WEDNESDAY, Oct. 31 (HealthDay News) -- Although rare, primary central nervous system vasculitis (PCNSV) is a heterogeneous disease that can result in serious neurologic outcomes or death, researchers report in the Annals of Neurology Early View, published online Oct. 9.
Carlo Salvarani, M.D., and colleagues from the Mayo Clinic in Rochester, Minn., and Jacksonville, Fla., analyzed clinical findings and outcomes in 101 patients with PCNSV seen during a 21-year period.
The researchers found that 31 patients were diagnosed by central nervous system biopsy and the remaining 70 were diagnosed by angiography, with three histologic patterns observed. One-quarter of the patients relapsed and mortality was higher even though most patients responded to treatment. Mortality rates and disability varied with clinical findings. The annual incidence of the disease was 2.4 cases per million person-years.
"In conclusion, our findings confirm that PCNSV is a rare disease associated with increased morbidity and mortality," Salvarani and colleagues write. "Careful delineation of findings on angiography and biopsy will ensure an accurate diagnosis of PCNSV, as shown by the similar clinical findings and outcomes of patients assessed by either method in our study."
Abstract
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