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Rasmussen encephalitis with active inflammation and delayed seizures onset
I Korn-Lubetzki1, C G Bien, J Bauer
1Neurological Service, Department of Pediatrics, Bikur Cholim Hospital, Jerusalem, Israel. ikl@md.huji.ac.il
Neurology
|March 24, 2004
Summary
Rasmussen encephalitis (RE) typically presents with severe focal epilepsy. However, this study reports two children with RE who did not experience seizures, expanding the known clinical spectrum of this neurological disorder.
Area of Science:
- Neurology
- Neuroscience
- Pediatric Neurology
Background:
- Rasmussen encephalitis (RE) is a rare, chronic inflammatory neurological disease affecting one cerebral hemisphere.
- Severe focal epilepsy is considered a hallmark clinical manifestation of RE.
Observation:
- Two pediatric patients presented with progressive hemiparesis and contralateral hemispheric atrophy.
- Histopathologic examination revealed features characteristic of RE in both cases.
Findings:
- Neither patient experienced seizures at the time of histologic diagnosis or during several months of follow-up.
- This observation challenges the traditional view of epilepsy as a mandatory symptom of RE.
Implications:
- The findings suggest that seizures may not be an obligatory presenting symptom of Rasmussen encephalitis.
- This expands the clinical spectrum of RE and may aid in earlier diagnosis for non-epileptic presentations.
- Further research is warranted to understand the diverse clinical phenotypes of RE.