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.
Background: Varicella zoster vasculopathy of the central nervous system (CNS-VZV) is rare. This topic has not been systemically analyzed in HIV patients. Method: We reviewed case reports/series in the literature from Pubmed using search term: varicella zoster, vasculopathy, stroke and HIV. Results: Forty-eight patients were identified. Eight patients (17%) had VZV vasculopathy after started on highly active antiretroviral therapy (HAART) consistent with central nervous system immune reconstitution inflammatory syndrome (CNS-IRIS). The average time from starting HAART to a neurological deficit was approximately 3 weeks. Rash occurred in ∼67%, with a mean 12 month interval from rash to CNS symptom onset though in 18 (64%) it occurred just prior to symptom onset. Patients had CNS lesions in the following distributions: posterior circulation, n=22 (46%); anterior circulation, n=13 (27%); and spinal, n=10 (21%). Specific abnormalities included stenosis/occlusion in 15 (83%), saccular aneurysm in 4 (22%), fusiform aneurysm in 2 (18%), and arterial wall enhancement in 2 (11%). The vasculopathy involved large vessels in 21/47 patients (45%), small arteries in 19 (40%), and both large and small arteries in 7 (15%). CSF pleocytosis was found in 30/36 patients (83%) (mean: 101), and CSF protein was elevated in 25/34 patients (74%). CSF immunological analysis was available in 29 patients. Twenty-five patient (89%) had either CSF positive for VZV DNA by PCR or anti-VZV IgG. 17/23 (74%) patients had VZV DNA positive in their CSF, and 13/21 (62%) had anti-VZV IgG antibody. Of the 35 patients with available treatment data, acyclovir was used as initial treatment in 27 (77%) with good recovery in 23 (85%). Thirteen cases received acyclovir with corticosteroids, and 11 had a good recovery (84%). Fourteen cases received acyclovir alone; 8 had a good recovery (57%). The mortality rate was 49%. VZV vasculopathy secondary from CNS-IRIS had better prognosis with a mortality rate 13%. Conclusion: Prompt treatment with acyclovir while waiting for CSF immunology is critical when CNS-VZV vasculopathy is a possibility. Rash, abnormal angiography or CSF pleocytosis give clues for diagnosis but not rule it out if negative. Steroid seems to improve the outcome of these patients.
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