To emphasize the importance of considering a broader differential including Varicella Zoster Virus (VZV)-associated vasculopathy when examining immunocompetent patients presenting with stroke when other signs of VZV infection are present, including acute retinal necrosis (ARN).

VZV is known to cause chickenpox. Upon reactivation later in life, it is also well-known for the clinical syndrome herpes zoster. Lesser-known complications exist including plexopathy, vasculopathy, and stroke. Here we describe a case of a 52-year-old male, recently diagnosed with systemic sarcoidosis (SS), never treated with immunosuppressives or corticosteroids, who presented with acute ischemic stroke due to VZV vasculopathy in the setting of ARN.

Case: A 52-year-old male with biopsy-confirmed SS presented with three weeks of progressive right eye pain and sudden onset of difficulty speaking. CT angiogram of brain demonstrated vessel narrowing of left superior M2 branch and wedge infarct of the left frontal lobe. MRI of the brain confirmed this and uncovered punctate areas of enhancement of right frontal and temporal lobes. Initial Cerebrospinal fluid (CSF) analysis was not conclusive for an infectious or inflammatory process. A dilated fundus eye exam demonstrated retinal whitening and vitritis consistent with ARN. Anterior chamber paracentesis was positive for VZV DNA on polymerase chain reaction (PCR). He was treated with antivirals and a course of systemic steroids. Subsequently, high levels of CSF VZV IgG antibodies were detected, though no index was obtained. MRI findings and subsequent results allowed us to formulate the final diagnosis and treatment plan.

Even in known SS, it is imperative to consider a broad differential diagnosis when unusual symptoms occur. VZV vasculopathy should be considered when other signs of VZV infection are discovered, including ARN. Diagnostic testing should involve CSF studies to evaluate for VZV IgG antibodies, and PCR analysis of ocular fluid.