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June 25, 2015: Rasmussen encephalitis

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June 25, 2015

Rasmussen encephalitis

Rasmussen encephalitis typically occurs in children and is characterized by a progressive course of pharmacotherapy resistant epilepsia partialis continua, hemiparesis, and cognitive decline. Brain magnetic resonance imaging shows focal cortical atrophy of one cerebral hemisphere. Histopathology demonstrates a T cell mediated process, cortical inflammation, and neuronal loss in the affected hemisphere. In a few cases, autoantibodies against GluR3 have been detected. However, immunomodulatory therapies such as plasmapheresis have had very limited success. Typically, the only cure for seizures related to Rasmussen encephalitis is cerebral hemispherectomy.

References

  1. Varadkar S, Bien CG, Kruse CA et al. Rasmussen's encephalitis: clinical features, pathobiology, and treatment advances. Lancet Neurology 2014; 13: 195-205.

Submitted by Laura Bishop, Chief Neurology Resident, Resident Quality Improvement Council Chair, Wake Forest Baptist Health, Winston-Salem, NC.

Disclosures: Dr. Bishop reports no disclosures.

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