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

Human Pathology
|March 10, 2012
PubMed

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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