Document Detail

Dual pathology in Rasmussen's encephalitis: a study of seven cases and review of the literature.
MedLine Citation:
PMID:  20051019     Owner:  NLM     Status:  MEDLINE    
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.
Hidehiro Takei; Angus Wilfong; Amy Malphrus; Daniel Yoshor; Jill V Hunter; Dawna L Armstrong; Meenakshi B Bhattacharjee
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2009-12-28
Journal Detail:
Title:  Neuropathology : official journal of the Japanese Society of Neuropathology     Volume:  30     ISSN:  1440-1789     ISO Abbreviation:  Neuropathology     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-07-21     Completed Date:  2010-11-12     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9606526     Medline TA:  Neuropathology     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  381-91     Citation Subset:  IM    
Department of Pathology, The Methodist Hospital, Houston, Texas, USA.
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MeSH Terms
Child, Preschool
Encephalitis / complications*,  immunology,  pathology*
HLA-DR Antigens / immunology
Malformations of Cortical Development / complications*,  pathology*
Reg. No./Substance:
0/HLA-DR Antigens

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