Diagnosing CNS vasculitis: the case against empiric treatment

Neurologist. 2012 Jul;18(4):233-8. doi: 10.1097/NRL.0b013e31825c6d23.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Brain Neoplasms / diagnosis
  • Cerebral Amyloid Angiopathy / diagnosis
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Intracranial Arteriosclerosis / diagnosis
  • Lymphoma, Large B-Cell, Diffuse / diagnosis
  • Male
  • Middle Aged
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / drug therapy*
  • Vasoconstriction


  • Immunosuppressive Agents