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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 ,
Jeffrey Donat ,
William Pryse-Phillips ,
Sheri Harder  and
Christopher A. Robinson
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
*
#107 445 Bayfield Cr., Saskatoon, Saskatchewan, S7V 1J1, Canada.
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Abstract:

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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
Information
Canadian Journal of Neurological Sciences , Volume 33 , Issue 3 , August 2006 , pp. 302 - 305
Copyright
Copyright © The Canadian Journal of Neurological 2006

References

1. Wennberg, R, Nag, S, McAndrews, MP, Lozano, AM, Farb, R, Mikulis, D. Chronic (Rasmussen’s) encephalitis in an adult. Can J Neurol Sci. 2003 Aug;30(3):263–5.CrossRefGoogle ScholarPubMed
2. McLachlan, RS, Girvin, JP, Blume, WT, Reichman, H. Rasmussen’s chronic encephalitis in adults. Arch Neurol. 1993 Mar;50(3):269–74.CrossRefGoogle ScholarPubMed
3. Vadlamudi, L, Galton, CJ, Jeavons, SJ, Tannenberg, AE, Boyle, RS. Rasmussen’s syndrome in a 54 year old female: more support for an adult variant. Clin Neurosci. 2000 Mar;7(2):154–6.CrossRefGoogle Scholar
4. Villani, F, Spreafico, R, Farina, L, Giovagnoli, AR, Bernasconi, P, Granata, T, et al. Positive response to immunomodulatory therapy in an adult patient with Rasmussen’s encephalitis. Neurology. 2001 Jan;56(2):248–50.CrossRefGoogle Scholar
5. Hart, YM, Andermann, F, Fish, DR, Dubeau, F, Robitaille, Y, Rasmussen, T, et al. Chronic encephalitis and epilepsy in adults and adolescents: a variant of Rasmussen’s syndrome? Neurology. 1997 Feb;48(2):418–24.CrossRefGoogle ScholarPubMed
6. Granata, T, Fusco, L, Gobbi, G, Freri, E, Ragona, F, Broggi, G, et al. Experience with immunomodulatory treatments in Rasmussen’s encephalitis. Neurology. 2003;61(12):1807–1.CrossRefGoogle ScholarPubMed
7. Leach, JP, Chadwick, DW, Miles, JB, Hart, IK. Improvement in adult-onset Rasmussen’s encephalitis with long-term immunomodulatory therapy. Neurology. 1999 Mar;52(4):738–42.CrossRefGoogle ScholarPubMed
8. Gray, F, Serdaru, M, Baron, H, Daumas-Duport, C, Loron, P, Sauron, B, et al. Chronic localized encephalitis (Rasmussen’s) in an adult with epilepsia partialis continua. J Neurol Neurosurg Psychiatry. 1987 Jun;50(6):747–51.CrossRefGoogle Scholar
9. Hennessy, MJ, Koutroumanidis, M, Dean, AF, Jarosz, J, Elwes, RD, Binnie, CD, et al. Chronic encephalitis and temporal lobe epilepsy: a variant of Rasmussen’s syndrome? Neurology. 2001; 56:678–81.CrossRefGoogle ScholarPubMed
10. Lagrange, AH, Blaivas, M, Gomez-Hassan, D, Malow, BA. Rasmussen’s syndrome and new-onset narcolepsy, cataplexy, and epilepsy in an adult. Epilepsy Behav. 2003 Dec;4(6):788–92.CrossRefGoogle ScholarPubMed
11. Takahashi, Y, Mori, H, Mishina, M. Autoantibodies and cell-mediated autoimmunity to NMDA-type GluRepsilon2 in patients with Rasmussen’s encephalitis and chronic progressive epilepsia partialis continua. Epilepsia. 2005;46 Suppl 5:S152–8.CrossRefGoogle ScholarPubMed
12. Whitney, KD, McNamara, JO. GluR3 autoantibodies destroy neural cells in a complement-dependent manner modulated by complement regulatory proteins. J Neurosci. 2000 Oct 1;20(19): 7307–16.CrossRefGoogle Scholar
13. Andrews, PI, McNamara, JO. Rasmussen’s encephalitis: an autoimmune disorder? Curr Opin Neurobiol. 1996 Oct;6(5):673–8.CrossRefGoogle ScholarPubMed
14. Palmer, CA, Geyer, JD, Keating, JM. Rasmussen’s encephalitis with concomitant cortical dysplasia: the role of GluR3. Epilepsia. 1999 Feb;40(2):242–7.CrossRefGoogle ScholarPubMed
15. Mantegazza, R, Bernasconi, P, Baggi, F, Spreafico, R, Ragona, F, Antozzi, C, et al. Antibodies against GluR3 peptides are not specific for Rasmussen’s encephalitis but are also present in epilepsy patients with severe, early onset disease and intractable seizures. J Neuroimmunol. 2002 Oct;131(1-2):179–85.CrossRefGoogle Scholar
16. Wiendl, H, Bien, CG, Bernasconi, P, Fleckenstein, B, Elger, CE, Dichgans, J, et al. GluR3 antibodies: prevalence in focal epilepsy but no specificity for Rasmussen’s encephalitis. Neurology. 2001 Oct ;57(8):1511–4.CrossRefGoogle ScholarPubMed