Background: Infections are a well-known cause of cerebral vasculopathy and vasculitis. We aimed to analyze the frequency of intracranial vasculopathy attributable to infection, the spectrum of causative microorganisms, imaging, and cerebrospinal fluid (CSF) characteristics as well as clinical course and outcome.
Methods: We used our institution's medical record system to identify all patients diagnosed with nonatherosclerotic central nervous system vasculopathy from January 1, 1999 through February 28, 2009. We reviewed their clinical charts, imaging data, and results of CSF studies.
Results: Twenty-five adult patients with nonatherosclerotic cerebral vasculopathy of large- and medium-sized intracranial vessels were identified. Eight patients had vasculopathy attributable to infection (32%). The underlying pathologies were acute bacterial meningitis (n = 4), varicella zoster virus (VZV) infection (n = 2), borreliosis (n = 1), and syphilis (n = 1). In six patients, magnetic resonance angiography was performed and showed vasculopathic changes in all patients examined (100%). In both patients with VZV-associated vasculopathy, the arterial wall enhanced on magnetic resonance imaging. The CSF examination of the patients with infectious vasculopathy showed a significantly higher white blood cell count. The outcome of the infectious cohort was unfavorable with one death, two patients with locked-in syndrome, and five patients discharged from intensive care with severe neurological deficits.
Conclusion: In this cohort, one-third of all cases of nonatherosclerotic vasculopathy were due to infectious vasculopathy of large and medium intracranial vessels.