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Status epilepticus in benign rolandic epilepsy manifesting as anterior operculum syndrome
V Colamaria1, V Sgrò, R Caraballo
1Child Neuropsychiatry, University of Verona, Italy.
Insights
This study details a rare case of prolonged seizures (partial status epilepticus) in benign epilepsy of childhood with rolandic spikes (BECRS). Effective treatment was achieved with standard antiepileptic drugs.
Area of Science:
- Pediatric Neurology
- Epileptology
Background:
- Benign epilepsy of childhood with rolandic spikes (BECRS) is a common childhood epilepsy syndrome.
- Partial status epilepticus (SE) is a rare but serious complication.
Observation:
- A case of partial SE in a child diagnosed with BECRS is presented.
- The child experienced prolonged seizures affecting the mouth and pharynx, with symptoms including speech arrest, sialorrhea, and drooling.
- Clinical presentation during SE mimicked opercular or Foix-Chavany-Marie syndrome.
Findings:
- Clinical and electroencephalogram (EEG) findings confirmed BECRS.
- EEG demonstrated continuous spike-waves during slow sleep.
- Interictal centrotemporal spikes were inhibited by voluntary mouth/tongue movements.
Implications:
- This case expands the understanding of potential SE presentations in BECRS.
- It highlights the importance of recognizing SE in children with BECRS.
- Standard antiepileptic drug therapy, including diazepam, clobazam, and valproate, proved effective for SE remission.
Abstract:
We report the fourth case of partial status epilepticus (SE) in benign epilepsy of childhood with rolandic spikes (BECRS). The child suffered long-lasting attacks involving the mouth and pharynx, clinically manifest as speech arrest, sialorrhea, and drooling. Both clinical and electroencephalogram (EEG) data were compatible with the diagnosis of BECRS. Only during SE was the clinical picture similar to that observed in the operculum or Foix-Chavany-Marie syndrome. SE remission was obtained with the usual antiepileptic drug therapy (diazepam, clobazam, valproate). EEG records showed additional patterns of continuous spike-waves during slow sleep and specific inhibition and blocking of interictal centrotemporal spikes by mouth and/or tongue voluntary movements.