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Uncommon Presentation of Rasmussen's Encephalitis

Arvind Vyas1, Jaypalsing Ramdhan Ghunawat1, Amit Kumar Bagaria1

  • 1Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India.

Neurology India
|September 11, 2021
PubMed

Insights

Rasmussen's encephalopathy (RE), a rare neurological disorder, can present unusually with movement disorders or prolonged prodromal stages. Early MRI and immunomodulator treatment show promising results even in severe cases.

Area of Science:

  • Neurology
  • Neuroimmunology
  • Pediatric Neurology

Background:

  • Rasmussen's encephalopathy (RE) is a rare, chronic inflammatory neurological disease.
  • It typically manifests as intractable focal epilepsy, progressive weakness, and cognitive decline.

Observation:

  • RE presenting with movement disorders like hemidystonia or hemichorea is uncommon.
  • Prolonged prodromal stages, lasting years, are exceptionally rare in RE.

Findings:

  • This report details two RE cases: one with hemidystonia, another with a 7-year prodromal stage.
  • Magnetic resonance imaging (MRI) serves as a key biomarker for RE progression.
  • Despite severe hemi-atrophy, the second case showed a dramatic response to immunomodulators.

Implications:

  • Highlights the diverse clinical presentations of Rasmussen's encephalopathy.
  • Emphasizes the diagnostic value of MRI in tracking RE.
  • Suggests immunomodulatory therapies may be effective even in advanced stages of RE.
Keywords:
Cerebellar diaschisisRasmussen's encephalopathyhemi-dystoniatacrolimus

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