Primary central nervous system vasculitis: analysis of 101 patients.
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.


CONCLUSIONS
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.
Salvarani
Carlo
C
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Brown
Robert D
RD
Calamia
Kenneth T
KT
Christianson
Teresa J H
TJ
Weigand
Stephen D
SD
Miller
Dylan V
DV
Giannini
Caterina
C
Meschia
James F
JF
Huston
John
J
Hunder
Gene G
GG
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002986:Clinical Trials as Topic / Q000379:methods; D015331:Cohort Studies; D003937:Diagnosis, Differential; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*
D005938:Glucocorticoids