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Paediatric epilepsy syndromes (Juvenile absence (Clinical presentation …
Paediatric epilepsy
syndromes
Juvenile
absence
Clinical presentation
Photosensitive absences/generalised T-C
Diagnosis
EEG (characteristic)
Epidemiology
10-20y
Management
AEDss (lifelong needed)
Prognosis
Remission unlikely
Juvenile
myoclonic
Epidemiology
10-20y
Clinical presentation
Myoclonic, generalised TC or absences
Often occur shortly after waking
Prognosis
No learning difficulties
Remission unlikely
Diagnosis
EEG: characteristic
Management
AEDss (lifelong needed)
West syndrome
(infantile spasms)
Clinical presentation
Violent flexor spasms of head, trunk, limbs then arm extension; last 1-2s, occur in bursts, often on when waking, multiple episodes per day
Deteriorating social interaction
Prognosis
Loss of skills
Learning disability
Continuing epilepsy
Epidemiology
Age 3-6m
Diagnosis
EEG: hypsarrhythmia
CT head: often underlying neurological cause
Management
Corticosteroids and/or vigabatrin (SEs, often relapse)
Lennox Gastaut
syndrome
Pathophysiology
Often complex neurological problems
Clinical presentation
Often atonic, atypical absence, or tonic seizures during sleep
Neurodevelopmental arrest
Behavioural disturbance
Diagnosis
EEG: slow generalised spike and wave
CT head: may show neurological lesion
Epidemiology
Age 1-3y
Prognosis
Poor
Childhood
absence
Prognosis
No developmental consequences but may miss school
Often remisses in adolescence, some may evolve to JME
Clinical presentation
Momentary unresponsive state (sudden, lasts <30s)
Motor arrest
Automatisms (eyelid flutter, lip smacking)
No recall after (but child often realises it has happened)
Diagnosis
Hyperventilation test: blow on piece of paper for 2-3m, induces absence seizure; EEG fast generalised spike wave
Epidemiology
4-12y
F>M
Rare
Benign rolandic epilepsy
with centro-temporal
spikes (BREC)
Clinical presentation
T-C seizures in sleep
Simple focal seizures (odd tongue sensation, facial distortion)
Diagnosis
EEG: focal sharp waves in rolandic area
Epidemiology
4-10y
15% childhood epilepsies
Prognosis
Often benign
Remits in adolescence
Panayiotopoulos
syndrome
Pathophysiology
Early onset benign occipital epilepsy
Abnormality in occipital region
Clinical presentation
Unresponsive staring in sleep
Head/eye deviation
May progress to T-C seizure
Epidemiology
Age 1-5y
Diagnosis
EEG: posterior focal sharp waves and
occipital discharge when eyes shut
Prognosis
Remits in childhood
May have learning difficulties