Intravenous immunoglobulin therapy is rarely effective as the initial treatment in West syndrome: A retrospective study of 70 patients.

PURPOSE: To evaluate factors influencing the efficacy and safety of intravenous immunoglobulins (IVIG) therapy for West syndrome.

METHODS: We investigated seizure outcomes in 70 patients who received IVIG treatment for West Syndrome during the first 3months after the onset of epileptic spasms. IVIG was administered for 3 consecutive days (initial IVIG treatment) at dosages ranging from 100 to 500mg/kg/day. If spasms disappeared within 2weeks of the initial treatment, maintenance IVIG treatment was commenced. We evaluated seizure outcomes at 2weeks (initial evaluation), at 2years (long-term evaluation), and the last visit (last follow-up evaluation) after the initial IVIG treatment. We analyzed dosages of IVIG, age at onset of spasms, treatment lag, and etiologies between responders and non-responders.

RESULTS: Among the patients, 7/70 (10.0%) had cessation of spasms and resolution of hypsarrhythmia at the initial evaluation. Another 6/70 patients (8.6%) were found to have cessation of spasms at the long-term evaluations. The treatment lag in responders was shorter than that in non-responders (P<0.01). There were no significant differences between responders and non-responders in IVIG dosages, age at onset of spasms, and etiologies. Two patients had relapse of partial seizures after cessation of spasms at the last follow-up evaluation. Adverse effects occurred in 2/70 patients.

CONCLUSIONS: The efficacy of IVIG was so low that it should not be considered as first-line treatment in West syndrome. IVIG therapy has a good safety profile and we would recommend it for West syndrome cases with drug resistance, severe complications associated with profound brain damage, severe brain atrophy, and in immunocompromised patients.