Diagnosing CNS vasculitis: the case against empiric treatment.
BACKGROUND
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.


METHODS
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.
Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.
Elisabeth B
EB
Marsh
Steven R
SR
Zeiler
Michael
M
Levy
Rafael H
RH
Llinas
Victor C
VC
Urrutia
D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000175:diagnosis; D016657:Cerebral Amyloid Angiopathy / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D002537:Intracranial Arteriosclerosis / Q000175:diagnosis; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000188:drug therapy*; D014661:Vasoconstriction
D007166:Immunosuppressive Agents