Paediatric

epilepsies:
principles &
approach
Dr Norhafizah Ahmad
Hospital Ampang
 •Systematic approach
•Basic knowledge on
objectives semiology
•Classification of epilepsy
•Basic knowledge on
common epilepsy syndrome
 Misdiagnosis.. WHY?

•Large differential diagnosis

•False perception – wrong diagnosis = grave risks
 Evolution of epilepsy
classifications

2010.2012,2017
1989
1981 2001
new classification
proposal for revised five axes streamlined terms
The ILAE published a
Classification of diagnostic acknowledgement of newer
classification of
Epilepsies and scheme was advances
epileptic seizures,
Epileptic Syndromes (genetics/imaging)
the ICES proposed
descriptors of effects of
seizures
 DECIDE
The onset or
beginning of a seizure

using 80%
confidence
 GE NE RALIZE D
ONSET SEIZURES

• originating at some point

within, and rapidly
engaging, bilaterally
distributed networks
• can include cortical and
subcortical structures, but
do not necessarily include
the entire cortex
 F O CA L O N SE T
SE I ZURES

• conceptualized as
originating within
networks limited to one
hemisphere
• may be discretely
localized or more
widely distributed
 LEVEL 1 : SEIZURE TYPE

AWARENESS MOVEMENT
A person’s level of Whether movements
awareness during a happen during a
seizure seizure
FOCAL ONSET
awar
e
motor non-
impaired
motor
awareness
 LEVEL 1 : SEIZURE TYPE
MYOCLONIC SEIZURE

• a single or series of jerks (brief muscle contractions

• Each jerk is typically milliseconds in duration
GENERALISED • Myoclonic status epilepticus is characterized by
• 1. ongoing (> 30 minutes) irregular jerking,
ONSET • 2. partially retained awareness
• These two features distinguish a myoclonic status
epilepticus from a generalized clonic seizure, where
MOTOR
consciousness is lost and the jerking is sustained and
rhythmic.)
 LEVEL 1 : SEIZURE TYPE
CLONIC SEIZURE

• seizure with bilateral, sustained rhythmic jerking and loss of

consciousness
GENERALISED • distinguished from repetitive serial myoclonic seizures by
the rhythmicity of the jerking and that it occurs in the setting
ONSET of loss of consciousness

MOTOR
 LEVEL 1 : SEIZURE TYPE
TONIC SEIZURE

• increased tone of the limbs typically lasting seconds to a

minute
GENERALISED • occur out of sleep and in runs of varying intensity of tonic
stiffening
ONSET

MOTOR
 LEVEL 1 : SEIZURE TYPE
ATONIC SEIZURE

• sudden loss or diminution of muscle tone without

apparent preceding myoclonic or tonic features
GENERALISED • very brief (<2 seconds) and may involve the head, trunk
or limbs
ONSET

MOTOR
 LEVEL 1 : SEIZURE TYPE
MYOCLONIC-ATONIC SEIZURE

• myoclonic seizure followed by an atonic seizure

• The head and limbs are affected, typically resulting in
GENERALISED rapid fall.

ONSET

MOTOR
 LEVEL 1 : SEIZURE TYPE
EPILEPTIC SPASMS

• sudden flexion, extension or mixed flexion-extension of

proximal and truncal muscles, lasting 1-2 seconds
GENERALISED • typically occur in a series, usually on wakening.
• Subtle forms may occur with only chin movement,
ONSET grimacing, or head nodding.
• Spasms may be bilaterally symmetric, asymmetric, or
unilateral, depending on whether they are generalised
MOTOR
onset or focal onset.
 LEVEL 1 : SEIZURE TYPE
TYPICAL ABSENCE SEIZURE

• generalized seizure with abrupt onset and offset of

altered awareness
GENERALISED • Clonic movements of eyelids, head, eyebrows, chin,
perioral or other facial parts may occur, most typically at
ONSET 3Hz
• Oral and manual automatisms are common and there
may be perseveration of behaviors occurring prior to
NON-MOTOR
seizure onset
 LEVEL 1 : SEIZURE TYPE
ATYPICAL ABSENCE SEIZURE

• less abrupt onset and offset of loss of awareness than

typical absence seizures
GENERALISED • often associated with other features such as loss of
muscle tone of the head, trunk or limbs (often a gradual
ONSET slump) and subtle myoclonic jerks
• often occur in individuals with intellectual impairment
• The loss of awareness may be minimal with the patient
NON-MOTOR
continuing an activity, but more slowly or with mistakes.
 LEVEL 1 : SEIZURE TYPE
MYOCLONIC ABSENCE SEIZURE

• Rhythmic myoclonic jerks of the shoulders and arms with

tonic abduction that results in progressive lifting of the
GENERALISED arms during the seizure
• typically bilateral but may be unilateral or asymmetric
ONSET • Seizures last 10-60 seconds and typically occur daily..

NON-MOTOR
 LEVEL 1 : SEIZURE TYPE
ABSENCE WITH EYELID MYOCLONIA

• Absence seizures accompanied by brief, repetitive, often

rhythmic, fast (4-6 Hz) myoclonic jerks of the eyelids with
GENERALISED simultaneous upward deviation of the eyeballs and
extension of the head.
ONSET • Seizures are typically very brief (<6s in duration) and
multiple seizures occur on a daily basis.
• Mostly awareness is retained.
NON-MOTOR
 DE FI NITI ON O F E PI LE PSY

Epilepsy is a disease of the brain defined by any of the following conditions:

1. At least two unprovoked (or reflex) seizures occurring more than 24 hours
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

 LEVEL 3:
EPILEPSY SYNDROME
epilepsies may also be organized (by reliably identified
common clinical and electrical characteristics) into epilepsy
syndromes
 Epilepsy Syndrome

Seizure EEG
Age
type

provides information
on
• which underlying etiologies should be
considered
• which anti-seizure medication(s) might be most
N O L O N G E R USE D EPILEPSIES BY
ETIOLOGY
'idiopathic', '
cryptogenic'
'symptomatic'
GE N E T IC E T IO L O G Y EPILEPSIES BY
ETIOLOGY
STRUCTURA L
E TIO L O G Y
IN F E CT IO US
E T IO L O G Y
ME T A B O L IC
E TIOLOGY
UN KN O W N
IMM UN E E T IO L O G Y E T IO L O G Y