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.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Brain and DevelopmentAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Acquired epileptiform opercular syndrome: a second case report, review of the literature, and comparison to the Landau-Kleffner syndrome.Epilepsia. 1995; 36: 1050-1057
- Prolonged intermittent drooling and oromotor dyspraxia in benign childhood epilepsy with centrotemporal spikes.Epilepsia. 1989; 30: 564-568
- The operculum syndrome.in: Vinken P.J. Bruyn G.W. Handbook of clinical neurology. North-Holland, Amsterdam1969: 776-783
- Acquired epileptiform aphasia in children (Landau-Kleffner syndrome).J Clin Neurophysiol. 1991; 8: 288-298
- Measurement of small time differences between EEG channels: method and application to epileptic seizure propagation.Electroenceph clin Neurophysiol. 1983; 56: 501-514
- Status epilepticus of benign partial epilepsies in children: report of two cases.Epilepsia. 1987; 28: 351-355
- Status epilepticus in benign rolandic epilepsy manifesting as anterior operculum syndrome.Epilepsia. 1991; 32: 329-334
- Speech and oromotor deficits of epileptic origin in benign partial epilepsy of childhood with rolandic spikes (BPERS). Relationship to the acquired aphasia-epilepsy syndrome.Neuropediatrics. 1993; 24: 83-87
- Continuous spike-and-wave activity during slow-wave sleep: syndrome or EEG pattern?.Epilepsia. 1999; 40: 1593-1601
- Suprabulbar paresis.Dev Med Child Neurol. 1974; 16: 1-33
- Landau-Kleffner syndrome. Treatment with subpial intracortical transection.Brain. 1995; 118: 1529-1546
- The spectrum of neuropsychiatric abnormalities associated with electrical status epilepticus in sleep.Brain Dev. 2000; 22: 279-295
Article info
Publication history
Accepted:
February 8,
2001
Received in revised form:
February 6,
2001
Received:
September 5,
2000
Identification
Copyright
© 2001 Elsevier Science B.V. Published by Elsevier Inc. All rights reserved.
