A syndrome of undetermined cause, with onset before age 6 months, nearly continuous multifocal seizures, and progressive psychomotor deterioration is described in 14 children examined in the Universite Rene Descartes, and Hopital Saint Vincent de Paul, Paris, France. The seizures were partial and motor, moved from one cortical area to another, in 6 infants they became secondarily generalized, and the EEG discharges involved multiple independent sites. Seizures were controlled by antiepileptic drugs in only 2 patients. The EEG ictal pattern consisted of rhythmic alpha or theta activity, followed by postictal slow waves. CT was normal initially and showed atrophy with hydrocephalus ex vacuo at follow-up. MRI showed no parenchymal abnormalities. Extensive biochemical and viral studies were negative. Apart from 3 who showed improvement, patients regressed developmentally and became quadriplegic and hypotonic. Three patients died at age 7 months, 7 years, and 8 years, respectively. Autopsies in 2 cases showed severe hippocampal neuronal loss and gliosis. [1]
COMMENT. The authors describe a new infantile epilepsy syndrome with onset at 3 months, multifocal partial seizures, resistance to antiepileptic drugs and corticosteroids, and psychomotor deterioration. The cause is undetermined and no familial incidence has been observed.