Atonic-BECTS: An unusual presentation of self-limited epilepsy in childhood

Objectives

We describe an unusual patient with self-limited epilepsy in childhood to aid in the accurate diagnosis and timely treatment of an atonic variant of self-limited focal epilepsy of childhood with centrotemporal spikes.

Methods

We reviewed the medical records documenting the clinical presentation, diagnostic evaluation, and treatment. We also reviewed the relevant video electroencephalograms (EEGs).

Patient Description

This 3-year-old girl with self-limited focal epilepsy of childhood (formerly called benign rolandic epilepsy) began having recurrent falls. Multiple clinical seizures were recorded on video EEG. The video documented generalized loss of tone resulting in falls, while the ictal EEG revealed one-second paroxysms of 4 Hz spike-slow-wave discharges in the left centrotemporal region, followed by a brief generalized electrodecrement for 400 milliseconds. These findings support the diagnosis of an atypical variant of benign epilepsy with centrotemporal spikes (BECTS), known as atonic-BECTS. Valproic acid was maximized. On follow-up, the patient was seizure-free with a normal EEG and normal development.

Discussion

Few prior publications describe atonic-BECTS. We present a child with atonic-BECTS whose ictal video EEG confirms atonic seizures. While atonic seizures typically occur with generalized epilepsies, our report highlights that they can present as an atypical manifestation of self-limited focal epilepsy in childhood.