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Rasmussen's syndrome.

T Granata1

  • 1Department of Child Neurology, National Neurological Institute C. Besta, Milan, Italy.

Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
|November 5, 2003
PubMed
Summary

Rasmussen's encephalitis (RE) involves seizures and neurological decline. Immunomodulatory treatments, like intravenous immunoglobulin, show promise, especially for adult-onset cases, but require further study for childhood-onset RE.

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Area of Science:

  • Neurology
  • Immunology
  • Pediatrics

Background:

  • Rasmussen's encephalitis (RE) is a rare neurological disorder.
  • It presents with focal seizures, progressive neurological deterioration, and hemispheric atrophy.
  • Inflammatory histopathology is a key feature of RE.

Purpose of the Study:

  • To explore the disimmune component in Rasmussen's encephalitis pathogenesis.
  • To evaluate the potential of immunomodulatory treatments for RE.
  • To assess the efficacy of intravenous immunoglobulins (IVIg) in RE.

Main Methods:

  • Investigated serum antibodies against glutamate receptor GluR3 in animal models and patients.
  • Reviewed outcomes of surgical hemispherectomy as a treatment for RE.
  • Analyzed existing data on IVIg use in adult-onset RE.

Main Results:

  • Serum antibodies against GluR3 suggest a disimmune pathogenesis in RE.
  • Surgical hemispherectomy is the only established treatment to halt disease progression.
  • IVIg has shown encouraging results in adult-onset RE.

Conclusions:

  • A disimmune component, potentially involving GluR3 antibodies, is implicated in RE.
  • Immunomodulatory therapies, particularly IVIg, are promising for RE management.
  • Further research is needed to establish optimal IVIg protocols for childhood-onset RE.

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