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Multifocal Varicella-Zoster Virus Vasculopathy Without Rash
Andrew N. Russman, DO;
Richard J. Lederman, MD, PhD;
Leonard H. Calabrese, DO;
Peter J. Embi, MD;
Bagher Forghani, PhD;
Donald H. Gilden, MD
Arch Neurol. 2003;60:1607-1609.
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.
From the Departments of Neurology (Drs Russman and Lederman) and Rheumatic and Immunologic Diseases (Drs Calabrese and Embi), The Cleveland Clinic Foundation, Cleveland, Ohio; Viral and Rickettsial Disease Laboratory, Division of Communicable Disease Control, California Department of Health Services, Richmond (Dr Forghani); and the Departments of Neurology and Microbiology, University of Colorado Health Sciences Center, Denver (Dr Gilden).
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