PP04.3 – 2650: Varicella angiopathy: The current challenges of management

Abstract

Stroke secondary to varicella angiopathy commonly occurs in healthy children presenting with hemiparesis with a history of Varicella Zoster Virus (VZV) infection less than 12 months earlier. It accounts for approximately 31% of all children presenting with arterial ischaemic strokes. With recent advances in intrathecal antibody testing clinicians are faced with novel challenges in the management of these children. We describe a 4-year-old child who presented to the emergency department with right arm and leg weakness, slurred speech, and right-sided facial weakness over the previous few hours. He had three episodes in the previous six months characterised by a sudden drop to the ground, without loss of consciousness or jerking. In thirty minutes he had returned to normal activity. MRI brain confirmed multiple focal areas of infarction in the left cerebral hemisphere. MRA showed significant abnormal narrowing of the terminal left internal carotid and proximal left middle cerebral artery. CSF testing confirmed active VZV infection with intrathecal antibody production. A literature search confirmed that in adults, characteristic ischaemic lesions in the setting of positive CSF markers are considered the consistent criteria for diagnosis of varicella angiopathy with no clear diagnostic guidelines for children. Equally there is no consensus in treatment with authors recommending combination of antiviral and immunosuppressive therapy, others anticoagulant and antiplatelet medication. In this case Infectious Disease and Virology consults were sought and a five-week course of intravenous Acyclovir was commenced with oral aspirin. Repeat CSF testing showed no improvement in VZV IgG levels. Following a 3-month course of oral valciclovir, repeat testing confirmed treatment of VZV infection. MRI and MRA showed persistent arterial narrowing and some resolution in areas of infarction. This case illustrates the challenges faced in diagnosis and treatment for children presenting with stroke suspected secondary to Varicella angiopathy and the benefits of multiprofessional working.