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T-Cell Mediated Diseases

Rasmussen’s Encephalitis

  • Rasmussen’s Encephalitis is a chronic, inflammatory brain disease that generally starts in one hemisphere of the brain[3]. It most often occurs in children under the age of 15
  • The etiology of Rasmussen’s Encephalitis is unknown; however it is thought to be a T-cell mediated autoimmune disorder. It is not clear what triggers the abnormal immune response. While some believe that a viral infection is the cause, there is no evidence to support this[1].
  • It is characterized by severe and periodic seizures, loss of motor skills, speech difficulties, hemiparesis, and mental deterioration,
  • Critical aspects of diagnosis are the history, Electroencephalography (EEG), Magnetic Resonance Imaging (MRI), and brain biopsy[1].
  • Treatment of RE has met with difficulties. Standard anticonvulsant therapy does not stop the seizures[2].
  • Corticosteroids are effective in the short-term, and there is promising research being done into the effects of immunosuppressive therapy[2].
  • Surgery has been a common treatment option, using hemispherectomy to separate and remove the affected hemisphere[2].

References:

  1. Bien CG, Granata T, Antozzi C, Cross JH, Dulac O, Kurthen M, Lassmann H, Mantegazza R, Villemure JG, Spreafico R, Elger CE. Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: a European consensus statement. Brain, 2005. 128(Pt 3): p. 454-71.
  2. Bien CG, Schramm J. Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma. Epilepsy Res, 2009. 86(2-3): p. 101-12.
  3. "Rasmussen's Encephalitis Information Page." National Institute of Neurological Disorders and Stroke. Web. 26 June 2012. < Treatment of Rasmussen encephalitis half a century after its initial description: promising prospects and a dilemma >.

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