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Late onset Rasmussen's encephalitis with triple pathology

J Y Cheong1, C Wong, A Bleasel

  • 1Department of Neurosurgery Westmead Hospital, Hawksbury Road, Westmead, New South Wales 2145, Australia. Santiago-james@hotmail.com

Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
|October 6, 2009
PubMed

Insights

This case study details a rare, delayed-onset Rasmussen's encephalitis variant. Hemispherectomy successfully treated refractory seizures in a patient with triple pathology, including cortical dysplasia.

Area of Science:

  • Neurology
  • Neurosurgery
  • Pathology

Background:

  • Rasmussen's encephalitis is a rare autoimmune disease causing progressive neuronal destruction in one brain hemisphere, leading to intractable focal seizures.
  • Delayed onset and triple pathology are exceptionally uncommon presentations of this condition.

Observation:

  • A 27-year-old male with a 16-year history of seizures presented with medically refractory focal seizures.
  • Investigations identified seizure onset in the right frontotemporal region, leading to a right frontotemporal lobectomy.
  • Post-surgery, seizures worsened, and epilepsia partialis continua developed, necessitating further intervention.

Findings:

  • The patient underwent a right hemispherectomy, which resulted in complete seizure freedom.
  • Histopathological examination revealed a combination of old ischemic changes, type II cortical dysplasia, and stage II Rasmussen's encephalitis.

Implications:

  • This case highlights the potential efficacy of hemispherectomy in severe, refractory Rasmussen's encephalitis, even with complex underlying pathologies.
  • The presence of triple pathology underscores the need for comprehensive diagnostic evaluation in atypical neurological presentations.
  • Understanding these rare variants can inform diagnostic and therapeutic strategies for autoimmune encephalitis and related neurological disorders.

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