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[Rasmussen syndrome]

R Caraballo1, S Tenembaum, R Cersósimo

  • 1Servicio de Neurología, Hospital de Pediatría Juan P. Garrahan, Buenos Aires, Argentina.

Revista De Neurologia
|July 11, 1998
PubMed
Summary
This summary is machine-generated.

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Rasmussen's encephalitis (RE) is an autoimmune neurological disorder. Intravenous immunoglobulin therapy provided temporary seizure control and halted neurological decline in 55% of patients, with surgery remaining the primary treatment for epilepsy.

Area of Science:

  • Neurology
  • Immunology
  • Pediatrics

Context:

  • Rasmussen's encephalitis (RE) is a rare, severe neurological disorder of probable autoimmune origin.
  • It is characterized by progressive neurological deterioration and intractable epilepsy.
  • Antibodies against NMDA receptors are often present.

Purpose:

  • To review the clinical experience with Rasmussen's encephalitis at an Argentinian Paediatric Neurology Service over seven years.
  • To analyze clinical and diagnostic findings in a cohort of 12 patients.

Summary:

  • The study analyzed 12 patients diagnosed with Rasmussen's encephalitis based on established criteria.
  • Seizure types included simple partial motor, secondarily generalized, and complex partial seizures.
  • Neuroimaging revealed cerebral hemiatrophy in all cases, with other abnormalities noted in some.

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  • Electroencephalography showed limited correlation with continuous partial seizures.

    • Impact:

    • Intravenous immunoglobulin (IVIg) therapy resulted in transient stabilization of neurological decline and seizure control in 55% of patients.
    • Surgical intervention remains the recommended treatment for managing refractory epileptic seizures in RE.
    • This case series contributes to understanding the clinical spectrum and management of RE in a pediatric population.