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Rasmussen’s Syndrome

Aetiology, Clinical Features and Treatment Options

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Abstract

Rasmussen’s syndrome (chronic encephalitis with epilepsy) is a rare neurological disorder characterised by progressive dysfunction of one cerebral hemisphere. Clinical and radiological features of the syndrome include intractable epilepsy, cognitive and motor decline, progressive unilateral cortical atrophy and the occurrence of epilepsia partialis continua (EPC) in many cases. Histopathological findings include the presence of perivascular cuffing, microglial nodules, gliosis and spongiosis. The aetiology of Rasmussen’s encephalitis is unknown but viral, autoimmune and genetic mechanisms have been postulated. Proposed therapies have included anticonvulsants, corticosteroids, antiviral agents, interferona, immunogobulin and plasmapheresis. The mainstay of therapy is hemispherectomy. To date, no medical therapies have permanently halted neurological deterioration. A better understanding of pathogenesis is required to develop safe and effective medical therapies.

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Authors and Affiliations

  1. Division of Paediatric Neurology, University of Florida, P.O. Box 100296, Gainesville, Florida, 32610, USA

    Thang X. Tran, Jonathan A. Day, Paul R. Carney &  Bernard L. Maria

  2. Department of Pathology, Immunology, and Laboratory Medicine, University of Florida, Gainesville, Florida, USA

    Thomas A. Eskin

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  1. Thang X. Tran
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Correspondence to Bernard L. Maria.

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Tran, T.X., Day, J.A., Eskin, T.A. et al. Rasmussen’s Syndrome. Mol Diag Ther 14, 343–354 (2000). https://doi.org/10.2165/00023210-200014050-00002

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