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 Volume 70, Number 11, March 11, 2008
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NEUROLOGY 2008;70:853-860
© 2008 American Academy of Neurology

The varicella zoster virus vasculopathies

Clinical, CSF, imaging, and virologic features

M. A. Nagel, MD, R. J. Cohrs, PhD, R. Mahalingam, PhD, M. C. Wellish, BS, B. Forghani, PhD, A. Schiller, MD, J. E. Safdieh, MD, E. Kamenkovich, BA, L. W. Ostrow, MD, M. Levy, MD, PhD, B. Greenberg, MD, A. N. Russman, DO, I. Katzan, MD, C. J. Gardner, DO, M. Häusler, MD, R. Nau, MD, T. Saraya, MD, H. Wada, MD, H. Goto, MD, M. de Martino, MD, M. Ueno, MD, W. D. Brown, MD, C. Terborg, MD and D. H. Gilden, MD

From the Departments of Neurology (M.A.N., R.J.C., R.M., M.C.W., 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.S.), University Hospital, Zurich, Switzerland; Department of Neurology and Neuroscience (J.E.S., E.K.), Weill Medical College of Cornell University, New York, NY; Department of Neurology (L.W.O., M.L., B.G.), Johns Hopkins Hospital, Baltimore, MD; Department of Neurology (A.N.R.), Henry Ford Hospital, Detroit, MI; Department of Neurology (I.K.), Cleveland Clinic Foundation, OH; Department of Neurology (C.J.G.), Thomas Jefferson University School of Medicine, Philadelphia, PA; Department of Pediatrics (M.H.), University Hospital RWTH Aachen, Germany; Department of Neurology (R.N.), University of Gottingen, Germany; Department of Respiratory Medicine (T.S., H.W., H.G.), Kyorin University School of Medicine, Tokyo, Japan; Department of Pediatrics (M.d.M.), University of Florence, Italy; Division of Child Neurology (M.U.), Institute of Neurological Sciences, Tottori University, Japan; Department of Neurology (W.D.B.), Children's National Medical Center, Washington, DC; and Department of Neurology (C.T.), Friedrich-Schiller-University, Jena, Germany.

Address correspondence and reprint requests to Dr Gilden, Department of Neurology, Mail Stop B182, University of Colorado Health Sciences Center, 4200 E. 9th Ave., Denver, CO 80262 don.gilden{at}uchsc.edu

Background: Varicella zoster virus (VZV) vasculopathy produces stroke secondary to viral infection of cerebral arteries. Not all patients have rash before cerebral ischemia or stroke. Furthermore, other vasculitides produce similar clinical features and comparable imaging, angiographic, and CSF abnormalities.

Methods: We review our 23 published cases and 7 unpublished cases of VZV vasculopathy. All CSFs were tested for VZV DNA by PCR and anti-VZV IgG antibody and were positive for either or both.

Results: Among 30 patients, rash occurred in 19 (63%), CSF pleocytosis in 20 (67%), and imaging abnormalities in 29 (97%). Angiography in 23 patients revealed abnormalities in 16 (70%). Large and small arteries were involved in 15 (50%), small arteries in 11 (37%), and large arteries in only 4 (13%) of 30 patients. Average time from rash to neurologic symptoms and signs was 4.1 months, and from neurologic symptoms and signs to CSF virologic analysis was 4.2 months. CSF of 9 (30%) patients contained VZV DNA while 28 (93%) had anti-VZV IgG antibody in CSF; in each of these patients, reduced serum/CSF ratio of VZV IgG confirmed intrathecal synthesis.

Conclusions: Rash or CSF pleocytosis is not required to diagnose varicella zoster virus (VZV) vasculopathy, whereas MRI/CT abnormalities are seen in almost all patients. Most patients had mixed large and small artery involvement. Detection of anti-VZV IgG antibody in CSF was a more sensitive indicator of VZV vasculopathy than detection of VZV DNA (p < 0.001). Determination of optimal antiviral treatment and benefit of concurrent steroid therapy awaits studies with larger case numbers.

GLOSSARY: EIA = enzyme immunoabsorbent assay; VZV = varicella zoster virus.

Received August 20, 2007. Accepted in final form October 4, 2007.

Supplemental data at www.neurology.org

Supported by grants AG06127, NS32623, and NS07321 to Dr. Gilden from the NIH.

Disclosure: The authors report no conflicts of interest.




Correspondence:

Read all Correspondence

The varicella zoster virus vasculopathies: Clinical, CSF, imaging, and virologic features
Mitchell SV Elkind
Neurology Online, 4 Jun 2008 [Full text]
Reply from the authors
Maria A. Nagel, et al.
Neurology Online, 4 Jun 2008 [Full text]

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