Case Presentation: An 84-year-old male with a past medical history of hypertension, type two diabetes mellitus, and left herpes zoster ophthalmicus treated three months prior presented to the hospital with two days of worsening confusion and altered mentation. Initial Computed Tomography (CT) of the head revealed no evidence of acute infarction. Initial serologic workup revealed no significant electrolyte abnormalities, normal thyroid stimulating hormone, normal B12/folate levels, negative Syphilis antibody, normal ammonia level, noninfectious urinalysis, and negative blood cultures. Continuous electroencephalogram (EEG) showed no epileptiform activity. Less than 24 hours into the patient’s hospitalization, he developed aphasia, and a repeat CT head showed a new area of low attenuation in the basal ganglia. A magnetic resonance imaging (MRI) of the brain revealed acute infarcts involving the left basal ganglia, corona radiata, and smaller infarcts of the superior left cerebellum and medial left temporal lobe. A lumbar puncture was performed and the cerebrospinal fluid (CSF) had 185 red blood cells, 39 white blood cells with 65% lymphocytes, normal glucose, and elevated protein. CSF aerobic culture was negative, herpes simplex virus polymerase chain reaction (PCR) was negative, but a CSF PCR panel was positive for varicella zoster virus (VZV). A cerebral angiogram was performed and showed moderate intracranial vascular disease including 50% narrowing of the proximal superior division of the left middle cerebral artery. Given the imaging, angiogram findings, and CSF PCR results, the diagnosis of acute CVAs secondary to VZV vasculopathy was confirmed. The patient was treated with a five-day course of prednisone 1 mg/kg daily and 3 weeks of IV acyclovir at 10 mg/kg three times per day. The patient had significant functional and behavioral changes following his CVAs and was discharged to a skilled nursing facility for further care.
Discussion: VZV vasculopathy is a complication that can occur in the months following primary VZV infection or reactivation (1). The risk of VZV vasculopathy appears to be increased in patients with herpes zoster ophthalmicus, but it can occur after any VZV infection. The average time between the initial rash and presentation of neurologic symptoms is approximately four months (2,3). VZV vasculopathy classically presents with multifocal acute CVAs but can also be unilateral. It presents with stroke-like symptoms, which can include altered mental status, aphasia, focal weakness, or hemiparesis. It is more common in immunocompromised individuals because of the increased risk of VZV reactivation, but it can also occur in immunocompetent patients. Imaging can suggest VZV vasculopathy, but diagnosis can only be confirmed with VZV PCR or VZV immunoglobulin G testing of the CSF (1). Early recognition and treatment are important to limit morbidity and mortality related to this condition. Treatment is three weeks of acyclovir 10 mg/kg three times daily with a five-day course of prednisone 1 mg/kg (2).
Conclusions: VZV vasculopathy is an important consideration in all patients with a history of VZV infection or reactivation presenting with stroke-like symptoms. Early identification is important to limit morbidity and mortality associated with this condition. VZV vasculopathy is more common in immunocompromised individuals, but this case serves as a reminder that immunocompetent patients can also be affected.
