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 Volume 70, Number 5, January 29, 2008
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NEUROLOGY 2008;70:374-377
© 2008 American Academy of Neurology

Limited chronic focal encephalitis

Another variant of Rasmussen syndrome?

A. Gambardella, MD, F. Andermann, MD, S. Shorvon, MD, E. Le Piane, MD and U. Aguglia, MD

From the Institute of Neurology (A.G.), University Magna Graecia Catanzaro, Italy; Montreal Neurological Institute (F.A.), Montreal PQ, Canada; Institute of Neurology (S.S.), National Hospital for Neurology and Neurosurgery, Queen Square, London, UK; Regional Epilepsy Center (E.L.P., U.A.), Azienda Ospedaliera Reggio Calabria, Italy.

Address correspondence and reprint requests to Dr. Antonio Gambardella, Institute of Neurology University Magna Graecia, Catanzaro Viale Europa, Località Germaneto, 88100 Catanzaro, Italy a.gambardella{at}isn.cnr.it.

Objective: To describe a more limited and less malignant form of Rasmussen encephalitis (RE).

Methods: Three subjects (all women; 37, 31, and 32 years of age) developed childhood or late onset chronic focal encephalitis, with a relatively nonprogressive form of the disorder.

Results: In our patients, clinical features were dominated by partial seizures without marked focal motor deficit and in two with choreo-dystonic movements. The diagnosis of RE was supported by histologic examination and anatomic and functional MRI.

Conclusions: These cases extend the phenotypic presentations of Rasmussen encephalitis and confirm Theodore Rasmussen's suggestion that there may be mild and nonprogressive forms of the disease.

Abbreviations: RE = Rasmussen encephalitis; VGKC = voltage-gated potassium-channel.

S.D.S. is supported by a cooperative group grant from the Medical Research Council.

Disclosure: The authors report no conflicts of interest.

Received January 8, 2007. Accepted in final form August 8, 2007.






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