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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
Summary
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.
