Diagnosing CNS vasculitis: The case against empiric treatment

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12 Scopus citations


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 languageEnglish (US)
Pages (from-to)233-238
Number of pages6
Issue number4
StatePublished - Jul 1 2012


  • arteriopathy
  • cerebral angiogram
  • immunosuppression
  • stroke
  • vasculitis

ASJC Scopus subject areas

  • Clinical Neurology


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