Atypical Benign Partial Epilepsy of Childhood

Abstract

Short Description Atypical benign partial epilepsy (ABPE) is an epileptic disorder characterized by seizures typical of benign childhood epilepsy with centrotemporal spikes (BCECTS), bilateral or segmental focal atonic seizures, and atypical absences. Awake EEG shows focal paroxysms resembling BCECTS associated with bursts of generalized paroxysms and with a marked activation of slow spike wave activity in non-REM sleep reproducing an ‘‘electric status epilepticus during sleep (ESES)’’ EEG pattern. This condition, firstly described by Aicardi and Chevrie (1982), was named ABPE because of the benign prognosis of epilepsy and cognitive function. The term ‘‘pseudo-lennox syndrome’’ was also used because of the multiple daily falls occurring in these patients mimicking semiological findings of the Lennox-Gastaut syndrome (LGS). Persistent mental handicap after remission of epilepsy occur in 56% of patients. ABPE has been shown to present broad overlap with BCECTS, continuous spikewaves during sleep syndrome (CSWS) and Landau-Kleffner syndrome (LKS) (Hahn 2002).