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.