A Case of Linear Nevus Sebaceous Syndrome Associated with Early-Infantile Epileptic Encephalopathy with Suppression Burst EIEE

Abstract

A female infant started having palpebral myoclonia on each feeding time at 16 hours and a generalized clonic convulsion at 48 hours after birth. They were controlled by i. v. PB-Na following by oral maintenance of PB 5 mg/kg/day. But emprosthotonic spasms with series formation appeared at 42 days of age. They occurred both at sleep and waking states. Her head was asymmetric and linear pigmentation was seen on the right side of her neck and the center of her forehead. These findings were compatible with the Linear Nevus Sebaceous syndrome. A CT scan revealed left hemiatrophy of the brain. The EEG taken after the initiation of tonic spasms revealed a typical suppression-burst pattern. Treatment with clonazepam, acetazolamide, sodium valproate and a high dose of vitamin Bs were tried but without success.
The two courses of ACTH-Z injection were tried, but the effectiveness was transient. The tonic spasms and tonic seizures have continued several times a day.
The EEG taken at one and nine months of age showed 1.5 to 3 cps slow spike and wave bursts and rapid rhythms during stage 2 and 3 of sleep. It suggested the change to Lennox-Gastaut syndrome.