Rasmussen encephalitis

Tiziana Granata1, Frederick Andermann

  • 1Department of Pediatric Neuroscience, Carlo Besta Neurological Institute, Milan, Italy.

Insights

Rasmussen encephalitis (RE) is a rare inflammatory brain disease causing progressive neurological decline and severe seizures. Treatment requires personalized strategies, balancing surgery for seizure control with immunotherapy to slow disease progression.

Area of Science:

  • Neurology
  • Immunology
  • Neuroinflammation

Background:

  • Rasmussen encephalitis (RE) is a rare, unilateral, inflammatory brain disorder.
  • Characterized by progressive neurological deficits and intractable seizures, often epilepsia partialis continua.
  • Distinctive MRI findings include unilateral atrophy and focal cortical/subcortical signal changes.

Purpose of the Study:

  • To summarize the key features, diagnostic challenges, and therapeutic approaches for Rasmussen encephalitis.
  • To highlight the need for tailored treatment strategies based on individual patient needs.

Main Methods:

  • Review of clinical, neuroimaging (MRI), and histopathological findings in Rasmussen encephalitis.
  • Analysis of treatment outcomes for antiepileptic drugs, surgery, and immunotherapy.
  • Emphasis on differential diagnosis to exclude other causes of focal encephalitis and seizures.

Main Results:

  • Histopathology reveals T-cell dominated inflammation, microglial activation, neuronal loss, and astrogliosis.
  • Surgical hemispherectomy offers high seizure freedom but causes permanent deficits.
  • Immunotherapy can slow disease progression but has limited impact on seizure burden.

Conclusions:

  • Diagnosis of RE is challenging, especially early on, requiring exclusion of other conditions.
  • Treatment must be individualized, considering the trade-offs between seizure control and neurological deficits.
  • A multidisciplinary approach is crucial for managing this complex neuroinflammatory disease.

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