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From the Departments of Neurology (M.A.N., R.M., M.C.W., R.J.C., D.H.G.) and Microbiology (D.H.G), University of Colorado Health Sciences Center, Denver; Viral and Rickettsial Disease Laboratory (B.F.), Division of Communicable Disease Control, California Department of Health Services, Richmond; Department of Neurology (A.N.R., I.K., R.L.), Cleveland Clinic Foundation, OH; and Department of Neurology (C.J.G.), Thomas Jefferson University School of Medicine, Philadelphia, PA.
Address correspondence and reprint requests to Dr Gilden at Department of Neurology, Mail Stop B182, University of Colorado Health Sciences Center, 4200 E. 9 Ave., Denver, CO 80262; e-mail: don.gilden{at}uchsc.edu
Background: Factors that may obscure the diagnosis of varicella zoster virus (VZV) vasculopathy include the absence of rash before TIAs or stroke as well as similar clinical features and imaging, angiographic, and CSF abnormalities to those of other vasculopathies. Diagnosis relies on virologic confirmation that detects VZV DNA, anti-VZV IgG antibody, or both in the CSF.
Methods: We reviewed our current 14 cases of patients diagnosed with VZV vasculopathy based on combined clinical, imaging, angiographic, or CSF abnormalities. All CSFs must have been tested for VZV DNA by PCR and for anti-VZV IgG antibody by enzyme immunoassay and found to be positive for either or both. Of the 14 subjects, 8 had a history of recent zoster, whereas 6 had no history of zoster rash before developing vasculopathy.
Results: All 14 subjects (100%) had anti-VZV IgG antibody in their CSF, whereas only 4 (28%) had VZV DNA. The detection of anti-VZV IgG antibody in CSF was a more sensitive indicator of VZV vasculopathy than detection of VZV DNA (p < 0.001).
Conclusions: In varicella zoster virus (VZV) vasculopathy, the diagnostic value of detecting anti-VZV IgG antibody in CSF is greater than that of detecting VZV DNA. Although a positive PCR for VZV DNA in CSF is helpful, a negative PCR does not exclude the diagnosis of VZV vasculopathy. Only when the CSF is negative for both VZV DNA and anti-VZV IgG antibody can the diagnosis of VZV vasculopathy be excluded.
This article was previously published in electronic format as an Expedited E-Pub on February 7, 2007, at www.neurology.org.
Supported in part by grants AG06127 and NS32623 to Dr. Gilden from the NIH.
Disclosure: The authors report no conflicts of interest.
Received September 8, 2006. Accepted in final form January 3, 2007.
This article has been cited by other articles:
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  M. S.V. Elkind, M. A. Nagel, and D. H. Gilden The varicella zoster virus vasculopathies: Clinical, CSF, imaging, and virologic features Neurology, March 17, 2009; 72(11): 1028 - 1030. [Full Text] [PDF]
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 K. P. J. Braun, M. M. M. Bulder, S. Chabrier, F. J. Kirkham, C. S. P. Uiterwaal, M. Tardieu, and G. Sebire The course and outcome of unilateral intracranial arteriopathy in 79 children with ischaemic stroke Brain, February 1, 2009; 132(2): 544 - 557. [Abstract] [Full Text] [PDF]
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M. A. Nagel, R. J. Cohrs, R. Mahalingam, M. C. Wellish, B. Forghani, A. Schiller, J. E. Safdieh, E. Kamenkovich, L. W. Ostrow, M. Levy, et al. The varicella zoster virus vasculopathies: Clinical, CSF, imaging, and virologic features Neurology, March 11, 2008; 70(11): 853 - 860. [Abstract] [Full Text] [PDF]
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H. T. Orme, A. G. Smith, M. A. Nagel, R. J. Bert, T. S. Mickelson, and D. H. Gilden VZV SPINAL CORD INFARCTION IDENTIFIED BY DIFFUSION-WEIGHTED MRI (DWI) Neurology, July 24, 2007; 69(4): 398 - 400. [Full Text] [PDF]
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 D. H. Gilden and M. Nagel Stroke Caused by Human Immunodeficiency Virus-Associated Vasculopathy? Arch Neurol, May 1, 2007; 64(5): 763 - 763. [Full Text] [PDF]
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