title: Severe myoclonic epilepsy in infancy: a systematic review and a meta-analysis of individual patient data.
abstract: Severe myoclonic epilepsy in infancy (SMEI) is a rare, but severe disorder with seizures typically resistant to conventional antiepileptic drugs. The objective of the present study was to systematically review the literature on the available treatments for SMEI. Databases searched included Medline, Embase, and Cochrane. We used a fixed effect model to summarize the odds ratio of seizures rates and a logistic model to evaluate the influence of patient characteristics on treatment effect. We found 23 uncontrolled studies and 2 randomized controlled trials (RCTs) that compared stiripentol with placebo. Overall, 64 children aged between 3 and 20 years were included in the two RCTs. The odds ratio of responding to stiripentol relative to placebo was 32 (CI: 6.2, 161) and stiripentol reduced seizure rate by 70% (93%; 47%). The multivariate analysis does not suggest any differences within subgroups of participants and cotherapy. Results of uncontrolled studies in children with SMEI are potentially biased and do not provide valid information on the benefits and harms of treatments. The two RCTs identified, however, were performed with the same objectives and design and showed that seizure frequency is greatly reduced by stiripentol in children with SMEI after 2 months of treatment.
authors.affiliation: Inserm, CIC201, EPICIM, Lyon, France. bk@upcl.univ-lyon1.fr
authors.forename: Behrouz
authors.initials: B
authors.lastname: Kassaï
authors.forename: Catherine
authors.initials: C
authors.lastname: Chiron
authors.forename: Ségolène
authors.initials: S
authors.lastname: Augier
authors.forename: Michel
authors.initials: M
authors.lastname: Cucherat
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Rey
authors.forename: François
authors.initials: F
authors.lastname: Gueyffier
authors.forename: Renzo
authors.initials: R
authors.lastname: Guerrini
authors.forename: Julien
authors.initials: J
authors.lastname: Vincent
authors.forename: Olivier
authors.initials: O
authors.lastname: Dulac
authors.forename: Gérard
authors.initials: G
authors.lastname: Pons
mesh_terms: D000293:Adolescent; D000328:Adult; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D019992:Databases as Topic / Q000706:statistics & numerical data; D004148:Dioxolanes / Q000627:therapeutic use; D004831:Epilepsies, Myoclonic / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D016017:Odds Ratio; D010919:Placebos; D017594:Publication Bias; D016032:Randomized Controlled Trials as Topic / Q000706:statistics & numerical data; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants; D004148:Dioxolanes; D010919:Placebos; C021092:stiripentol