Rasmussen encephalitis: Predisposing factors and their potential role in unilaterality
Susanne Fauser1, Christian E Elger2, Friedrich Woermann1,3
1Department of Epileptology (Mara Hospital), Medical School, Bielefeld University, Bielefeld, Germany.
Epilepsia
|November 25, 2021
Summary
Rasmussen encephalitis (RE) may be predisposed by perinatal complications and facial autoimmune conditions. Ipsilateral early lesions, whether brain or autoimmune, may explain RE's unilateral presentation.
Area of Science:
- Neurology
- Immunology
- Pediatric Neurology
Background:
- Rasmussen encephalitis (RE) is a rare, progressive inflammatory brain disease affecting one hemisphere.
- The etiology of RE remains largely unknown, posing diagnostic and therapeutic challenges.
Purpose of the Study:
- To investigate potential predisposing factors, including comorbidities and laterality, in Rasmussen encephalitis.
- To compare risk factors between RE patients and control groups with genetic generalized epilepsy and focal cortical dysplasia Type II.
Main Methods:
- Retrospective comparison of medical histories from 160 RE patients against 154 genetic generalized epilepsy and 148 FCD II patients.
- Analysis of demographic data, age at onset, affected hemisphere, perinatal history, and associated autoimmune conditions.
Main Results:
- RE predominantly affected females with onset after age 7, with the left hemisphere affected in 65.6%.
- Perinatal complications and ipsilateral facial autoimmune conditions (e.g., scleroderma en coup de sabre) were more frequent in RE.
- Early brain or facial autoimmune lesions ipsilateral to the affected hemisphere were observed in 11.9% of RE patients, suggesting a link to laterality.
Conclusions:
- Perinatal complications and facial autoimmune conditions are potential predisposing factors for Rasmussen encephalitis.
- Fever may trigger RE manifestation, and ipsilateral early lesions might explain the disease's unilateral nature.
- Future research should explore genetic or infectious contributors and the role of combined hits in RE pathogenesis.
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