Treatment of infantile spasms

Infantile spasms (West's Syndrome) is a syndrome which includes a peculiar type of epileptic seizure, usually hypsarrhythmia and in the majority of people, psychomotor retardation. It remains poorly understood and despite modern imaging techniques an underlying cause is often not found. Little is known about their pathophysiological basis and treatment remains problematic.

To compare the effects of single drugs used to treat infantile spasms in terms of long-term psychomotor development, subsequent epilepsy, control of the spasms and adverse effects.

Our search included the Cochrane Epilepsy Group trials register, MEDLINE (1966 to 2003) and EMBASE (1981 to 2003), contacting pharmaceutical companies and appeals at international conferences.

All randomised controlled trials (RCTs) of the administration of drugs to people with infantile spasms.

Three reviewers independently selected trials for inclusion and extracted data. Outcomes included cessation of spasms, time to cessation of spasms, participants with cessation of spasms remaining spasm free, reduction in spasms, resolution of hypsarrhythmia, subsequent epilepsy rates and adverse effects.

Eleven RCTs were included, who in total recruited just 514 participants and tested eight different drugs. Overall, methodology of the studies was poor. No study assessed long-term psychomotor development or onset of other seizure types. One small study found vigabatrin to be more efficacious than hydrocortisone in stopping infantile spasms in a group of people with tuberous sclerosis. One underpowered study showed a trend for vigabatrin to be more efficacious than placebo in stopping infantile spasms. Two small studies when combined showed ACTH to be more efficacious than low-dose prednisone (2 mg/kg). One study also suggested that control of spasms occurred more frequently with high dose vigabatrin as compared to low dose vigabatrin. It was not possible to compare reduction in the number of spasms between the different treatments because of differences in methods of analysis. Overall, only 18 individuals were reported to have been withdrawn from the trial treatments due to adverse effects and 4 deaths were reported.

We found no single treatment to be proven to be more efficacious in treating infantile spasms than any of the others (other than vigabatrin in the treatment of infantile spasms in tuberous sclerosis in one underpowered study). Few studies considered psychomotor development or subsequent seizure rates as outcomes and none had long-term follow-up. Further trials with larger numbers of participants, and longer follow-up are required.