Abstract
Introduction: Primary central nervous system vasculitis (PCNSV) is a rare inflammatory arteriopathy confined to the brain, spinal cord, and leptomeninges. Because of its nonspecific presentation and difficulties in making a positive diagnosis, empiric treatment is often instituted. Case Series: We report a case series of 5 patients who were admitted or transferred to the Johns Hopkins Hospital with a clinical history and magnetic resonance imaging findings suggestive of PCNSV. Four patients had received at least 1 course of immunosuppression with high-dose intravenous (IV) corticosteroids and/or a corticosteroid-sparing agent. Each underwent an extensive workup including 4-vessel cerebral angiography and, in the majority of cases, brain biopsy to evaluate for mimics of PCNSV. In each of the 5 cases, an alternative diagnosis was found. Conclusions: We propose a cautious, multistep approach to the diagnosis of PCNSV, which takes into account more common diagnoses and avoids the pitfalls of empiric treatment.
Original language | English (US) |
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Pages (from-to) | 233-238 |
Number of pages | 6 |
Journal | Neurologist |
Volume | 18 |
Issue number | 4 |
DOIs | |
State | Published - Jul 2012 |
Keywords
- arteriopathy
- cerebral angiogram
- immunosuppression
- stroke
- vasculitis
ASJC Scopus subject areas
- Clinical Neurology