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Malignant rolandic-sylvian epilepsy in children

Diagnosis, treatment, and outcomes

From the Department of Paediatrics, Division of Neurology (Drs. Otsubo, Chitoku, Ochi, and Snead, and I. Elliott), and Departments of Pathology (Dr. Jay), Surgery, Division of Neurosurgery (Dr. Rutka), and Psychology (Dr. Smith), The Hospital for Sick Children; and Bloorview Epilepsy Research Program (Drs. Otsubo, Chitoku, Ochi, and Snead, and I. Elliott), and University of Toronto, Ontario, Canada.

Address correspondence and reprint requests to Dr. H. Otsubo, Division of Neurology, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G IX8, Canada; e-mail: hiroshi.otsubo{at}sickkids.on.ca

Objective:— To describe the diagnosis, treatment, and outcomes in children with malignant rolandic-sylvian epilepsy (MRSE), defined as a form of epilepsy characterized by sensorimotor seizures, medical refractoriness, normal MRI, frontocentrotemporal EEG spikes, rolandic-sylvian spike sources on magnetoencephalography (MEG), and cognitive problems.

Methods:— A retrospective chart analysis of seven patients who had shown these characteristics and undergone extensive diagnostic testing, including MEG and intracranial video-EEG was performed.

Results:— Interictal scalp EEG spikes were seen over the frontocentrotemporal regions bilaterally (6) and unilaterally (1). MEG showed spike sources in the perisylvian region in two patients (both bilateral) and in the perirolandic fissure in five (two bilateral). Three patients required bilateral subdural strips to lateralize seizures before electrocorticography. Final electrocorticograms showed an ictal onset zone around the rolandic (four cases) and rolandic-sylvian regions (three cases). Six patients showed neuropsychological deficits. After cortical excision and multiple subpial transection, three were seizure free and four had seizures rarely (30 months’ mean follow-up). No child had a permanent deficit in sensorimotor or cognitive functions, although two showed exacerbation of preexisting attentional deficits. Tissue analysis showed definite evidence of neuronal migration disorders (3) and gliosis (2).

Conclusions:— MEG was helpful for localizing both malignant rolandic-sylvian neuronal activities and functional cortex. Successive neuropsychological assessments are necessary to detect cognitive deterioration and indicate remedial programming. If, after careful observation over at least 5 years, surgery is considered to control refractory seizures, intracranial video-EEG is needed to localize the epileptogenic zone.

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