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Bilateral Rasmussen Encephalitis: Good Outcome Following Hemispherotomy.

Noah Trapp1, Dominic O Co2, Susan Rebsamen3

  • 1Section of Pediatric Neurology, Department of Neurology, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.

Pediatric Neurology
|December 2, 2023
PubMed
Summary
This summary is machine-generated.

Bilateral Rasmussen encephalitis, a rare condition, can be treated with hemispherotomy, offering improved quality of life. This surgical approach shows promise even in severe, bilateral cases, challenging previous assumptions.

Keywords:
Bilateral RasmussenChronic encephalitisEpilepsy surgeryRasmussen encephalitisRasmussen syndrome

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Area of Science:

  • Neuroscience
  • Pediatric Neurology
  • Epileptology

Background:

  • Rasmussen encephalitis is a rare, severe neurological disorder typically affecting one brain hemisphere.
  • Bilateral cases are exceptionally rare, often associated with poor outcomes and limited treatment options.
  • Surgical interventions like hemispherotomy are frequently considered contraindicated in bilateral presentations.

Observation:

  • A 4-year-old male presented with escalating focal seizures and progressive neurological deficits indicative of bilateral Rasmussen encephalitis.
  • Despite initial transient responses to immunosuppressants, the patient developed super-refractory status epilepticus.
  • Histopathological examination confirmed bilateral involvement of the disease.

Findings:

  • The patient underwent a right functional hemispherotomy 4.5 years after symptom onset.
  • Post-surgery, the patient achieved an Engel 1D outcome classification, signifying excellent seizure control.
  • A 2.5-year follow-up revealed a substantially improved quality of life.

Implications:

  • This case challenges the contraindication of hemispherotomy in bilateral Rasmussen encephalitis.
  • Favorable outcomes may be achievable with hemispherotomy when seizures remain predominantly unihemispheric.
  • Highlights the potential of targeted surgical intervention in refractory, bilateral neurological conditions.