LONG-TERM FOLLOW-UP-STUDY OF VIGABATRIN IN PRETREATED CHILDREN WITH WEST-SYNDROME

A multicentre, long-term, open-label, add-on study of vigabatrin was u ndertaken in 23 pretreated children with infantile spasms. After 3 mon ths of vigabatrin therapy 11 of the 23 patients had become seizure-fre e. At this time two-thirds of these 11 children still received other a ntiepileptic drugs (AEDs) in addition to vigabatrin (mostly valproic a cid and/or dexamethasone). After a mean follow-up time of 51/4 years ( range: 41/4 - 61/2) 72% of 18 evaluable patients (two children died, t hree were lost to follow-up) revealed seizure freedom for at least 1 y ear. The mean duration of vigabatrin therapy had been 21/2 years (rang e: 2 weeks to 43/4 years). Two-thirds of the 18 children continued to take AEDs, three of them undergoing vigabatrin monotherapy. Relapses o f infantile spasms had occurred in 14% of the children. The rate of vi gabatrin side effects (10%) was low. At follow-up, the EEG of 13 and t he 18 patients demonstrated focal or multifocal epileptic discharges. Fifty-five percent had developed another epilepsy (focal epilepsy, sec ondary generalized epilepsy or myoclonic-astatic epilepsy). With respe ct to mental functions, three children were normal or slightly retarde d, four showed moderate retardation and 11 revealed severe or very sev ere retardation. This long-term result is comparable to that in ACTH s tudies with unselected patients. The conclusions are: (1) vigabatrin i s an effective drug for the short-term and long-term treatment of refr actory infantile spasms; (2) the relapse rate is low; (3) vigabatrin i s well tolerated; (4) with respect to secondary epilepsies and mental functions the long-term outcome in these pretreated children is simila r to that in earlier studies with ACTH or corticosteroids.