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Montreal Neurological Institute and Hospital (Drs. Hart, Andermann, and Sherwin), Montreal, Canada; the Hospital for Sick Children (Drs. Cortez and Hwang), Toronto, Canada; the National Hospital for Neurology and Neurosurgery (Dr. Fish), London, UK. Hôpital Saint Vincent de Paul (Dr. Dulac), Paris, France; the Montreal Children's Hospital (Dr. Silver), Montreal, PQ Canada; the Hospital Juan Garrahan (Drs. Fejerman and Caraballo), Buenos Aires, Argentina; and the Hospital for Sick Children (Dr. Cross), London, UK
We treated 19 patients with Rasmussen's syndrome (chronic encephalitis and epilepsy)–a rare progressive disorder of unknown etiology causing focal epilepsy, hemiparesis, and intellectual deterioration–with intravenous immunoglobulins, high-dose steroids, or both, to control seizures and improve the end point of the disease. Ten of 17 patients receiving steroids, and eight of nine patients receiving immunoglobulins, had some reduction of seizure frequency in the short term. Improvement in hemiparesis was slight. The effect of these drugs in ameliorating the end point of the disease in the long term remains unknown, and further multicenter studies with standardized protocols are warranted.
Address correspondence to Dr. F. Andermann, Montreal Neurological Institute and Hospital, 3801 University Street, Montreal, PQ, Canada H3A 2B4.
Dr. Hart is supported by a Harrison Memorial Lectureship funded by the Neurosciences Research Fund of Atkinson Morley's Hospital, London, UK.
Received August 19, 1993. Accepted for publication in final form December 13, 1993.
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