Intracerebral Varicella Zoster Virus Vasculopathy in a Patient with Systemic Lupus Erythematosus and Imaging-Clinical Discordance

Objective: Challenging differential diagnosis Background: Varicella zoster virus (VZV) infection can increase the risk of cerebrovascular disease, involving small and large arteries, especially in immunosuppressed patients with ophthalmic division of the trigeminal nerve involvement. We present the case of a patient with intracerebral VZV vasculopathy without overt clinical manifestation but with abnormal imaging findings in the brain magnetic resonance (MR). Case Report: A 59-year-old woman with systemic lupus erythematosus (SLE), without other traditional cardiovascular risk factors, presented to the hospital due to headache, vertical diplopia, decreased of visual acuity of right eye, and disseminated varicella zoster virus (VZV) infection with predominant skin lesions distributed along the ophthalmic division of the right trigeminal nerve. Cerebrospinal fluid (CSF) testing revealed meningitis and positive polymerase chain reaction (PCR) for VZV, and a brain MRI scan showed a right occipital hemorrhagic lesion; thus, she was diagnosed with disseminated VZV infection with neurological involvement. She received intravenous acyclovir for 10 days. One month later, a physical examination was unremarkable and she was asymptomatic, but control brain MR angiography showed stenosis of the right internal carotid and the right middle cerebral artery, compatible with VZV vasculopathy. The PCR for VZV turned negative in CSF but the titers of anti-VZV IgG antibodies in CSF were high, and no increase of plasma autoimmune biomarkers were detected at any time in the course of the clinical evolution. Conclusions: Discordance between imaging findings and clinical manifestations can appear in intracerebral VZV vasculopathy. A differential diagnosis is mandatory, especially if there is underlying immunosuppression.

Automated summary

This case report presents the rare occurrence of intracerebral VZV vasculopathy in a patient with systemic lupus erythematosus (SLE). The patient exhibited neurological symptoms, but no overt clinical manifestation. Imaging findings and CSF testing confirmed the diagnosis. Interestingly, the patient showed symptomatic improvement after treatment, but control brain MR angiography still showed vascular stenosis.