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Serial MR Imaging of Adult-Onset Rasmussen's Encephalitis

Published online by Cambridge University Press:  23 July 2018

Myriam Irislimane ,
François Guilbert ,
Jean-Maxime Leroux ,
Lionel Carmant  and
Dang Khoa Nguyen
Myriam Irislimane
Affiliation:
Département de radiologie, Département de radiologie, Centre hospitalier de l'Université de Montréal - Hôpital Notre-Dame
François Guilbert
Affiliation:
Département de radiologie, Département de radiologie, Centre hospitalier de l'Université de Montréal - Hôpital Notre-Dame
Jean-Maxime Leroux
Affiliation:
Département de radiologie, Département de radiologie, Centre hospitalier de l'Université de Montréal - Hôpital Notre-Dame
Lionel Carmant
Affiliation:
Service de neurologie, Hôpital Sainte-Justine, Université de Montréal, Montréal, Québec, Canada
Dang Khoa Nguyen*
Affiliation:
Service de neurologie, Centre hospitalier de l'Université de Montréal - Hôpital Notre-Dame
*
Service de neurologie, Hôpital Notre-Dame du CHUM, 1560 rue Sherbrooke Est, Montréal, Québec, H2L 4M1, Canada
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A 52-year-old woman was referred for a progressive neurological condition which started a year before with continuous irregular twitching of the right facial and arm muscles as well as the tongue and palate, followed by progressive dysphagia, right hemiparesis, ataxia, dysphasia and dysarthria. Though magnetic resonance imaging (MRI) at clinical presentation and a year after were both normal (Figure A1), Rasmussen's encephalitis (RE) was strongly suspected and treatment with corticosteroids and immunoglobulins were begun. A third MRI, two years after onset, revealed mild T2 hyperintense subcortical white matter changes over the left perisylvian region (Figure A) which confirmed our initial suspicion of RE as she now met clinical, electrophysiological and morphological criterias for RE. While waiting for a cerebral biopsy after unsatisfactory response from antiepileptic drugs, corticosteroids and immunoglobulins, her condition markedly worsened less than three years after onset with the sudden occurrence of status epilepticus requiring intubation and continuous infusions of midazolam and propofol.

Type
Neuroimaging Highlights
Information
Canadian Journal of Neurological Sciences , Volume 38 , Issue 1 , January 2011 , pp. 141 - 142
Copyright
Copyright © Canadian Neurological Sciences Federation 2011

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