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Atonic-BECTS: An unusual presentation of self-limited epilepsy in childhood
Varun Sampat1, Avantika Singh1, Hema Patel1
1Division of Pediatric Neurology, Department of Neurology Medical College of Wisconsin Milwaukee Wisconsin USA.
Insights
This case study highlights an unusual presentation of childhood epilepsy. A child experienced atonic seizures, a rare form of self-limited focal epilepsy with centrotemporal spikes (BECTS), which were successfully managed with medication.
Area of Science:
- Pediatric Neurology
- Epileptology
Background:
- Self-limited focal epilepsy of childhood with centrotemporal spikes (BECTS), previously known as benign rolandic epilepsy, is common.
- Atonic seizures are typically associated with generalized epilepsies, making their occurrence in focal epilepsy rare.
Purpose of the Study:
- To describe an unusual case of atonic variant of self-limited focal epilepsy of childhood with centrotemporal spikes.
- To aid in the accurate diagnosis and timely treatment of this rare epilepsy subtype.
Main Methods:
- Retrospective review of medical records, including clinical presentation, diagnostic evaluation, and treatment.
- Analysis of video electroencephalograms (EEGs) to document seizure activity.
Main Results:
- A 3-year-old girl presented with recurrent falls due to atonic seizures.
- Video EEG confirmed generalized loss of tone during seizures, with ictal EEG showing left centrotemporal spike-slow-wave discharges.
- Diagnosis of atonic BECTS was established, and treatment with valproic acid resulted in seizure freedom and normal development.
Conclusions:
- Atonic seizures can be an atypical manifestation of self-limited focal epilepsy in childhood.
- This case underscores the importance of recognizing atonic seizures in the context of BECTS for appropriate management.
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.
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