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Intimal thickening of meningeal arteries after serial corticectomies for Rasmussen encephalitis
Aaron S Wagner1, Nicole S Yin, Spencer Tung
1Department of Pathology and Laboratory Medicine (Neuropathology), Brain Research Institute, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA. brainpathology@gmail.com
Abstract:
Rasmussen encephalitis is a rare cause of intractable epilepsy in children. Between 2008 and 2010, 4 patients had second cortical resections performed after a primary corticectomy for Rasmussen encephalitis. In each case, we observed some degree of vessel wall change in leptomeningeal arteries, consisting of moderate to moderately severe intimal hyperplasia. The intervals between original resection and second operation ranged from 8 months to 10 years. Ages of the patients ranged from 9 to 12 years at their first resection and from 10 to 19 years at the time of revision. Four other Rasmussen encephalitis cases operated upon in the years 2006 to 2010 and 2 surgical revisions for severe cortical dysplasia, 1 for mild cortical dysplasia and 1 for recurrent dysembryoplastic neuroepithelial tumor, did not show significant vascular abnormalities (with surgical intervals of 10 months to 16 years). Leptomeningeal intimal hyperplasia appears to develop in the interval between repeated cortical resections for Rasmussen encephalitis, an inflammatory disorder. The pathogenesis of this vascular change may be related to meningeal inflammation in Rasmussen encephalitis. This finding in children undergoing surgical resection for Rasmussen encephalitis may itself lead to "secondary" ischemic change that contributes to worsening of epilepsy.
Insights
Rasmussen encephalitis surgery may cause vessel changes. Repeated resections for this pediatric epilepsy can lead to intimal hyperplasia, potentially worsening seizures.
Area of Science:
- Neurology
- Pediatric Neurology
- Neurosurgery
Background:
- Rasmussen encephalitis is a rare, severe neurological disorder causing intractable epilepsy in children.
- Surgical resection is a treatment option for medically refractory epilepsy due to Rasmussen encephalitis.
Purpose of the Study:
- To investigate vascular changes in leptomeningeal arteries following repeat cortical resections in children with Rasmussen encephalitis.
- To determine if these vascular changes are specific to Rasmussen encephalitis compared to other pediatric epilepsy surgeries.
Main Methods:
- Retrospective review of 4 pediatric patients with Rasmussen encephalitis who underwent second cortical resections between 2008-2010.
- Histopathological examination of leptomeningeal arteries for intimal hyperplasia.
- Comparison with 6 control cases involving epilepsy surgery for cortical dysplasia or dysembryoplastic neuroepithelial tumor.
Main Results:
- All 4 Rasmussen encephalitis patients showed moderate to severe intimal hyperplasia in leptomeningeal arteries after repeat resections.
- Vascular abnormalities were not observed in control epilepsy surgery cases, even with long surgical intervals.
- Intimal hyperplasia appears to develop between serial resections for Rasmussen encephalitis.
Conclusions:
- Leptomeningeal intimal hyperplasia is a potential consequence of repeated cortical resections for Rasmussen encephalitis.
- This vascular change may be linked to the underlying meningeal inflammation characteristic of Rasmussen encephalitis.
- Secondary ischemic changes resulting from intimal hyperplasia could contribute to worsening epilepsy in these patients.
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