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Double pathology in Rasmussen's syndrome

A window on the etiology?

From the Montreal Neurological Institute and Hospital (Drs. Hart, Andermann, and Rasmussen), Department of Neurology and Neurosurgery, McGill University, Montreal; Sainte-Justine Hospital and the University of Montreal (Dr. Robitaille), Quebec, Canada; and The Medical Centre at the University of California, San Francisco (Drs. Laxer and Davis).

Address correspondence and reprint requests to Dr. F. Andermann, Montreal Neurological Hospital and Institute, 3801 University Street, Montreal, Quebec H3A 2B4, Canada.

The syndrome of chronic encephalitis with epilepsy (Rasmussen's syndrome) typically occurs in children and is characterized by the development of intractable focal seizures, progressive hemiparesis and intellectual deterioration. The etiology is unknown, and the pathological abnormalities vary from those of active disease, with numerous microglial nodules, with or without neuronophagia, perivascular round cells and glial scarring, to those of remote disease, demonstrated by neuronal loss, gliosis and perivascular round cells but few microglial nodules. We describe five patients presenting with clinical features typical of Rasmussen's syndrome, in whom pathological examination showed a second, previously unsuspected pathology in addition to the changes of chronic encephalitis. Two of the patients had vascular abnormalities bearing some resemblance to cavernous angiomata, one had a tumor, one had tuberous sclerosis, and one the forme fruste of tuberous sclerosis. The coexistence of a second pathology in these patients may provide information about the underlying mechanism of this rare condition.

Received January 27, 1997. Accepted in final form August 20, 1997.

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