Multifocal Varicella-Zoster Virus Vasculopathy Without Rash

Abstract

A 51-year-old woman with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) developed stepwise progressive focal neurological deficits without zoster rash. Multifocal ischemic infarcts were seen on magnetic resonance imaging, and cerebral angiography revealed focal stenosis of arteries affecting the intracranial circulation. A brain biopsy was nondiagnostic. Virological etiology of the disease was verified by the detection of varicella-zoster virus antibody in cerebrospinal fluid and by reduced serum-cerebrospinal fluid varicella-zoster virus IgG ratios (compared with normally high ratios of total IgG and albumin). Treatment with intravenous acyclovir stabilized but did not significantly improve her neurological deficits.

Concurrent maxillary herpes zoster, meningitis, focal cerebral arteriopathy, and ischemic stroke: A case report

“…One study found that the most affected site of cerebral arteriopathy was the M1 segment of the middle cerebral artery, 10 while another study showed involvement of both large and small arteries in half of the participants followed by involvement of pure small‐artery and pure large‐artery disease 5 . In our case, the segmental luminal narrowing and dilatation of the right cavernous internal carotid artery were in line with some specific morphological pattern of varicella‐zoster vasculopathy described in previous publications 4,11 . Significant narrowing of the arterial lumen, possibly due to inflamed intima, could lead to profound hemodynamic changes that further caused the watershed infarction together with emboli dislocated to distal arterial sites if additional thrombus was formed 6,12 …”

Concurrent maxillary herpes zoster, meningitis, focal cerebral arteriopathy, and ischemic stroke: A case report

“…One study found that the most affected site of cerebral arteriopathy was the M1 segment of the middle cerebral artery, 10 while another study showed involvement of both large and small arteries in half of the participants followed by involvement of pure small‐artery and pure large‐artery disease 5 . In our case, the segmental luminal narrowing and dilatation of the right cavernous internal carotid artery were in line with some specific morphological pattern of varicella‐zoster vasculopathy described in previous publications 4,11 . Significant narrowing of the arterial lumen, possibly due to inflamed intima, could lead to profound hemodynamic changes that further caused the watershed infarction together with emboli dislocated to distal arterial sites if additional thrombus was formed 6,12 …”

Herpes zoster involving the abducens and vagus nerves without typical skin rash

“…Immunohistochemical examination, viral isolation tests, PCR for VZV DNA, and cell immunoassays are sometimes necessary to ensure differential diagnosis from other diseases. [9,10] In the present case, we confirmed a diagnosis of herpes zoster using a PCR-based test of erosional exudate.…”

Ischemic Optic Neuropathy Secondary to Varicella-Zoster Vasculitis Mimicking Giant Cell Arteritis: Case Report

“…The failure of acute phase reactants to respond to high-dose steroids and devastating bilateral visual loss prompted testing revealing a viral infection. The antiviral therapy stabilized the inflammatory markers and vision as in previously reported cases [13][14][15][16][17].…”

Concurrent maxillary herpes zoster, meningitis, focal cerebral arteriopathy, and ischemic stroke: A case report

“…One study found that the most affected site of cerebral arteriopathy was the M1 segment of the middle cerebral artery, 10 while another study showed involvement of both large and small arteries in half of the participants followed by involvement of pure small‐artery and pure large‐artery disease 5 . In our case, the segmental luminal narrowing and dilatation of the right cavernous internal carotid artery were in line with some specific morphological pattern of varicella‐zoster vasculopathy described in previous publications 4,11 . Significant narrowing of the arterial lumen, possibly due to inflamed intima, could lead to profound hemodynamic changes that further caused the watershed infarction together with emboli dislocated to distal arterial sites if additional thrombus was formed 6,12 …”

Herpes zoster involving the abducens and vagus nerves without typical skin rash

“…Immunohistochemical examination, viral isolation tests, PCR for VZV DNA, and cell immunoassays are sometimes necessary to ensure differential diagnosis from other diseases. [9,10] In the present case, we confirmed a diagnosis of herpes zoster using a PCR-based test of erosional exudate.…”

Ischemic Optic Neuropathy Secondary to Varicella-Zoster Vasculitis Mimicking Giant Cell Arteritis: Case Report

“…The failure of acute phase reactants to respond to high-dose steroids and devastating bilateral visual loss prompted testing revealing a viral infection. The antiviral therapy stabilized the inflammatory markers and vision as in previously reported cases [13][14][15][16][17].…”

Concurrent maxillary herpes zoster, meningitis, focal cerebral arteriopathy, and ischemic stroke: A case report

“…One study found that the most affected site of cerebral arteriopathy was the M1 segment of the middle cerebral artery, 10 while another study showed involvement of both large and small arteries in half of the participants followed by involvement of pure small‐artery and pure large‐artery disease 5 . In our case, the segmental luminal narrowing and dilatation of the right cavernous internal carotid artery were in line with some specific morphological pattern of varicella‐zoster vasculopathy described in previous publications 4,11 . Significant narrowing of the arterial lumen, possibly due to inflamed intima, could lead to profound hemodynamic changes that further caused the watershed infarction together with emboli dislocated to distal arterial sites if additional thrombus was formed 6,12 …”

Herpes zoster involving the abducens and vagus nerves without typical skin rash

“…Immunohistochemical examination, viral isolation tests, PCR for VZV DNA, and cell immunoassays are sometimes necessary to ensure differential diagnosis from other diseases. [9,10] In the present case, we confirmed a diagnosis of herpes zoster using a PCR-based test of erosional exudate.…”

Ischemic Optic Neuropathy Secondary to Varicella-Zoster Vasculitis Mimicking Giant Cell Arteritis: Case Report

“…The failure of acute phase reactants to respond to high-dose steroids and devastating bilateral visual loss prompted testing revealing a viral infection. The antiviral therapy stabilized the inflammatory markers and vision as in previously reported cases [13][14][15][16][17].…”