Partial Status Epilepticus in Benign Rolandic Epilepsy: Clinical Accompaniment with Focal [dsquote]Interictal Discharges[dsquote].
Abstract number :
1.149
Submission category :
Year :
2001
Submission ID :
1198
Source :
www.aesnet.org
Presentation date :
12/1/2001 12:00:00 AM
Published date :
Dec 1, 2001, 06:00 AM
Authors :
D.L. Gregory, Neurophysiology, BC Children[ssquote]s Hosp., Vancouver, BC, Canada; K. Farrell, MB BS, FRCP, Paediatrics, UBC/BC Children[ssquote]s Hosp, Vancouver, BC, Canada; P.K.H. Wong, MD, FRCP, Paediatrics/EEG, UBC/BC Children[ssquote]s Hosp, Vancouv
RATIONALE: Opercular status, a form of partial status epilepticus characterized by dysarthria, drooling, eyelid twitching and head nods, has been reported in 4 children with benign Rolandic epilepsy of childhood (BREC). We describe 2 children with opercular status epilepticus and BREC, and suggest a possible mechanism for this rare disorder.
METHODS: The children, aged 4 & 6 years, each had a previous history of typical benign Rolandic seizures and EEG interictal discharges compatible with the diagnosis of BREC. Both children had a history of independent left and right partial motor seizures. In addition, there was a history of prolonged episodes of persistent drooling, dysarthria, eyelid twitching or poor head control. We studied these episodes in both patients using video/EEG.
RESULTS: The EEG done during the episodes demonstrated very frequent centrotemporal spike discharges with a typical temporal-frontal dipole field. The semiology associated with the discharges comprised synchronous clonic movements of the periorbital muscles, drooling and/or brief loss of head control with speech arrest. In the one patient tested, inhibition of the discharges and clinical manifestations accompanied motor activity or sensory stimulation around the mouth and intravenous diazepam.
CONCLUSIONS: Although the typical spike dipole often occurs independently on either hemisphere, a history of independent right and left benign Rolandic seizures is uncommon. This occurrence in both of our patients suggests that the predisposition to independent right and left Rolandic seizures may play a role in the mechanism of opercular status. This would be analagous to the the anterior operculum syndrome in adults with cerebrovascular disease, where the clinical symptoms are not seen after a first stroke but only when there is a further stroke involving the contralateral hemisphere.
Prolonged episodes of dysarthria, drooling, eyelid twitching and head nods are clinical features of a rare seizure type in BREC. The occurence of independent right and left typical Rolandic seizures in our two children suggests that bilateral Rolandic dysfunction may play a role in the pathophysiology of this form of status epilepticus.
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