Rasmussen’s Encephalitis

Česká verzia

Authors: G. Timárová ¹; I. Lisá ¹; P. Mikula ²; M. Šimko In Memoriam ³
Authors place of work: II. Neurologická klinika LF UK a UN Bratislava ¹; Rádiologická klinika LF UK, SZU a UN Bratislava ²; I. interná klinika Dionýza Diešku, LF SZU a UN Bratislava ³
Published in the journal: Cesk Slov Neurol N 2016; 79/112(5): 500-512
Category: Minimonography
doi: https://doi.org/10.14735/amcsnn2016500

Summary

Rasmussen’s encephalitis is a rare chronic neurologic disease characterized by three main features: refractory epilepsy, progressive unihemispheric atrophy of the brain and progressive neurological deficit. This chronic focal encephalitis typically affects children but adolescents and adults are also involved. Neuropathological and immunological studies indicate that Rasmussen’s encephalitis is probably driven by a T-cell cytotoxic response with potential additional contribution by autoantibodies and activated microglia. Primary cause of the disease remains unsolved. MRI may be a good biomarker in Rasmussen’s encephalitis but we do not have relevant serological markers yet. Immunomodulatory treatments seem to slow disease progressiaon in Rasmussen’s encephalitis but long-term outcomes remain unclear. Functional hemispherectomy remains the only effective treatment for seizures but there are inevitable functional compromises associated with it. The decision and timing of surgery may be problematic in the absence of a dense neurological deficit, and if the patient is older. For patients, their families and treating physicians, choosing the right time to move from medical management to surgery is a real therapeutic dilemma. Due to poor prognosis, patients with susspected Rasmussen’s encephalitis should immediately be referred to an epilepsy centre.

Key words:
chronic encephalitis – refractory epilepsy – diagnostics – immunomodulatory therapy – hemispherectomy

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

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