Abstract

BackgroundSeizures are common in juvenile Huntington’s disease (HD), but considered to be rare in adult-onset HD. We studied the occurrence of epilepsy and seizures in a nationwide cohort of Finnish patients with adult-onset HD.MethodsPatients with HD and their diagnoses of epilepsy or seizures were identified by a search into a nationwide registry. Cases were verified in a subsequent review of patient charts.ResultsThree out of 114 HD patients alive on prevalence date had been diagnosed with epilepsy giving a prevalence of 2.6 % (95 % CI, 0.6–7.5). In addition, one patient with a single unprovoked seizure, one patient with a medication-induced seizure and two patients with transient nonspecific attacks were identified. Epilepsy was not associated with clinical severity of HD and seizures were controlled with antiepileptic medications (AEDs). Generalized tonic-clonic seizures (GTCs) were the most common seizure type.ConclusionsPrevalence of epilepsy is similar in patients with adult-onset HD compared to general population. Seizures are easily controlled with AEDs.

Highlights

  • Seizures are common in juvenile Huntington’s disease (HD), but considered to be rare in adultonset HD

  • We examined the incidence of epilepsy and seizures in a nationwide cohort of Finnish patients with adult-onset HD

  • We found that the point prevalence of diagnosed epilepsy in our cohort of adult-onset HD patients was 2.6 %

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Summary

Introduction

Seizures are common in juvenile Huntington’s disease (HD), but considered to be rare in adultonset HD. We studied the occurrence of epilepsy and seizures in a nationwide cohort of Finnish patients with adult-onset HD. Epileptic seizures are common in juvenile-onset Huntington’s disease (HD), where they occur at a frequency of 38 % [1]. Seizures are thought to be rare in adult-onset HD and, even more, it has been proposed that the clinical diagnosis of HD should be re-evaluated, if seizures occur in an adult patient [4]. Research on the subject is scarce, as the occurrence of seizures and epilepsy in adult-onset HD has not been thoroughly investigated since the introduction of genetic testing for HD in 1993.

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