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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 nondiagnositc. 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. |
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angiography,cerebral
brain biopsy
brain biopsy,false negative
cerebrovascular accident
cerebrovascular accident,multiple
MRI
MRI,abnormal
serologic testing
thalamus,lesion of
thalamus,lesion of-bilateral
varicella zoster virus
vasculopathy
zoster sine herpete
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