Central Nervous System Varicella Zoster Virus Vasculopathy in Human Immunodeficiency Virus Patients

Abstract

Central nervous system varicella zoster virus (CNS-VZV)–related vasculopathy can be severe and fatal. Delayed diagnosis impedes recovery. We reviewed case reports and case series in the literature. Fifty-five patients were identified. Forty patients (85%) had CD4 counts of less than 200 cells/μL. Eight patients (15%) had VZV vasculopathy secondary to CNS immune reconstitution inflammatory syndrome (IRIS). A quarter of the patients never had a history of VZV rash. Eighty-five percent of the reported angiographic studies were abnormal. Cerebrospinal fluid (CSF) pleocytosis was found in 58% (21/36). Eighty-six percent (24/28) of patients had VZV DNA in their CSF, and 71% of patients (12/17) had anti-VZV immunoglobulin G antibodies. Eighty-nine percent of the patients without CNS-IRIS who received acyclovir with corticosteroids had a good recovery. Only 30% of patients who received acyclovir alone had good outcomes. All CNS-IRIS patients except 1 had a good outcome whether they received corticosteroids. The mortality in patients without CNS-IRIS was 55%; it was 12% in CNS-IRIS patients. A history of VZV rash, abnormal vascular imaging, and CSF studies usually make the diagnosis of VZV vasculopathy in human immunodeficiency virus patients, but negative studies do not rule it out in some cases. A combination of acyclovir and corticosteroids seems to improve the outcome in patients without CNS-IRIS.