Presentation
History of fever for 5 days, followed by altered sensorium. No rash.
Patient Data
There are numerous small lesions consistent with acute infarcts predominantly involving the grey-white matter junction and also the cortex and subcortical white matter. Few similar lesions are also seen in the anterior aspect of the midbrain.
Generalized leptomeningeal enhancement is seen consistent with meningitis.
Few small lesions are seen along the anterior aspect of the lower dorsal cord on the right side, probably representing spinal cord infarction.
Case Discussion
Varicella zoster vasculopathy is an infection of the arteries of the brain caused by reactivation of the varicella zoster virus, lying dormant in the dorsal root ganglion or cranial nerves. The virus reaches the arteries via hematogenous seeding or transaxonal spread.
Clinically, it usually presents as a stroke with a history of recent zoster infection. Altered sensorium, cognitive impairment, and acute onset myelopathy remain rarer presentations. A pathognomonic rash may not always be present.
Ischemic lesions are more prevalent than hemorrhagic lesions. MRI usually demonstrates infarcts more frequently at the grey-white matter junction than in the grey or white matter. Lesions in the brainstem along the trigeminal pathway and leptomeningeal enhancement can occur. Ischemic lesions of the spinal cord are rare. Angiography often demonstrates segmental narrowing of large vessels, while smaller vessel involvement remains occult.
Presence of VZV IgG antibody or viral DNA in CSF are diagnostic.
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