Varicella zoster virus, a cause of waxing and waning vasculitis

Abstract

A 73-year-old man developed an ill-defined fatal vasculitis involving the central nervous system. The case report was published as a clinicopathologic exercise in February 1995 in The New England Journal of Medicine. We restudied the pathologic material and found both varicella zoster virus (VZV) DNA and VZV-specific antigen, but not herpes simplex virus (HSV) or cytomegalovirus (CMV) DNA or HSV- or CMV-specific antigen, in three of the five cerebral arteries examined. The inflammatory response, disruption of the internal elastic lamina, and detection of viral antigen were patchy from one artery to another, as well as within a given artery. A search for VZV should be conducted in cases of vasculitis when both the central and peripheral nervous systems are involved, when focal narrowing is present in large arteries, when brain imaging reveals infarction in gray and white matter, both deep and superficial, and when white matter is disproportionally involved. Zosteriform rash is not required for diagnosis.

The varicella zoster virus vasculopathies

“…Postmortem virologic analysis reveals VZV antigen ( figure 2D) in cerebral vessels. 19 Thus, all patients are typically treated with IV acyclovir based on Category 3 evidence (opinions of respected authorities based on clinical experience, descriptive studies, or reports of expert committees). Herein, of all patients treated with acyclovir alone, a total of 66% improved or stabilized compared to 75% who improved or stabilized when treated with both acyclovir and steroids.…”

Varicella Zoster Virus: A Common Cause of Stroke in Children and Adults

“…Again, VZV vasculopathy in adults is often protracted. Cases that lasted one year before death (17) or that were confirmed virologically after 6 or 12 months of waxing and waning disease are welldocumented, including a favorable response to antiviral treatment (18,19). CT or MRI scanning often reveals a single or multiple areas of ischemia/infarction in the distribution of large or small arteries and often both.…”

Central Nervous System Vasculopathy In Varicella Zoster Virus Infection

“…VZV vasculopathies point to active viral replication [11] which is reflected by the presence of multinucleated giant cells, Cowdry A inclusion bodies and viral particles, and VZV antigen and DNA in the blood vessels [12]. Several studies have demonstrated the presence of afferent fibres from certain cranial nerve ganglia to both intra-and extra-cranial blood vessels, which can provide an anatomical pathway for the trans-axonal spread of virus [12] to the cerebral vasculature.…”