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Multifocal varicella-zoster virus vasculopathy without rash
Andrew N Russman1, Richard J Lederman, Leonard H Calabrese
1Department of Neurology, The Cleveland Clinic Foundation, Cleveland, OH 44195, USA. russmaa@ccf.org
Archives of Neurology
|November 19, 2003
Summary
Varicella-zoster virus (VZV) can cause progressive neurological deficits and intracranial artery stenosis in CREST syndrome patients. Early antiviral treatment may stabilize, but not fully reverse, these severe neurological outcomes.
Area of Science:
- Neurology
- Virology
- Rheumatology
Background:
- CREST syndrome, a variant of systemic sclerosis, presents with diverse systemic manifestations.
- Neurological complications in CREST syndrome are rare but can be severe.
Observation:
- A 51-year-old woman with CREST syndrome developed progressive focal neurological deficits.
- Imaging revealed multifocal ischemic infarcts and intracranial artery stenosis.
- No zoster rash was present.
Findings:
- Cerebrospinal fluid analysis confirmed varicella-zoster virus (VZV) infection.
- Reduced serum-cerebrospinal fluid VZV IgG ratios indicated intrathecal VZV antibody production.
- Brain biopsy was nondiagnostic.
Implications:
- This case highlights VZV as a potential cause of cerebrovascular disease in CREST syndrome.
- Intravenous acyclovir stabilized neurological deficits, suggesting a role for antiviral therapy.
- Further research is needed to understand the VZV-CREST syndrome association and optimize treatment strategies.