ATYPICAL CHILDHOOD EPILEPSY WITH CENTROTEMPORAL SPIKES

OVERVIEW

Atypical childhood epilepsy with centrotemporal spikes (previously known as pseudo-Lennox syndrome, atypical benign partial epilepsy of childhood and atonic-benign childhood epilepsy with centrotemporal spikes) is recognized as an atypical evolution of childhood epilepsy with centrotemporal spikes. This syndrome is self-limiting, but characterized by frequent seizures of multiple types, including nocturnal focal motor and fronto-parietal opercular seizures, and daytime focal motor seizures with negative myoclonus and atypical absence seizures. Centrotemporal sharp waves are seen on EEG. During the phase of the epilepsy when seizures are frequent, neuropsychological deficits and motor impairment may be present. These deficits improve when seizures remit.

NOTE Self-limiting refers to there being a high likelihood of seizures spontaneously remitting at a predictable age.

NOTE Childhood epilepsy with centrotemporal spikes, atypical childhood epilepsy with centrotemporal spikes, epileptic encephalopathy with continuous spike-and-wave during sleep and Landau Kleffner syndrome are syndromes that have in common certain EEG features, with variable severity of focal seizures and neurocognitive impairment. They may be considered as a spectrum, an individual child may transition from one of these syndromes to another over time.

CAUTION If cognitive or language regression occurs in atypical childhood epilepsy with centrotemporal spikes consider performing a sleep EEG.

Clinical context

This syndrome is characterized by onset of seizures between 2-8 years of age (peak 5-6 years). Both sexes are affected. Antecedent and birth history are unremarkable. Head size and neurological examination are normal. Development and cognition before onset of the syndrome are typically normal, however neuropsychological and motor impairments are seen in the active phase of the epilepsy.