Epileptic syndromes in children with hydrocephalus shunted in the first 2 years of life: A retrospective study of 67 cases

We present 67 patients with early-onset shunted hydrocephalus and epilepsy, focusing on the prevalence of epileptic syndromes. Thirty-nine boys and 28 girls were studied. Mean follow-up was 12 yr (range, 9-17 yr). Forty-two patients had epilepsy. Epilepsy with focal seizures only was seen in 13 patients and West syndrome in 12 patients, preceded by focal symp- tomatic epilepsies in six. Eleven patients had epileptic encephalopathy with continuous spikes and waves during slow sleep, preceded by focal electroencephalography abnormalities in all and by West syndrome in one. Four patients presented with Lennox-Gastaut syndrome, of whom three had had West syndrome in the first year of life. One patient had epilepsy with myoclonic-astatic seizures and another multifocal spikes associated with epileptic spasms in clusters. West syndrome evolved into focal seizures in seven. Seven patients had acute symptomatic seizures, secondary to shunt dysfunction in five and shunt infection in two. Focal epilepsy and epileptic encephalopathies were the most frequent syndromes. Hemorrhage, infections, and cortical dysplasia were associated with a higher prevalence of epilepsy.