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Rasmussen's Syndrome: Intractable Epilepsy and Progressive Neurological Deterioration From a Unilateral Central Nervous System Disease

Published online by Cambridge University Press:  07 November 2014

Paul R. Carney
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Abstract

Rasmussen's syndrome (chronic encephalitis with epilepsy) is a rare neurological disorder of unknown cause characterized by severe epilepsy, hemiplegia, dementia, and inflammation of the brain, and progressive functional and structural destruction of a single cerebral hemisphere. While one mechanism underlying the pathogenesis of Rasmussens encephalitis has been hypothesized to be mediated by production of excitotoxic GluR3 autoantibodies to the a-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) receptor, other neuropathological etiologies have also been indicated. Proposed therapies have included antiepileptics, steroids, antiviral agents, alpha-interferon, and immunoglobulin. The mainstay of therapy is surgical hemispherectomy. To date, no medical therapies have permanently halted neurologic deterioration.

Type
Feature Article
Information
CNS Spectrums , Volume 6 , Issue 5: Neuroimmunology , May 2001 , pp. 398,409 - 416
Copyright
Copyright © Cambridge University Press 2001

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