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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
Summary
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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