PMID- 20051019 OWN - NLM STAT- MEDLINE DCOM- 20101112 LR - 20100721 IS - 1440-1789 (Electronic) IS - 0919-6544 (Linking) VI - 30 IP - 4 DP - 2010 Aug TI - Dual pathology in Rasmussen's encephalitis: a study of seven cases and review of the literature. PG - 381-91 LID - 10.1111/j.1440-1789.2009.01079.x [doi] AB - 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. FAU - Takei, Hidehiro AU - Takei H AD - Department of Pathology, The Methodist Hospital, Houston, Texas, USA. takei327@aol.com FAU - Wilfong, Angus AU - Wilfong A FAU - Malphrus, Amy AU - Malphrus A FAU - Yoshor, Daniel AU - Yoshor D FAU - Hunter, Jill V AU - Hunter JV FAU - Armstrong, Dawna L AU - Armstrong DL FAU - Bhattacharjee, Meenakshi B AU - Bhattacharjee MB LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20091228 PL - Australia TA - Neuropathology JT - Neuropathology : official journal of the Japanese Society of Neuropathology JID - 9606526 RN - 0 (HLA-DR Antigens) SB - IM MH - Adolescent MH - Child MH - Child, Preschool MH - Encephalitis/*complications/immunology/*pathology MH - Female MH - HLA-DR Antigens/immunology MH - Humans MH - Immunohistochemistry MH - Male MH - Malformations of Cortical Development/*complications/*pathology EDAT- 2010/01/07 06:00 MHDA- 2010/11/13 06:00 CRDT- 2010/01/07 06:00 PHST- 2010/01/07 06:00 [entrez] PHST- 2010/01/07 06:00 [pubmed] PHST- 2010/11/13 06:00 [medline] AID - NEU1079 [pii] AID - 10.1111/j.1440-1789.2009.01079.x [doi] PST - ppublish SO - Neuropathology. 2010 Aug;30(4):381-91. doi: 10.1111/j.1440-1789.2009.01079.x. Epub 2009 Dec 28.