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

Background: West syndrome is an age dependant syndrome, which includes a pe culiar type of epileptic seizure (infantile spasms), usually hypsarrhythmia and in the majority psychomotor retardation. Despite huge advances in medi cine it still remains a poorly understood entity and although with newer im aging techniques we are more often able to elicit the underlying 'causes' o f 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, su bsequent epilepsy, control of the spasms and side effects. Methods: A search of the central trials register of the Cochrane Epilepsy G roup, medline database, embase database and the reference lists of all retr ieved articles was undertaken. Correspondence with colleagues and drug comp anies and appeals at international conferences were also undertaken to try and discover unpublished data. All randomised controlled trials (RCTs) on t he medical treatment of infantile spasms were included. Data was then extra cted independently by the three reviewers and analysed using the RevMan sof tware package. Main results: We found ten small RCTs on the pharmacological treatment of i nfantile spasms. No unpublished trials were discovered. These ten studies l ooked at just 335 patients treated with a total of eight different pharmace utical 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 lon,a-term psychomotor development or onset of other seizure types. One small study found vigabatrin to be mor e efficacious in stopping infantile spasms in a group of patients with tube rous sclerosis than hydrocortisone. One underpowered study showed a trend f or vigabatrin to be more efficacious than placebo in stopping infantile spa sms, another two equally underpowered studies suggested adrenocorticotrophi c hormone (ACTH) to be more efficacious than low-dose prednisone. It was no t possible to compare reduction in the number of spasms between the differe nt treatments because of differences in methods of analysis. Overall, only nine patients were reported to have been withdrawn from the trial treatment s clue to side effects and two deaths were reported. Conclusions: There is still little evidence available on the optimum treatm ent for infantile spasms. Further trials with larger number of patients, an d longer follow-up are required. (C) 2001 Elsevier Science B.V. All rights reserved.