Immunoglobulin therapy in the West syndrome

An examination was made of high dose non-treated immunoglobulin (NTIG) therapy at an early stage of the West syndrome (WS). Six patients with cryptogenic WS who suffered attacks ranging from 15 days to 6 months (mean 70 days) and 5 patients with symptomatic WS who suffered attacks ranging from 14 days to 4 months (mean 32 days), were administered NTIG 6–10 times intravenously at 100–200 mg/kg of body weight at intervals of 2 or 3 weeks. All patients with cryptogenic WS showed complete remission in accordance with normalized electroencephalogram (EEG) without following anticonvulsants medication. Of the 5 patients with symptomatic WS one patient showed cessation of clinical seizures in agreement with EEG improvement and 2 other patients revealed transient cessation of clinical seizures with recurrence. In cases of complete remission, the energy percentage of the power spectrum for each frequency band displayed a tendency toward gradual decrease of 8 wave band in correspondence with the increase of 6 wave band percentage after NTIG therapy, suggesting that high dose NTIG may be useful for early treatment of cryptogenic WS and for inhibiting brain deterioration owing to epileptic encephalopathy.