Treatment of Varicella Zoster Virus (VZV)-associated vasculitis and stroke in a 5-year-old girl

VZV-associated vasculitis is a rare cause of stroke in children. Its pathogenesis is equivocal and no established guidelines for treatment exist. We report the case of a 5-year-old girl with acute onset of left-sided hemiparesis 2 month after chickenpox infection. MRI and MR-DWI displayed an acute infarction in the territory of the right ACI. Cerebral angiography demonstrated a stenosis of the right distal ACI. Over two weeks, the neurological status improved, but MRA and Doppler sonography revealed increasing narrowing of the right ACI. In addition, stenosis of the right and left MCA newly occured. At this time, CSF-examination showed mild pleocytosis and normal protein content. The VZV-PCR in CSF was negative, but the VZV-specific antibody index was strongly positive. The girl was treated with acyclovir, prednisolone, azathioprin and aspirin leading to complete resolvement of neurological symptoms and marked improvement of cerebral vessel stenosis. The findings in this case suggest a para- or postinfectious pathogenesis of VZV-associated vasculitis. We conclude that a combined antiviral and immunosuppressive medication may be a reasonable therapeutic approach for the treatment of patients with VZV-related stroke.