The treatment of West syndrome: a Cochrane review of the literature to December 2000

Abstract

Background: West syndrome is an age dependant syndrome, which includes a peculiar type of epileptic seizure (infantile spasms), usually hypsarrhythmia and in the majority psychomotor retardation. Despite huge advances in medicine it still remains a poorly understood entity and although with newer imaging techniques we are more often able to elicit the underlying ‘causes’ of these spasms, still little is known about their pathophysiological basis and treatment remains problematic. Objectives: To compare the effects of single pharmaceutical therapies used to treat infantile spasms in terms of long-term psychomotor development, subsequent epilepsy, control of the spasms and side effects. Methods: A search of the central trials register of the Cochrane Epilepsy Group, medline database, embase database and the reference lists of all retrieved articles was undertaken. Correspondence with colleagues and drug companies and appeals at international conferences were also undertaken to try and discover unpublished data. All randomised controlled trials (RCTs) on the medical treatment of infantile spasms were included. Data was then extracted independently by the three reviewers and analysed using the RevMan software package. Main results: We found ten small RCTs on the pharmacological treatment of infantile spasms. No unpublished trials were discovered. These ten studies looked at just 335 patients treated with a total of eight different pharmaceutical agents. Overall methodology of the studies was poor, partly because of ethical dilemmas such as giving placebo injections to children. No study considered the effects of treatment on long-term psychomotor development or onset of other seizure types. One small study found vigabatrin to be more efficacious in stopping infantile spasms in a group of patients with tuberous sclerosis than hydrocortisone. One underpowered study showed a trend for vigabatrin to be more efficacious than placebo in stopping infantile spasms, another two equally underpowered studies suggested adrenocorticotrophic hormone (ACTH) to be more efficacious than low-dose prednisone. 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 Overall, only nine patients were reported to have been withdrawn from the trial treatments due to side effects and two deaths were reported. Conclusions: There is still little evidence available on the optimum treatment for infantile spasms. Further trials with larger number of patients, and longer follow-up are required.