Document Detail

Nonvasculitic autoimmune inflammatory meningoencephalitis.
MedLine Citation:
PMID:  15139593     Owner:  NLM     Status:  MEDLINE    
Caselli and colleagues described five cases with encephalopathy, progressive cognitive decline, ataxia, abnormal CSF studies and steroid responsiveness, and proposed the term non-vasculitic autoimmune inflammatory meningoencephalitis (NAIM). Many of these cases had brain biopsy showing mild leptomeningeal perivascular lymphocytic inflammation, however, none of the cases had a post-mortem. Nonvasculitic autoimmune mediated meningoencephalitis has been described in patients with Sjogren's syndrome, systemic lupus erythematosus and, more recently, with Hashimoto's disease. The present study is the first post-mortem report of a case with a clinical diagnosis of NAIM. Neuropathological examination revealed a panencephalitis with intact vessel walls. T and B immunostaining showed a mixture of T and B cells. The findings were not consistent with other reported findings in collagen vascular diseases including Sjogren's syndrome, CNS vasculitis or Hashimoto's encephalopathy. There was no evidence of neoplasia, bacteria, acid-fast bacilli, fungi or atypical infectious agents. This is the first post-mortem report of a case with a clinical diagnosis of NAIM and demonstrates a panencephalitis without evidence of a vasculitis. The pathology seems unique, however, Sjogren and Hashimoto's encephalopathy might be variants of NAIM.
Keith A Josephs; Frank A Rubino; Dennis W Dickson
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neuropathology : official journal of the Japanese Society of Neuropathology     Volume:  24     ISSN:  0919-6544     ISO Abbreviation:  Neuropathology     Publication Date:  2004 Jun 
Date Detail:
Created Date:  2004-05-13     Completed Date:  2004-06-17     Revised Date:  2008-03-10    
Medline Journal Info:
Nlm Unique ID:  9606526     Medline TA:  Neuropathology     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  149-52     Citation Subset:  IM    
Department of Neurology, Mayo Clinic, Jacksonville, Florida 32224, USA.
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MeSH Terms
Autoimmune Diseases / pathology*
Brain / blood supply,  pathology*
Diagnosis, Differential
Fatal Outcome
Inflammation / pathology*
Meningoencephalitis / pathology*

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