Hostname: page-component-76fb5796d-2lccl Total loading time: 0 Render date: 2024-04-27T04:33:02.284Z Has data issue: false hasContentIssue false
  • English
  • Français

Early Treatment of a Progressive Rasmussen's Like Syndrome with Ganciclovir

Published online by Cambridge University Press:  02 December 2014

Richard S. McLachlan ,
David Diosy  and
Simon Levin
Richard S. McLachlan*
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada
David Diosy
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada
Simon Levin
Affiliation:
Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada
*
London Health Sciences Centre-University Hospital, London, Ontario, N6A 5A5, Canada
Rights & Permissions [Opens in a new window]

Abstract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.
Background:

Escalating focal sensorimotor seizures, progressive neurologic deficit and cognitive decline with associated typical magnetic resonance imaging (MRI) findings or pathological confirmation constitute the syndrome of Rasmussen's encephalitis.

Methods:

Three patients with this clinical scenario had long-term follow-up after being given the antiviral drug ganciclovir 10 mg/kg/day IV for ten days within one to three months of disease onset.

Results:

Seizures occurred at least hourly and were localized to the Rolandic region. The MRIs were normal in one patient and in two showed changes consistent with ongoing seizures. Two patients, one whose short-term outcome was reported previously, had immediate and sustained cessation of seizures and resolution of their neurologic deficit. One patient with seizures from both hemispheres did not respond and went on to hemispherectomy that confirmed chronic encephalitis. Seizures continued in five other patients treated 6 to 72 months after disease onset.

Conclusion:

The sustained seizure control after ganciclovir in two of three patients suggests this drug may be effective when given early in the course of this subacute intractable focal epilepsy syndrome.

Type
Original Article
Information
Canadian Journal of Neurological Sciences , Volume 38 , Issue 2 , March 2011 , pp. 296 - 298
Copyright
Copyright © The Canadian Journal of Neurological 2011

References

1Rasmussen, T, Olszewski, J, Lloyd-Smith, D.Focal seizures due to chronic localized encephalitis. Neurology. 1958; 8: 435–45.Google Scholar
2Bien, CG, Granata, T, Antozzi, C, et al.Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain. 2005; 128: 454–71.Google Scholar
3McLachlan, RS, Levin, S, Blume, WT.Treatment of Rasmussen’s syndrome with ganciclovir. Neurology. 1996; 47: 925–8.Google Scholar
4Lee, JS, Juhász, C, Kaddurah, AK, Chugani, HT.Patterns of cerebral glucose metabolism in early and late stages of Rasmussen’s syndrome. J Child Neurol. 2001; 16: 798–805.Google Scholar
5Freeman, JM.Rasmussen’s syndrome: progressive autoimmune multifocal encephalopathy. Pediatr Neurol. 2005; 32: 295–9.Google Scholar
6Granata, T, Fusco, L, Gobbi, G, et al.Experience with immunomodulatory treatment in Rasmussen’s encephalitis. Neurology. 2003; 61: 1807–10.Google Scholar
7Bien, CG, Gleissner, U, Sassen, R, Widman, G, Urbach, H, Elger, CE.An open study of tacrolimus therapy in Rasmussen encephalitis. Neurology. 2004; 62: 2106–9.CrossRefGoogle ScholarPubMed
8Power, C, Poland, SD, Blume, WT, Girvin, JP, Rice, GPA.Cytomegalovirus and Rasmussen’s syndrome. Lancet. 1990; 336: 1282–4.CrossRefGoogle Scholar