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Rapidly progressive primary central nervous system vasculitis.
MedLine Citation:
PMID:  20959356     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Objective. To describe a subset of cases in a large cohort of patients with primary CNS vasculitis (PCNSV) who appear to have a rapidly progressive clinical course. Method. In the present study, we use our updated cohort of 131 consecutive patients with PCNSV seen over the 25-year period of 1983-2007 at Mayo Clinic, Rochester, MN, USA. The diagnosis of PCNSV was based on brain/spinal cord biopsy or cerebral angiography. The modified Rankin scale was used to identify rapidly progressive disease and included patients with Rankin scores indicating severe disability or death at diagnosis or within 6 months after the diagnosis. We compared patients with rapidly progressive disease to those without. Results. Compared with the 120 patients without rapidly progressive vasculitis, the 11 patients with rapidly progressive vasculitis more frequently had paraparesis/quadriparesis at presentation, angiographic presence of bilateral, large-vessel vasculitis and MRI evidence of cerebral infarctions; those infarctions were more frequently multiple and bilateral, and more frequently involved both the cortex and subcortical regions on initial MRI. Granulomatous and/or necrotizing histopathological patterns of vasculitis were observed in patients with positive biopsies. Conclusion. Rapidly progressive PCNSV appears to form a subset of PCNSV at the worst end of the clinical spectrum of this vasculitis, characterized by bilateral, multiple, large cerebral vessel lesions and multiple CNS infarctions.
Authors:
Carlo Salvarani; Robert D Brown; Kenneth T Calamia; Teresa J H Christianson; John Huston; James F Meschia; Caterina Giannini; Dylan V Miller; Gene G Hunder
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Publication Detail:
Type:  Journal Article     Date:  2010-10-19
Journal Detail:
Title:  Rheumatology (Oxford, England)     Volume:  50     ISSN:  1462-0332     ISO Abbreviation:  Rheumatology (Oxford)     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-01-18     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883501     Medline TA:  Rheumatology (Oxford)     Country:  England    
Other Details:
Languages:  eng     Pagination:  349-58     Citation Subset:  AIM; IM    
Affiliation:
Jr, Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. brown@mayo.edu.
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