Background and purpose Drug‐resistant idiopathic generalized epilepsy (IGE) remains challenging despite a favourable overall prognosis of IGE, and little is known about basic epidemiology and long‐term outcome of drug‐resistant IGE. The aim of the study was to describe the incidence, prevalence and outcome of IGE in an unbiased, population‐based cohort. Methods In 2014–2018, all patients (≥17 years) with IGE inhabiting the island of Funen (496 000 inhabitants) were recruited. The socioeconomic and clinical information available for 406 individuals was assessed. Median follow‐up was 15 years. Results The average IGE incidence (2008–2017) was 2.9/100 000 inhabitants/year. The point prevalence of identifiable IGE patients was 1.0/1000 adults (juvenile myoclonic epilepsy 0.4/1000; absence epilepsy 0.3/1000, epilepsy with generalized tonic–clonic seizures alone 0.3/1000); 92.1% of the patients were diagnosed before 25 years of age. When correcting for unequal age distribution in the cohort, 1102 people on the island of Funen fulfilled the diagnostic criteria for IGE at the age of 25 (estimated prevalence 2.7/1000 adults). In the year before data closure, 121 patients reported seizures. Fifty patients met the definition of drug‐resistant IGE (12.1% of the cohort, 4.5% of the estimated 1102 IGE patients). The average seizure burden of all patients with drug‐resistant IGE was 2.2 generalized tonic–clonic seizures per year; only 14 patients suffered more than two generalized tonic–clonic seizures per year. Drug‐resistant IGE was associated with an increased risk of requiring treatment for affective disorders and a reduced probability of working full time. Conclusion Idiopathic generalized epilepsy was associated with a low risk of persistent drug‐resistant seizures requiring specialist medical attention. Drug resistance was associated with a negative socioeconomic outcome.
This study aimed at defining clinical predictors of drug resistance in adults with genetic generalized epilepsy (GGE) who were treated with a broad spectrum of antiepileptic drugs. Of a cohort of 137 unselected adult GGE patients with long-term follow up, clinical and demographic data, putative prognostic factors (e.g., psychiatric comorbidities, electroencephalography [EEG]), treatment response, and data indicative of social status were collected. Fifty-eight patients had seizures within the past year. Thirty-three patients met the definition of "drug-resistant epilepsy" according to the International League Against Epilepsy (ILAE) definition. Psychiatric comorbidities, age at first diagnosis, and absences were associated with worse seizure control, whereas focal changes in EEG remained without prognostic impact. Resistance to valproic acid was the most important prognostic factor for refractory seizures. Resistance to valproic acid had a specificity of 100% to identify patients with drug resistance and correlated strongly with bad social outcome and seizure burden. Conversely, 21.2% of all patients with refractory seizures according to the ILAE definition later became seizure free (mainly with valproic acid). Our data suggest that "drug resistant GGE" must not be declared unless patients were adequately treated with valproic acid, and advocate resistance to valproic acid as a new clinical biomarker for drug-resistant GGE. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
Objective:To determine annual incidence, etiology, severity, and short-and long-term mortality of first-time, nonanoxic status epilepticus (SE) in adults in a population-based retrospective cohort study. Methods: We systematically identified all episodes of SE in the year 2014 on the island of Funen. Patients with SE due to anoxia, patients with recurrent SE, and patients <18 years old were excluded. Nonconvulsive SE in coma was diagnosed according to the Salzburg criteria. Etiology, semiology, modified Rankin Scale (mRS) at discharge, survival, and the Status Epilepticus Severity Score were retrospectively determined from patients' records. Patients with first-time nonanoxic SE diagnosed during 2008-2013 from our database (n = 88) were used to confirm the results. Results: The incidence of first-time, nonanoxic SE in 2014 was 10.7/100 000 persons at risk (n = 41). Median Status Epilepticus Severity Score was 3; in-hospital mortality was 24.4%. After median follow-up of 39.2 months, 53.7% of the patients had died (age-and gender-adjusted mortality rate of 5.2/100 000). Mortality stabilized 2 years after diagnosis. Analysis of the cohort from 2008-2013 confirmed stabilization of survival after 2-3 years and the high mortality 2 years after discharge. When correcting for acute symptomatic causes, the in-hospital mortality was 16.7% and 46.7% at follow-up (crude mortality rate of nonhypoxic and nonacute symptomatic SE = 3.5/100 000). An exploratory multivariate analysis of pooled patients with SE from 2008 to 2014 revealed mRS ≥ 2 at discharge as a prognostic factor for long-term mortality. Significance: In this cohort, the overall mortality of first-time nonhypoxic SE was >50%. Mortality of SE after discharge was substantially higher than in-house mortality and stabilized after 2 years. The degree of disability as indicated by mRS at discharge was associated with long-term mortality after discharge. K E Y W O R D Slong-term outcome, long-term survival, population-based, prognosis, status epilepticus, STESS
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