[The risk of second seizure in children with benign childhood epilepsy with centrotemporal spikes without treatment--a prospective study].

Purpose: To determine the rate of second seizure occurrence within and after six months of the first seizure in children with benign childhood epilepsy with centrotemporal spikes (BECTS) who did not undergo treatment after the first seizure. The results of this analysis may help elucidate the dilemma whether or not to treat the child after the first seizure.

Patients And Methods: Thirty-nine children with BECTS from our department (aged 3-11 years) were analyzed as candidates to be enrolled in a prospective multicenter randomized double-blind placebo controlled study on therapeutic efficacy of sulthiame.

R208X mutation in CLN2 gene associated with reduced cerebrospinal fluid pterins in a girl with classic late infantile neuronal ceroid lipofuscinosis.

Clinical picture of neuronal ceroid lipofuscinosis with late infantile onset (LINCL) is characterized by myoclonic seizures and psychomotor regression. We present a case of classic LINCL and reduced cerebrospinal fluid (CSF) pterins in a girl of normal psychomotor development and born to non-consanguineous parents. She first presented with febrile seizures at the age of four.

Complex disorder of neuronal migration in an infant with possible congenital cytomegalovirus infection.

A ten-months-old girl was evaluated for developmental delay, increased muscle tone and seizures. CT and MRI revealed un uncommon combination of two different manifestations of neuronal migration disturbance: agyria/pachygyria and subcortical laminar heterotopia ("double cortex" syndrome). The occurrence of these two manifestations of neuronal migration dosorders in the same individual is quite unusual.

[Monozygotic twins with centrotemporal spikes on the electroencephalogram--differences in clinical manifestations and the effect of valproate therapy].

Monozygotic twin sisters who had almost identical electroencephalographic abnormalities, but different clinical features and different response of this abnormalities to valproate at the age of 6 years are described. One twin was admitted to the hospital because of numerous brief myotonic seizures of sudden onset with corresponding abnormalities in the electroencephalogram. The seizures disappeared completely and the electroencephalogram became normal within a week of treatment with valproate.