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Perioral myoclonia with absence seizures: a rare epileptic syndrome
1University of Istanbul, Istanbul Faculty of Medicine, Department of Neurology, çapa, Millet Cad 34390, Istanbul, Turkey.
Insights
This study details a case of absence seizures with perioral myoclonia, a rare epilepsy syndrome. Valproate and clonazepam showed varying efficacy in managing these complex absence seizures.
Area of Science:
- Neurology
- Epileptology
Background:
- Absence seizures (AS) are a type of generalized epilepsy.
- Perioral myoclonia is a rare seizure manifestation.
Observation:
- A boy presented with absence seizures and perioral movements starting at 1.5 years.
- Frequent absence status epilepticus (ASE) with perioral myoclonia occurred from age 12.
- Generalized tonic-clonic convulsions accompanied a prolonged ASE at age 17.
Findings:
- Continuous 3 Hz spike and wave paroxysms on EEG confirmed ASE.
- Intravenous clonazepam provided immediate clinical and EEG improvement.
- Diagnosis of Panayiotopoulos syndrome (perioral myoclonia with absence seizures) was established.
Implications:
- Highlights the importance of recognizing perioral myoclonia in absence seizures.
- Suggests clonazepam as a potential treatment for severe ASE episodes.
- Contributes to understanding rare epilepsy syndromes and their management.
Abstract:
We present the clinical and video-EEG data on an epileptic boy whose absence seizures with marked perioral movements had started at the age of 1.5 years. From age 12 years, he experienced frequent episodes of typical absence status epilepticus (ASE) lasting 1-2 hours with marked perioral myoclonia and moderate confusion. Initial therapy with carbamazepine was substituted by valproate because of worsening of the absence seizures. At the age of 17, the patient was admitted to our clinic with his usual, but long lasting ASE attack, accompanied by 2 generalized tonic-clonic convulsions. ASE was confirmed with the EEG which showed continuous 3 Hz spike and wave paroxysms with occasional normal intervals of 1-5 seconds. IV injection of clonazepam improved the clinical and EEG findings immediately. Video- EEG examination performed after a few weeks demonstrated typical absence seizures with perioral myoclonia. Based on the characteristics of seizure semiology, other clinical data and EEG findings, the patient was diagnosed as having the syndrome of "perioral myoclonia with absence seizures" described by Panayiotopoulos.