Cerebral varicella-zoster vasculopathy sine herpete Atypical Ramsay-Hunt syndrome in an immunocompetent patient

OBJECTIVE: Report the case of an immunocompetent patient with atypical Ramsay Hunt syndrome sine herpete associated with acute Varicella zoster virus (VZV) central nervous system (CNS) vasculopathy. BACKGROUND: VZV can be reactivated causing neurological complications including CNS vasculitis. The incidence of VZV vasculopathy in adults is unknown. It is more common in immunocompromised individuals. A history of recent zoster, varicella rash and cerebrospinal fluid (CSF) pleocytosis is the classic presentation. However, 30% of cases present with previous no rash or without typical CSF pleocytosis. CASE REPORT: A 54 year-old, immunocompetent man without medical history of varicella or zoster, was admitted to our center with left hearing loss, tinnitus and vertigo. Physical examination revealed minor peripheral left facial paresis and horizontal nystagmus evoked by lateral gaze bilaterally. No lesions were seen in the ear channel or on the palate. Magnetic resonance imaging (MRI) showed multiple lesions consistent with acute stroke. Angio-RM demonstrated narrowing of anterior cerebral arteries and left posterior cerebral artery. HIV serology was negative, and CSF had a protein level of 60 mg/dL, without pleocytosis. CNS vasculitis was suspected, and the patient was started on intravenous (IV) methylprednisolone. A control brain MRI at day 3 of admission showed new asymptomatic ischemic lesions. Intravenous cyclophosphamide was initiated. Polymerase-chain-reaction (PCR) tested in the initial CSF resulted positive for VZV. Intravenous acyclovir was started with substantial clinical and imaging improvement. Thirty days after admission, the patient presented transient right brachio-crural paresis, without new brain lesions. He underwent a new lumbar puncture with persistence of positive VZV PCR. A second course of IV acyclovir was completed. The patient was asymptomatic 14 months later. CONCLUSIONS: VZV CNS vasculopathy may present with atypical clinical and CSF characteristics. Patients with suspected CNS vasculitis without pathological confirmation should be tested for this treatable condition, regardless of their immune status. Disclosure: Dr. Gonzalez Otarula has nothing to disclose. Dr. Bruno has nothing to disclose. Dr. Pujol-Lereis has nothing to disclose. Dr. Ameriso has nothing to disclose.