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Childhood absence epilepsy

Author
Christian M Korff, MD
Section Editor
Douglas R Nordli, Jr, MD
Deputy Editor
April F Eichler, MD, MPH

INTRODUCTION

Childhood absence epilepsy (CAE) is a common generalized epilepsy syndrome with a presumed genetic cause, characterized by typical absence seizures (TAS) appearing in otherwise healthy school-aged children. CAE is one of the most common forms of pediatric epilepsy.

TAS manifest as episodes of sudden, profound impairment of consciousness without loss of body tone. They are characteristically provoked by hyperventilation and correspond with generalized 2.5 to 5 Herz spike wave activity on electroencephalogram (EEG). Seizures may be subtle and often go unnoticed for prolonged periods, even with multiple seizures per day.

In most cases, TAS are the only seizure type observed in CAE, and they respond well to ethosuximide as well as other broad spectrum antiseizure drugs. CAE remits by early puberty in most patients without major sequelae, although persistent cognitive or psychiatric comorbidities may be present in some children.

The clinical features, diagnosis, treatment and prognosis of CAE are discussed here. Other epilepsy syndromes with an onset in childhood are discussed separately. (See "Seizures and epilepsy in children: Classification, etiology, and clinical features" and "Epilepsy syndromes in children" and "Juvenile myoclonic epilepsy".)

EPIDEMIOLOGY

Estimates of the annual incidence of childhood absence epilepsy (CAE) are derived from studies performed in primarily Caucasian populations. In a retrospective analysis of EEG findings of Swedish children aged 0 to 15 years, the annual incidence of “absence epilepsy” was 6.3 per 100,000 [1]. In another report based on a questionnaire sent to patients with seizures in south-West France, the annual incidence of CAE was estimated at 8 per 100,000 [2]. CAE accounted for 12 percent of epilepsy diagnoses in one prospective community-based study that included 613 children with epilepsy aged 0 to 16 years, making it one of the most common forms of pediatric epilepsy [3,4].

                  

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Literature review current through: Nov 2016. | This topic last updated: Wed Sep 07 00:00:00 GMT+00:00 2016.
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