Refractory juvenile myoclonic epilepsy: A meta-analysis of prevalence and risk factors

Abstract

BACKGROUND: Juvenile myoclonic epilepsy (JME) is a common epilepsy syndrome for which treatment response is generally assumed to be good. We aimed to determine the prevalence and prognostic risk factors for refractoriness of JME. METHODS: We systematically searched PubMed and Embase and included 43 eligible studies, reporting seizure outcome after anti-epileptic drug treatment JME cohorts. We defined refractory JME as persistence of any seizure despite AED treatment and performed a random-effects meta-analysis to assess the prevalence of refractory JME and of seizure-recurrence after AED withdrawal in individuals with well-controlled seizures. Studies reporting potential prognostic risk factors in relation to seizure outcome were included for subsequent meta-analysis of risk factors for refractoriness. RESULTS: Overall 35% (95%CI: 29 - 41%) of individuals (n=3311) were refractory. There was marked heterogeneity between studies. Seizures recurred in 78% (95%-CI: 52-94%) of individuals who attempted to withdraw treatment after a period of seizure-freedom (n=246). Seizure outcome by publication year suggests that prognosis has not improved over time. Meta-analysis suggested six variables as prognostic factors for refractoriness: having three seizure types, absence seizures, psychiatric comorbidities, earlier age at seizure onset, history of childhood absence epilepsy, and having praxis-induced seizures. CONCLUSION: A third of people with JME were refractory, which is more prevalent than expected. Risk factors were identified and can be used to guide treatment and counselling of people with JME. This article is protected by copyright. All rights reserved