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Bilateral Rasmussen encephalitis: postmortem documentation in a five-year-old.

Sharon M Tobias1, Yves Robitaille, William F Hickey

  • 1Pathology Department, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire 03756-0001, USA.

Epilepsia
|February 13, 2003
PubMed
Summary
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This case study reviews Rasmussen encephalitis, a rare brain disease causing seizures and cognitive decline. It highlights an unusual bilateral presentation in a young child, leading to rapid progression and death.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Neuroimmunology

Background:

  • Rasmussen encephalitis is a rare, chronic inflammatory neurological disease affecting the brain.
  • It typically presents with unilateral cerebral hemisphere involvement, leading to progressive neurological deficits.
  • Characterized by intractable focal epilepsy, cognitive decline, and motor deficits.

Observation:

  • This report details a unique case of Rasmussen encephalitis with initial onset at age 2 years.
  • The disease exhibited bilateral cerebral involvement, suspected within 5 months and confirmed by biopsies.
  • The patient experienced severe progression, succumbing to the illness by age 5 despite interventions.

Findings:

  • The case demonstrates Rasmussen encephalitis with bilateral frontal lobe involvement, diverging from the typical unilateral presentation.

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  • Histopathological findings from biopsies and autopsy confirmed the diagnosis and bilateral nature of the disease.
  • Rapid disease progression and severity were noted, underscoring the aggressive nature of this variant.

    • Implications:

    • This case expands the understanding of Rasmussen encephalitis presentation, particularly its rare bilateral form.
    • It emphasizes the need for vigilance in diagnosing atypical presentations in pediatric patients.
    • Further research into the pathogenesis and treatment of bilateral Rasmussen encephalitis is warranted.