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Rasmussen's encephalitis: Early diagnostic criteria in children.

N Villeneuve1, A Lépine1, N Girard2

  • 1Service de neuropédiatrie, centre hospitalo universitaire de la Timone, 264, rue Saint-Pierre, 13005 Marseille, France.

Revue Neurologique
|May 14, 2022
PubMed
Summary
This summary is machine-generated.

Rasmussen's encephalitis (RE) is a rare brain disorder causing progressive neurodegeneration and drug-resistant epilepsy. Early diagnosis and targeted immunotherapies are crucial for managing this complex inflammatory condition.

Keywords:
ChildDiagnosticHistoryRasmussen's encephalitis

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Area of Science:

  • Neurology
  • Neuroimmunology
  • Pediatric Neurology

Background:

  • Rasmussen's encephalitis (RE) is a rare, chronic inflammatory brain disorder.
  • It leads to progressive neurodegeneration in one cerebral hemisphere, causing functional loss and drug-resistant epilepsy.
  • Diagnosis is challenging due to the lack of specific markers, particularly in early stages.

Purpose of the Study:

  • To review the diagnostic complexities of Rasmussen's encephalitis.
  • To discuss current and emerging therapeutic strategies for RE.
  • To highlight challenges in early detection and management.

Main Methods:

  • Review of clinical, electroencephalographic, radiological, and biochemical diagnostic criteria for RE.
  • Analysis of treatment outcomes for anti-seizure medications, immunomodulatory therapies, and hemispherotomy.
  • Exploration of the immunopathogenesis and potential of biotherapies.

Main Results:

  • Seizures in RE are often refractory to standard anti-seizure medications and conventional immunotherapies.
  • Hemispherotomy can achieve seizure cessation but results in permanent motor and cognitive deficits.
  • Evidence suggests a T-cell mediated immunopathogenic mechanism underlying RE.

Conclusions:

  • Early diagnosis of RE is critical to prevent severe neurological and cognitive consequences.
  • Novel biotherapies targeting cytokines offer promising therapeutic avenues.
  • Managing RE requires a multidisciplinary approach, addressing diagnostic, therapeutic, and surgical challenges.