Multifocal varicella-zoster virus vasculopathy without rash

Arch Neurol. 2003 Nov;60(11):1607-9. doi: 10.1001/archneur.60.11.1607.


A 51-year-old woman with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) developed stepwise progressive focal neurological deficits without zoster rash. Multifocal ischemic infarcts were seen on magnetic resonance imaging, and cerebral angiography revealed focal stenosis of arteries affecting the intracranial circulation. A brain biopsy was nondiagnostic. Virological etiology of the disease was verified by the detection of varicella-zoster virus antibody in cerebrospinal fluid and by reduced serum-cerebrospinal fluid varicella-zoster virus IgG ratios (compared with normally high ratios of total IgG and albumin). Treatment with intravenous acyclovir stabilized but did not significantly improve her neurological deficits.

Publication types

  • Case Reports
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Brain Infarction / virology*
  • CREST Syndrome / complications
  • Cerebral Arterial Diseases / pathology
  • Cerebral Arterial Diseases / virology*
  • Cerebrovascular Circulation
  • Exanthema / pathology
  • Female
  • Herpes Zoster / cerebrospinal fluid
  • Herpes Zoster / complications
  • Herpes Zoster / pathology*
  • Herpesvirus 3, Human / immunology
  • Herpesvirus 3, Human / isolation & purification
  • Humans
  • Immunoglobulin G / cerebrospinal fluid
  • Immunoglobulin M / cerebrospinal fluid
  • Magnetic Resonance Angiography
  • Middle Aged


  • Immunoglobulin G
  • Immunoglobulin M