The challenging patient with varicella-zoster virus disease

Abstract

Varicella-zoster virus (VZV) reactivation from latently infected ganglia causes multiple neurologic diseases. The most common is herpes zoster, which is frequently complicated by postherpetic neuralgia, meningoencephalitis, and vasculopathy, including VZV temporal arteritis, myelopathy, and retinal necrosis. All of these disorders can develop without rash. Importantly, VZV vasculopathy is emerging as a significant cause of TIAs and stroke. In particular, a subset of patients who present with symptoms and signs of giant cell arteritis (GCA), but whose temporal artery biopsies are GCA-negative, have multifocal VZV vasculopathy with temporal artery infection. Herein we focus on the specific diagnostic and therapeutic challenges that clinical neurologists encounter in diseases caused by VZV, discuss guidelines for zoster vaccine, and highlight molecular features of VZV latency with a focus on preventing the serious neurologic and ocular complications of VZV reactivation.

Figure 1. Characteristic varicella-zoster virus and herpes simplex virus–1 rash

Varicella-zoster virus reactivation is manifest by dermatomal skin lesions (left), while herpes simplex virus reactivation is manifest by mucosal or patchy skin lesions (right). Both are vesicular on an erythematous base.

Figure 2. With a decline in varicella-zoster virus–specific cell-mediated immunity, virus reactivates from ganglionic neurons and spreads peripherally or centrally to cause disease

Peripheral spread to the skin causes herpes zoster. Central spread to the brain causes meningoencephalitis, while central spread to intracranial and extracranial arteries produces vasculopathy and varicella-zoster virus temporal arteritis, respectively. Central spread to the spinal cord causes myelopathy, while spread to spinal cord arteries causes spinal cord infarction.

Figure 3. Varicella-zoster virus vasculopathy and myelopathy on MRI

(A) Varicella-zoster virus (VZV) vasculopathy is characterized by deep-seated lesions, typically at gray–white matter junctions (arrows). (B) VZV myelopathy is characterized by longitudinal serpiginous lesions in the spinal cord (arrow).