Dual pathology in Rasmussen's encephalitis: A study of seven cases and review of the literature

Hidehiro Takei, Angus Wilfong, Amy Malphrus, Daniel Yoshor, Jill V. Hunter, Dawna L. Armstrong, Meenakshi B. Bhattacharjee

Research output: Contribution to journalArticlepeer-review

36 Scopus citations

Abstract

Dual pathology has previously been reported in less than 10% of cases of Rasmussen's encephalitis (RE). Given the rarity of RE, it appears unlikely that dual pathology in RE is merely a coincidence. We therefore reviewed all cases of RE experienced in our institution to assess for an additional/associated pathology. A total of seven patients with RE were identified in our archives. Seven children (4 boys and 3 girls, age range: 3-16 years, mean: 9.5 years) with medically refractory epilepsy underwent surgical resection for intractable seizures. The surgical specimens were examined with routine neurohistological techniques, and immunohistochemistry was performed with an extensive panel of antibodies for viruses, lymphocytes, microglia/macrophages, human leukocyte antigen (HLA)-DR, astrocytes, and neurons. Relevant literature was reviewed. Microscopically, all seven cases demonstrated the inflammatory pathology of RE in the cortex and white matter with leptomeningeal and perivascular lymphocytic infiltration, microglial nodules with/without neuronophagia, neuronal loss and gliosis. The HLA-DR antibody was extremely helpful in highlighting the extent of microglial cell proliferation/activation that was not appreciable with standard histology. An unexpected finding in all seven cases was the presence of cortical dysplasia. In our series of seven cases, there was co-occurrence of the inflammatory/destructive pathology of RE with malformative/dysplastic features in cortical architecture in 100% of cases, raising questions about the possible relationships between the two entities. Awareness of the possibility of dual pathology in RE is important for clinical and pathological diagnosis, and may affect the management and outcome of these patients. Immunohistochemistry is very helpful to make a definitive diagnosis of both pathologies.

Original languageEnglish (US)
Pages (from-to)381-391
Number of pages11
JournalNeuropathology
Volume30
Issue number4
DOIs
StatePublished - Aug 1 2010

Keywords

  • central nervous system
  • cortical dysplasia
  • Dual pathology
  • immunohistochemistry
  • Rasmussen's encephalitis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology

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