Document Detail

Primary central nervous system vasculitis: analysis of 101 patients.
MedLine Citation:
PMID:  17924545     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: To analyze the clinical findings, response to therapy, outcome, and incidence of primary central nervous system vasculitis (PCNSV) in a large cohort from a single center. METHODS: We retrospectively studied 101 patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 21-year period. This was a collaborative study by five departments at a large multispecialty clinic. Clinical findings and outcomes were compared among patients categorized by method of diagnosis, response to therapy, survival, and degree of disability. An annual incidence rate was calculated. RESULTS: Seventy patients were diagnosed by angiography and 31 by central nervous system biopsy. Three histological patterns were observed during biopsy. Although most patients responded to therapy, an increased mortality rate was observed. Relapses occurred in one fourth of patients. Mortality rate and disability at last follow-up were greater in those who presented with a focal neurological deficit, cognitive impairment, cerebral infarctions, and angiographic large-vessel involvement but were lower in those with prominent gadolinium-enhanced lesions when evaluated by magnetic resonance imaging. The annual incidence rate of PCNSV was 2.4 cases per 1,000,000 person-years. INTERPRETATION: PCNSV is a rare disease that may result in serious neurological outcomes or death. Angiography and brain biopsy may complement each other when determining the diagnosis. Early recognition and treatment may reduce poor outcomes. PCNSV is a variable syndrome that appears to consist of several subsets of heterogeneous diseases.
Carlo Salvarani; Robert D Brown; Kenneth T Calamia; Teresa J H Christianson; Stephen D Weigand; Dylan V Miller; Caterina Giannini; James F Meschia; John Huston; Gene G Hunder
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Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Annals of neurology     Volume:  62     ISSN:  1531-8249     ISO Abbreviation:  Ann. Neurol.     Publication Date:  2007 Nov 
Date Detail:
Created Date:  2007-12-06     Completed Date:  2007-12-27     Revised Date:  2008-09-19    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  442-51     Citation Subset:  IM    
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
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MeSH Terms
Aged, 80 and over
Clinical Trials as Topic / methods
Cohort Studies
Diagnosis, Differential
Glucocorticoids / therapeutic use
Middle Aged
Nervous System Diseases / diagnosis,  mortality,  therapy
Retrospective Studies
Survival Rate / trends
Vasculitis, Central Nervous System / diagnosis*,  mortality,  therapy*
Reg. No./Substance:
Comment In:
Ann Neurol. 2008 Aug;64(2):228; author reply 229   [PMID:  18350577 ]
Ann Neurol. 2008 Aug;64(2):228; author reply 229   [PMID:  18350582 ]
Ann Neurol. 2007 Nov;62(5):430-2   [PMID:  18041087 ]

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