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Rasmussen's Encephalitis in a 58-Year-Old Female: Still a Variant?

Published online by Cambridge University Press:  02 December 2014

Gary R. W. Hunter
Affiliation:
Department of Neurology, University of Saskatchewan, SK, Canada
Jeffrey Donat
Affiliation:
Department of Neurology, University of Saskatchewan, SK, Canada
William Pryse-Phillips
Affiliation:
Memorial University, St. John's NL, Canada
Sheri Harder
Affiliation:
Department of Medical Imaging, University of Saskatchewan, SK, Canada
Christopher A. Robinson
Affiliation:
Saskatoon Health Region and Department of Pathology, University of Saskatchewan, SK, Canada
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Abstract:

Background:

We report the case of a 58-year-old female with clinical, radiological, and histopathological evidence of Rasmussen's encephalitis, representing the oldest confirmed case to date.

Case Summary:

The patient presented with complex partial seizures characterized by numbness of the left face and staring spells. These progressed to a state of epilepsia partialis continua with jerking of the left face, as well as severe cognitive impairment and loss of all communication. The patient responded well to Intravenous Immunoglobulin (IVIG) therapy despite early complications and with ongoing treatment is living independently with minimal cognitive impairment.

Conclusions:

This represents the oldest confirmed case of Rasmussen's encephalitis and suggests that this diagnosis should be considered in patients of any age with an appropriate clinical picture. We recommend IVIG as a first line therapy for adult cases of Rasmussen's encephalitis.

Résumé:

RÉSUMÉ: Contexte:

Nous rapportons le cas d’une femme de 58 ans présentant des signes cliniques, radiologiques et histopathologiques d’encéphalite de Rasmussen (ER), qui serait le cas confirmé le plus âgé rapporté jusqu’à maintenant.

Observation:

La patiente a consulté pour des crises d’épilepsie partielle complexe caractérisées par un engourdissement du côté gauche du visage et des absences épileptiques évoluant vers une épilepsie partielle continue avec secousses à l’hémiface gauche, déficit cognitif sévère et absence de toute communication. La patiente a bien répondu au traitement par gammaglobulines polyvalentes par voie intraveineuse malgré des complications précoces. Elle présente un déficit cognitif minime, vit de façon autonome et demeure sous traitement.

Conclusions:

Ce cas d’encéphalite de Rasmussen est le cas confirmé le plus âgé qui soit connu. Ce diagnostic devrait être envisagé chez les patients de tous âges ayant un tableau clinique suggestif de cette maladie. Nous recommandons les gammaglobulines polyvalentes par voie intraveineuse comme traitement de première ligne chez les adultes atteints d’ER.

Type
Original Articles
Copyright
Copyright © The Canadian Journal of Neurological 2006

References

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