ゾニサミドが著効した軽症PVL(periventricular leukomalacia)関連性West症候群の1例

Abstract

We report the case of a ten-month-old male, the second baby of twins. He was delivered by caesarian section at a gestational age of 28 weeks and 5 days because of maternal infection and uncontrolled tocolysis. His birth weight was 1,390g. Post-natal respiratory care was required for respiratory distress syndrome. Group B Streptococcal (GBS) sepsis was diagnosed at birth and the patient was treated intensively with antibiotics. Cranial CT and magnetic resonance examination indicated irregularities consistent with periventricular leukomalacia (PVL) on the right anterior lateral horn and in the periventricular area. He had a febrile convulsion at the age of ten months, and bilateral parieto-occipital multi-spikes and hypsarrhyttmia were evident on electroen- cephalogram (EEG) 10 days after the seizure. Valpronic acid (VPA) was prescribed for the prevention of attacks, but further infantile spasms occurred after 5 days. Convulsions increased with series formation, and retardation of development was also noted. He was admitted to our hospital with the diagnosis of West syndrome. Increased doses of VPA and high doses of vitamin B6 (VB6) were administered without clinical benefit. In view of the clinical deterioration, Zonisamide (ZNS) was additionally prescribed. Eleven days after the initiation of ZNS treatment, the spasms disappeared, and the hypsarhytlmia and irregular spike and wave complex patterns on EEG were resolved. The effects of therapy with Zonisamide in West syndrome following PVL deserve wider discussion.