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Acquired epileptiform opercular syndrome: a case report and results of single photon emission computed tomography and
E Tachikawa1, H Oguni, S Shirakawa
1Department of Pediatrics, Tokyo Woman's Medical University, 8-1 Kawada-cho, Shinjuku-ku, 162, Tokyo, Japan.
Insights
This study describes a child with acquired epileptiform opercular syndrome (AEOS), a rare epilepsy type. Clonazepam effectively treated the child's symptoms and EEG abnormalities, suggesting its therapeutic potential.
Area of Science:
- Neurology
- Epileptology
- Pediatric Neurology
Background:
- Epilepsy is a neurological disorder characterized by recurrent seizures.
- Localization-related epilepsy involves seizures originating in a specific brain area.
- Acquired epileptiform opercular syndrome (AEOS) is a recently proposed epileptic syndrome.
Observation:
- A 5-year-old girl presented with dysarthria, dysphagia, drooling, and right arm paresis.
- Continuous sharp-slow wave complexes were observed during sleep on EEG, unrelated to seizures.
- Single-photon emission computed tomography revealed high perfusion in the left posterior frontal region.
Findings:
- Clonazepam treatment led to complete resolution of clinical symptoms and EEG abnormalities within two weeks.
- A recurrence after 4 months showed EEG worsening, with characteristics matching AEOS.
- Computer-assisted EEG analysis localized the epileptic focus to the left sylvian fissure, causing secondary bilateral synchrony.
Implications:
- The findings support the hypothesis that continuous spike-and-wave activity during slow sleep (CSWS) can cause AEOS through secondary bilateral synchrony.
- This case highlights clonazepam as a potential treatment for AEOS.
- Understanding the pathophysiology of AEOS is crucial for improved diagnosis and management in pediatric epilepsy.
Abstract:
We report here a girl aged 5 years 3 months with cryptogenic localization-related epilepsy who showed a prolonged episode characterized by dysarthria, dysphagia, drooling and paresis of the right arm associated with almost continuous diffuse sharp-slow wave complexes during sleep. These symptoms were not directly related to seizures or to each sharp-slow wave complex revealed by examination during the video electroencephalographic (EEG) recording. The interictal single photon emission compute tomography showed a localized high perfusion area in the left posterior frontal region. The introduction of clonazepam completely controlled the clinical symptoms as well as the EEG abnormality within 2 weeks. After 4 months of remission, a similar episode recurred which was associated with aggravation of EEG. The clinical and EEG characteristics of this patient were identical to those of acquired epileptiform opercular syndrome (AEOS), a newly proposed epileptic syndrome, in which a transient operculum syndrome develops in association with continuous spike-and-wave activity during slow sleep (CSWS). Computer-assisted EEG analysis demonstrated that the epileptic EEG focus was located in the left sylvian fissure, and produced secondary bilateral synchronous sharp-slow complexes. The present study further supports the hypothesis that the electrical interference by CSWS creates bilateral opercular dysfunction through the mechanism of secondary bilateral synchrony, thus producing AEOS.
