Rapidly progressive primary central nervous system vasculitis.
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.


METHODS
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.


CONCLUSIONS
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.
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
Huston
John
J
Meschia
James F
JF
Giannini
Caterina
C
Miller
Dylan V
DV
Hunder
Gene G
GG
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000000981:diagnostic imaging; D002533:Cerebral Angiography / Q000379:methods; D002544:Cerebral Infarction / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D018810:Magnetic Resonance Angiography / Q000379:methods; D008297:Male; D008875:Middle Aged; D013997:Time Factors; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000503:physiopathology; D055815:Young Adult