1) The document defines epilepsy and provides the new ILAE 2017 classification framework for epilepsy syndromes. 2) The classification involves assessing the seizure type, epilepsy type, syndrome, cause, and other relevant impairments. 3) Classifying epilepsy according to this framework helps to determine appropriate testing, treatment, and prognosis for patients. It provides a standardized method for clinicians.

1. EPILEPSY
DR FATIMAH BINTI JAMIL

2. DEFINITION
Epilepsy is a disorder of the brain characterized by an enduring
predisposition to generate epileptic seizures and by the neurobiologic,
cognitive, psychological, and social consequences of this condition. •
An epileptic seizure is the transient occurrence of clinical
manifestation of abnormal excessive or synchronous neuronal activity
in the brain.
An epileptic syndrome is an epileptic disorder characterized by a
cluster of signs and symptoms. Syndromes are classified on age of
onset, seizure type(s), clinical and developmental features, EEG
abnormalities and MRI brain findings. It has therapeutic and prognostic
implications.

3. Operational (Practical) Clinical Definition of Epilepsy (any of the
following conditions):
• At least two unprovoked (or reflex) seizures occurring >24 h apart.
• 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.
• Diagnosis of an epilepsy syndrome.

4. IMITATOR OF EPILEPSY
The first important step in
management of childhood
epilepsy is to differentiate
epileptic seizures from paroxysmal
non epileptic event

5. Approach to a child with a first seizure
-25-50 % unprovoked seizure in
child will recur
Routine investigation
- FBC,BUSE,Ca,Mg, RBS
-LP (if suspicious brain infection)
-Toxicology screening
-EEG
-Neuro imaging (MRI)
Treatment not required
Approach to a child with a epilepsy
Diagnosis of epilepsy mainly
clinical
detailed history of
seizure/video,past
history,developmental history,
family history
look for
dysmorphism,neurocutaneous
stigmata, thorough CNS and
developmental examination
-perform general and systemic
examination to look for clue of
underlying etiology
Investigation
-blood : FBC, RP,LFT, BLLOD
GLUCOSE,CALCIUM , MAGNESIUMTO
EXCLUDE METABOLIC CAUSE AND BEFORE
STARTING AED
-METABOLIC AND GENETIC STUDY IF
INDICATED
- EEG
- MRI
INDICATED IN : Epilepsy occurring in first
year of life,focal epilepsy, developmental
delay or regressin,difficult to
control,refractory epilepsy

6. ILAE 2017 Epilepsy classification:
Updates & how to apply it

7. Level 1
Level 2
Level 3
At all level
At all level

8. Why the need for new classification?
• Use words that mean what they say
• Previous classification was based on anatomical basis
• but now accepted that epilepsy is a network disorder
(eg: neocortical, limbic)
• Places emphasis on symptoms and signs of the event (semiology)
– Sometimes supported by additional data: EEG, MRI, genes, antibodies, etc
– Is not based on EEG ictal or subclinical patterns
• Easier for clinicians to use
• Allow patients to understand terminology

9. Why is classifying epilepsy important?
▪ Children & family need a:
-diagnosis
-prognosis
-management plan (specific & precise to pt)
▪ Diagnosing epilepsy is not enough!!!
▪ Short and long term management of specific
epileptic syndrome differs markedly
▪ Can direct investigations and appropriate Tx

10. ▪level 1: Semiology / seizure type (is it seizure?)
▪level 2: Epilepsy type
▪level 3: Epilepsy syndrome
▪level 4: Aetiology
▪level 5: Co-morbidities ILAE task force Epilepsia2001, 2017
HOW TO APPROACH EPILEPSY

11. The “ DESSCRIBE “Approach
How do I approach epilepsy?“the DESSCRIBE approach”
▪level 1: D escription
E pileptic or non-epileptic episode(s)?
▪ level 2: S eizure type(s)?
▪ level 3: S yndrome
▪ level 4: C ause
▪ level 5: R elevent;
I mpairment
B ehavioural and emotion
E ducation issues?

12. level 1-Semiology (Epileptic seizure or not) =
D,E
• Need to decide if epileptic
seizure
• Crucial as 25% of patients
misdiagnosed as epileptic
• There are no gold standard
investigative tool to diagnose
epilepsy (EEG, MRI ….. Only
Supportive to confirm clinical
suspicion)
• Good clinical history taking
cornerstone of diagnosis of
epilepsy
• After taking history of event
(patient and witnesses); is it
consistent with epileptic seizure?
- Yes: appropriate Mx,
consider Ix (EEG,
MRI,etc)
- No: Consider differential
diagnosis, +/- other Ix
• Uncertain: wait & see, need more
info & time,observing natural Hx
important but takes time
▪ If not sure: say “uexplained
paroxysmal event”
▪ If not clear Hx, better to observe
patient rather
• than rush into diagnosis

13. level 1 : Seizure type

14. Focal seizure
• Originate within
networks limited
to one
hemisphere
• May be
discretely
localized or more
widely
distributed
• Awareness or
impaired
awareness
Generalized seizures
• Originate at some
point within and
rapidly engage
bilaterally distributed
networks
• Can include cortical
and subcortical
structures but not
necessarily the entire
cortex
Unknown Seizures
• Unknown onset if no
supplementary information to
determine if onset focal or
generalized
• If child found by Mum having
bilateral tonic-clonic seizure in bed
but onset not witnessed; EEG and
MRI normal
= Unknown onset tonic-clonic sz
• If child found by Mum having
bilateraltonic-clonic sz in bed but
onset not witnessed; EEG showing
R parietal slowing, MRI R parietal
cortical dysplasia
= Focal to bilateral tonic-clonic sz

16. ICTAL SEMIOLOGY: MOTOR SEIZURE
• Myoclonic jerks: v. brief muscular contractions
thatoccur singly / repeated only a few times
(<100ms)
• Spasms: widespread muscular contractions (< 2sec)
• Tonic: sustained muscle contraction (few sec–mins)
• Clonic: rhythmic myoclonus / repetitive myoclonus
involving same muscle groups approx 2-3 c/sec
• Atonic: sudden loss of erect posture
• Automatism: repetitive stereotypical voluntary
motor activity when cognition impaired
- Simple
- Complex
• Postural changes: adoption of posture (symmetrical,
asymmetrical)
ICTAL SEMIOLOGY: NON-MOTOR
SEIZURE
• Impaired awareness: altered conscious level
• Somatosensory
• Visual
• Olfactory
• Gustatory
• Auditory
• Autonomic

17. level 2 – Seizure type = S
• Epileptic seizures are usually
divided into
generalised and focal
• A generalised epileptic seizure is:
“A seizure whose initial semiology
indicates, or is consistent with, more
than minimal involvement of both
cerebral hemispheres”
• A focal epileptic seizure is:
“A seizure whose initial semiology
indicates, or is consistent with, initial
activation of only part of one
cerebral hemisphere”

18. Level 2: Epilepsy type
GENERALISED
EPILEPSY
• Made on clinical
grounds supported by
typical interictal EEG
findings :Typically show
generalized spike wave
on EEG
• NB: caution in patient
with GTCS and a normal
EEG
-These patients need to
have supportive evidence
of generalized epilepsy like
family history or myoclonic
jerks
FOCAL EPILEPSY
• Considered in
focal, (multi)focal
and also
hemispheric
epilepsies • New group as there are
patients with both epilepsies
• Diagnosis made on clinical
grounds supported by EEG
findings
– Both focal impaired awareness
seizures + generalized absence
seizures
– Interictal EEG: both GSW and
focal discharges but epileptiform
activity not required for the
diagnosis
• Examples include Dravet
syndrome and Lennox
Gastaut syndrome
COMBINED GENERALISED
AND FOCAL EPILEPSY:

19. Level 3: Epilepsy syndromes
An epileptic syndrome
is an epileptic disorder
characterized by a
cluster of signs and
symptoms. Syndromes
are classified on age of
onset, seizure type(s),
clinical and
developmental
features, EEG
abnormalities and MRI
brain findings. It has
therapeutic and
prognostic
implications.

20. level 4: Causes of epilepsy
• Idiopathic → genetic
• Symptomatic → structural, metabolic,(auto)immune, infectious
• Cryptogenic → abandoned replaced with unknown cause

21. Level 5- “Relevant Impairments, Behavioural &
emotional or Educational probs
• Majority of children with attend mainstream school; but studies
suggest 50% have some behavioural problem / receive educational
input
• Specific seizure syndromes (especially symptomatic) predispose child
to behavioural impairments:
-Infantile spasm, Complex focal: ASD, ADHD
-L Gastaut: social unresponsive, violent behaviours
-BECTS: attention deficit, mild cognitive problem

22. Example ILAE approach
• 1. (DE) Sz semiology
• 2. (S) Epilepsy type
• 3. (S) Epilepsy syndrome
• 4. (C) Cause
• 5. (RIBE) Additional
impairments
1. Cry out, trunk flexion
2. Generalised, tonic
3. Epileptic encephalopathy-
infantile spasm
4. Tuberous sclerosis
5. Developmental delay ,autistic
features

23. SUMMARY ILAE 2017 CLASSIFICATION OF EPILEPSIES
• Meant to make framework more straightforward
- Seizure type: assessing onset
- Epilepsy type
- Epilepsy syndrome
• Aetiology, aetiology, aetiology considered at all stages
• Retained IGE = CAE, JAE, JME, GTCSA
• Abandoned symptomatic generalised epilepsies replaced with
developmental and / or epileptic
encephalopathies
• Benign epilepsies replaced to self-limited epilepsies

24. Principle of antiepileptic drug (AED) therapy
for epilepsy
- Attempt to classify the seizure type and epilepsy syndrome
- Treatment recommended if ≥ 2 episodes
- Monotherapy as far as possible
- Choose most appropriate drug based on epilepsy syndrome, seizure type( if epilepsy syndrome
not identify yet and associated comorbidities
- Increased dose gradually until seizure controlled or maximum dose reached or side effect occur.
- Add on second drug if first drug failed, optimise second drug, then try to withdraw first drug
- Avoid starting female of child bearing potential syrup Valproate because of risk of teratogenicity
and neurodevelopmental impairment to unborn child.
- Trial of vitamin and co factor such as vitamin b6, biotin, folinic acid, should be considered in
infantile epilepsies not responding to AED
- With withdrew medication is planned,(generally after seizure free for 2 years) consideration
should be given to epilepsy syndrome, likely prognosis, and individual circumstances before
attempting slow withdrawal of medication over 3-6 month.
- If seizure recur, last dose reduction is reserved and medical advice sought.

25. Key points for rational polytherapy
• Key principle: “Start low, go slow”
– Give gradual titration up plan
– Ensure non-medication factors optimized (sleepquality, stress)
• The selected AED should not be abandoned too early
• The correct AED is the smallest one that achieves sz control without
ADRs
• Children more susceptible than adults to specific ADRs
• Prevent over-medication

28. Definition of status epilepticus (ILAE 2015)
‘SE is due failure of the mechanisms responsible for seizure termination or
from the initiation of mechanisms which lead to abnormally prolonged
seizure (after time point 1). it is a condition that can have long term
consequences (after time point 2), including neuronal death, neuronal injury,
and alteration of neuronal networks’
• Two conceptual time points with operational dimensions:
– Time point 1 (TP1): point beyond which a seizure should be classified as
‘continuous seizure activity’ indicates the moment when treatment is
indicated
– Time point (TP2): during ongoing continuous seizure activity denotes the
juncture at which the risk of long-term neurological sequelae develops
• Determines the aggressiveness of treatment

29. INTRODUCTION
New Definition of SE as Proposed by the
International League Against Epilepsy (2015)
Status epilepticus type Time 1
(Treatment
Commenced)
Time 2
(Consequences
Expected)
Tonic-clonic 5min 30 min
Focal with impaired
consciousness
10 min >60 min
Absence 15 min unknown

32. Summary
• Good clinical history crucial to diagnose sz
- Never use EEG to solely diagnose epilepsy
• Classification is important – allows methodical
approach to testing, treatment and prognosis
• AED is chosen based on epilepsy type / syndrome