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Eponym : Rasmussen syndrome.

Mario Mastrangelo1, Rosanna Mariani, Alessandra Menichella

  • 1Child Neurology Division, Department of Pediatrics, La Sapienza-University of Rome, Viale Regina Elena, 324 00161 Rome, Italy. mari0mastrangelo@tiscali.it

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Summary

Rasmussen's syndrome (RS) is a rare autoimmune encephalopathy causing severe seizures and cognitive decline. Functional hemispherectomy offers the best chance to halt disease progression and improve quality of life.

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Area of Science:

  • Neurology
  • Immunology
  • Neuroscience

Background:

  • Rasmussen's syndrome (RS) is a rare, acquired, progressive inflammatory encephalopathy.
  • Characterized by drug-resistant partial seizures and cognitive deterioration affecting a single brain hemisphere.

Purpose of the Study:

  • To review the current understanding of Rasmussen's syndrome.
  • To discuss potential therapeutic strategies for managing this condition.

Main Methods:

  • Literature review of etiopathogenesis and treatment options for RS.
  • Analysis of the role of autoimmune processes, including T lymphocytes and autoantibodies against GluR3Ab.
  • Evaluation of surgical intervention (functional hemispherectomy) as a definitive treatment.

Main Results:

  • The presumed mechanism involves a complex autoimmune process targeting the ionotropic glutamate receptor subunit 3 (GluR3Ab).
  • Antiepileptic drugs and immunomodulatory therapies provide only temporary relief.
  • Functional hemispherectomy effectively arrests disease progression, controls seizures, and improves quality of life.

Conclusions:

  • Rasmussen's syndrome is an autoimmune disorder, not a viral infection.
  • Surgical hemispherectomy is the most effective treatment for halting RS and improving patient outcomes.