-501
Manifestasi motorik
Manifestasi sensorik Bangkitan
Manifestasi autonomik mioklonik
Manifestasi psikik
Bangkitan
Bangkitan Parsial klonik
Kompleks
Bangkitan
Parsial sederhana diikuti gangguan tonik
kesadaran
Dari awal dgn gangguan kesadaran Bangkitan
atonik / astatik
Bangkitan Parsial yang menjadi Bangkitan
Umum Sekunder tonik – klonik
www.t
Classification by ILAE 1989 heme
Epilepsy Syndrome galler
y.com
I. Localisation-related (f II. Generalised e
ocal-local, partial) epile
pilepsies and sy
psies and syndromes
ndromes
III. Epilepsies and sy
ndromes undetermi IV. Special
ned whether focal o syndromes
r generlised
Klasifikasi type Bangkitan – ILAE 1981
Unsclassified
Replace old “secondarily
“Generalized tonic-clonic seizure”
Fisher R et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia, **(*):1–12, 2017
Classification not hierarchical
• Can skip level
• Arrows intentionally omitted
Fisher R et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia, **(*):1–12, 2017
Note : when a seizure begins with
“focal, generalized or absence then th
e word “onset” can be presumed
Fisher R et al. Instruction manual for the ILAE 2017 operational classification of seizure types. Epilepsia, **(*):1–12, 2017
ILAE 2017 Classification Of Seizure Type Expanded Version
• Not include level of awareness
for atonic seizures or epileptic
spasm
• Cognitive seizures
– Impaired language
– Other cognitive domains
– Positive features : Déjà vu,
hallucination, perceptual
distortions
• Emotional seizures
– Ansiety, fear, joy, etc
ILAE 2017 Classification Of Seizure Type Expanded Version
• Focal aware/ impaired
awareness seizure my
than be classified by first
prominent sign or
symptom in seizure
• Eg.
Focal impaired
awareness
automatism seizure
Simply : focal impaired
awareness seizure
Focal reconceptualized
• For seizures:
– Focal epileptic seizures are conceptualized as
originating within networks limited to one
hemisphere. These may be discretely localized
or more widely distributed.…
Focal seizures
Blume et al, Epilepsia 2001
• Without impairment of consciousness or awareness
– Previous term: simple partial
– With observable motor or autonomic components
• eg. focal clonic, autonomic, hemiconvulsive
– With subjective sensory or psychic phenomena
• Aura - specific types
• Where alteration of cognition is major feature
– Previous term: complex partial
Focal seizures
Blume et al, Epilepsia 2001
• Evolving to bilateral, convulsive seizure : Focal to
Bilateral tonic-clonic (FBTCS)
– Previous terms: partial seizure secondarily
generalized; secondarily generalized tonic-clonic
seizure
– With tonic, clonic or tonic and clonic components
Generalized - reconceptualized
• For seizures
– Generalized epileptic seizures are
conceptualized as originating at some point
within, and rapidly engaging, bilaterally
distributed networks in the brain…can include
cortical and subcortical structures, but not
necessarily include the entire cortex.
A conceptual network for generalized
seizures involving the corticothalamic
circuitry
Theoretically a generalized seizure
could start at different points in the
network and engage bilaterally
distributed networks.
Thus a seizure could start frontally or
even parietally.
The key point is that a generalized
seizure can start from a focal point.
Old term New Term
Unconscious Impaired awareness
Partial Focal
Simple Partial Focal aware
Complex partial Focal impaired awareness
Dyscognitive Focal impaired awareness
Psychic Cognitive
Secondarily generalized tonic-clonic Focal to bilateral tonic-clonic
Arrest, freeze, pause, interruption Behavior arrest
Abbreviations for the most important seizure types
Seizure type Abbreviation
Focal Aware seizure FAS
Focal Impaired awareness seizure FIAS
Focal motor seizure FMS
Focal nonmotor seizure FNMS
Focal epileptic spasm FES
Focal to bilateral tonic-clonic seizure FBTCS
Generalized tonic-clonic seizure GTCS
Generalized absence seizure GAS
Generalized motor seizure GMS
Generalized epileptic spasm GES
Unknown onset tonic-clonic seizure UTCS
Istilah bangkitan yang tidak digunakan lagi
Complex partial Simple partial Partial
Secondarily
Psychic Dyscognitive generalized tonic-
clonic
Old Term : “Benign”
• Many epilepsies are not benign
- Childhood absence epilepsy : psychosocial impact
- RoLandic epilepsy (BECTS) : learning concerns
• Replace by term :
- Self-limited
- Pharmacoresponsive
• No Longer in use :
- Malignant
- Catastrophic
Words that don’t mean what the say Word that mean what they say
Partial Seizures
• Complex
– Means complicated
– Does not mean ”altered
awareness” Focal Seizure
• Simple
– Straightforward
– Does not mean “ retained
awareness”
Etiology
Seizure type
Structural
Focal impaired awareness automatism seizure
Genetic
Epilepsy type Infectious
Focal
Metabolic
Immune
Autosomal dominant nocturnal frontal
Unknown
Lobe epilepsy
Impact on Clinical Care and Practice
• New Classification framework will
- Change the approach to diagnosis in the clinic
- Be applied to patients and guide management
• Terminology update to reflect current thinki
ng
- Scientific advanced
Unclear Whether the New Definition
Increases or Decreases the Number of
People with Epilepsy
New
definition
No longer How
Old definition
present Big ?
Epilepsy Resolved
*Epilepsy is now considered to be resolved* for individuals who
had an age-dependent epilepsy syndrome but are now past the applica
ble age or those who have remained seizure-free for the last 10 years,
with no seizure medicines for the last 5 years.
* Avoiding preconceptions associated with the words “cure” and
“remission.”
“Resolved” has the connotation of “no longer present,” but it do
es not guarantee that epilepsy will never come back
Fisher et al, Epilepsia 55 (4): 475-482, 2014
KESIMPULAN
• Definisi epilepsi yang terbaru ini dapat lebih
sejalan dengan strategi terapi, baik pada saat
memulai ataupun menghentikan terapi.
• Definisi yang baru ini memungkinkan kita untuk
dapat berkomunikasi dengan pasien
• Mungkin di masa yang akan datang dapat dite
mukan marker penentu/keberadaan epilepsi
TERIMA KASIH
