When Shingles Strike the Brain: Malignant Middle Cerebral Artery Syndrome due to Varicella Zoster Virus Vasculopathy: A Case Report

Case Diagnosis: Malignant Left Middle Cerebral Artery syndrome secondary to Varicella Zoster Virus Vasculopathy

Case Description or Program Description: A 23-year-old-male male with a history of recent shingles presented to the emergency department due to acute onset lethargy and word finding difficulty. Brain CT scan revealed left territory hypodensities with CTA showing proximal left M1 and distal M2 vessel occlusions. He was administered tPA followed by mechanical thrombectomy, and ultimately left M1 stent placement. Patient’s course was complicated by in-stent thrombosis and intracranial edema, so ultimately underwent decompressive hemicraniectomy. He suffered severe right sided hemiparesis. Digital subtraction angiography (DSA) during thrombectomy was consistent with a vasculopathy thought to be Varicella-zoster virus (VZV)-related. He was started on anti-platelet, statin, famciclovir, and eventually discharged to inpatient rehabilitation. The patient received a comprehensive rehabilitation program and was eventually discharged to home safely.

Setting: Inpatient rehabilitation.

Assessment/Results: Patient demonstrated Increased functionality including right upper and lower extremities by the time he completed inpatient rehabilitation

Discussion (relevance): Varicella-zoster virus (VZV) can lead to many different clinical pathologies including vasculopathy, and subsequently, stroke. Pathogenesis of VZV vasculopathy as well as the presence of autoantibodies and coagulation proteins may contribute to the occlusion of cerebral arteries. Laboratory analysis (CSF studies) and imaging (CT, MRI) are the main drivers of diagnosis. The first line treatment for VZV vasculopathy is antiviral therapy for at least 14 days with some studies recommending a short course of high-dose steroids. As greater than 90% of individuals become infected with VZV, this has massive clinical significance as varicella precedes transient cerebral arteriopathy of childhood in 44 percent of cases, however it is exceedingly rare in the adult population.

Conclusions: This case highlights a rare but critical complication of VZV infection: malignant MCA syndrome. Early recognition and treatment, including antiviral and antiplatelet or anticoagulant therapy, are essential for managing VZV-related vasculopathy and improving patient outcomes.