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Rasmussen's encephalitis followed by lupus erythematosus.

K Lascelles1, A F Dean, Richard O Robinson

  • 1Newcomen Centre, Guy's Hospital, London, UK. karine.lascelles@kcl.ac.uk

Developmental Medicine and Child Neurology
|September 11, 2002
PubMed
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Rasmussen's encephalitis (RE) in a child led to intractable seizures and hemiplegia, successfully treated with hemispherectomy. Later, the patient developed systemic lupus erythematosus (SLE), suggesting a potential link between these conditions.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Autoimmune Diseases

Background:

  • Rasmussen's encephalitis (RE) is a rare, chronic inflammatory neurological disease affecting one cerebral hemisphere, typically in children.
  • It often presents with progressive neurological deficits, including motor impairments and intractable epilepsy.
  • The etiology of RE remains unclear, though autoimmune mechanisms are suspected.

Observation:

  • A 6-year-old female initially presented with hemichorea, progressing to hemiplegia and intractable focal to generalized tonic-clonic seizures.
  • Brain biopsy confirmed Rasmussen's encephalitis (RE).
  • A left hemispherectomy at 9.5 years resulted in seizure resolution and good recovery.

Findings:

  • At age 12, the patient developed recurrent abdominal pain, fever, and malar rash, consistent with systemic lupus erythematosus (SLE).

Related Experiment Videos

  • Serological tests confirmed SLE.
  • This case presents a potential association between RE and SLE.
  • Implications:

    • The co-occurrence of RE and SLE in this patient suggests a possible shared autoimmune pathogenesis or a link between these distinct conditions.
    • Further research is warranted to explore potential connections between neuroinflammatory and systemic autoimmune diseases.
    • This case highlights the complex and varied clinical manifestations of autoimmune disorders in children.