Vasculopathies caused by varicella zoster virus

Last revised by Derek Smith on 14 Dec 2022

Vasculopathies caused by varicella zoster virus (VZV) represent a group of illnesses involving both small and large CNS arteries caused by a inflammatory process involving the vascular media and endothelium. It usually occurs in immunocompromised individuals due viral reactivation and spread through the vessel wall. As a consequence, it may cause ischemic infarction, aneurysm, cerebral and subarachnoid hemorrhage, and arterial ectasia 3.

For a complementary discussion on CNS involvement, please refer on varicella zoster virus encephalitis.

In children, varicella zoster virus vasculopathy is responsible for ~ 30% of all arterial ischemic strokes 2 and often occurs weeks to months after zoster or varicella infection. In adults, it is more common in immunocompromised individuals 3.

A variety of neurological symptoms could be present related to a stroke or transient ischemic attacks.

Varicella zoster virus vasculopathy should be suspected when after patients present with a transient ischemic attack or stroke corroborated by MRI abnormalities following a recent of varicella or zoster infection 3.

CSF analysis demonstrates modest pleocytosis, predominantly mononuclear. Protein is commonly increased and glucose concentration is normal 4. The presence of anti-VZV IgG antibody or VZV DNA usually confirms the diagnosis.

Varicella zoster is an alpha herpesvirus found exclusively in humans 1. Primary VZV infection, which usually occurs in children, results in chickenpox (varicella) and, after the acute illness resolves, the virus remains latent in ganglionic neurons. In immunocompromised or elderly patients, or rarely in younger healthy patients too, the virus may reactivate. 

Different theories seek to explain how the virus involves the intracranial vessels, such as hematogenous seeding or spreading via the sympathetic nervous system. The infection causes a inflammatory reaction and a functional damage to the vascular endothelium may resulting in thrombosis and promoting subendothelial proliferation of smooth muscle cells, fibroblasts, and collagen, leading to areas of stenosis and occlusion 1,3.

Imaging can show cortical and deep abnormalities involving both the grey and white matter, particularly the grey-white matter junctions, mainly demonstrated as ischemic lesions 3. Varicella zoster virus vasculopathy can also rarely affect the spinal cord causing infarction.

Subarachnoid and intracerebral hemorrhage can also occur. 

Contrast enhancement could be present in some lesions secondary to breakdown of the blood–brain barrier.

About 70% of patients show vascular abnormalities on angiographic studies (CTA/MRA/DSA) 4. The main feature is of segmental arterial constrictions often followed by post stenotic dilatation, creating a beading pattern. Occlusions could also be shown. This is a nonspecific finding.

Complications of varicella zoster virus vasculopathy can include cerebral aneurysm and dissection.

It is important to note that a negative angiogram does not exclude the diagnosis, as the disease in small arteries is not detected as readily as in large arteries 3

Patients are usually treated with antiviral medications, such as intravenous acyclovir. 

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