Abstract

The categorisation of the childhood epilepsies into a number
of different syndromes has allowed greater insight into the prognosis,
not only with regard to seizure control but also in relation to cognitive
and behavioural outcome. The role of genetics in determining both the
syndrome and the behavioural outcome remains promising, although the
promise is still largely unfulfilled. The behavioural/psychiatric outcome of
a selection of the large number of childhood epilepsy syndromes is presented. The rate of autism in West syndrome, particularly in children who
have tuberous sclerosis with temporal tubers, is high. In Dravet syndrome
there is a loss of skills, with an associated increase in behavioural problems.
The frequency of both subtle and overt seizures in the Lennox-Gastaut
syndrome almost certainly accounts for the apparent poor motivation;
however, a marked improvement in seizure control with treatment can
also result in behavioural problems, probably as a result of the “release
phenomenon”. A number of cognitive problems can arise in the socalled “benign” syndrome of epilepsy with centrotemporal spikes (BECTS) and the rate of ADHD is high. Autistic features and ADHD have been
described in the Landau-Kleffner syndrome and other syndromes associated with electrical status epilepticus of slow-wave sleep (ESES). Early
effective treatment may reverse some of these features. There is clear evidence for a behavioural syndrome in relation to juvenile myoclonic epilepsy
(JME), in which both clinical descriptions and functional neuroimaging indicate frontal lobe deficits.

Key words: West syndrome, Dravet syndrome, Lennox-Gastaut syndrome,
Rolandic, Landau-Kleffner syndrome, juvenile myoclonic epilepsy