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Primary central nervous system vasculitis presenting with intracranial hemorrhage
Arthritis & Rheumatism, 09/02/2011
Salvarani C et al. – The findings suggest that intracranial hemorrhage (IH) may not be an infrequent occurrence in early primary central nervous system vasculitis (PCNSV). Necrotizing vasculitis may be a predominant histopathological pattern.
Methods- In the present study the authors use the updated cohort of 131 consecutive patients with PCNSV seen over the 25 year period of 1983 to 2007 at Mayo Clinic, Rochester, MN.
- The diagnosis of PCNSV was based on brain/spinal cord biopsy or cerebral angiography, or both.
- IH at presentation was defined as the presence on brain computed tomography or magnetic resonance imaging of intracerebral or subarachnoid hemorrhage within 3 months of the date of PCNSV diagnosis.
- Clinical, laboratory, radiological, and pathological findings, therapy and outcomes of patients presenting with IH were assessed and compared with those without.
- 16 patients (12.2%) had evidence of IH at or near the diagnosis.
- Twelve patients had intracerebral and 4 subarachnoid hemorrhage.
- 12 patients were diagnosed by angiography and 4 by CNS biopsy.
- Compared with the 115 patients without, the 16 patients presenting with IH more frequently were females, and less frequently had altered cognition, a persistent neurological deficit or stroke at presentation, nor MRI evidence of cerebral infarctions, and less frequently required therapy at last follow–up.
- A necrotizing histopathological pattern of vasculitis was observed in 3 of 4 (75%) of the patients with positive biopsies.