CGRA7: A GAME OF HIDE AND SEEK: VARICELLA ZOSTER CEREBRAL VASCULOPATHY IN AN ADULT WITH WISKOTT‐ALDRICH SYNDROME

Abstract

A 37 year old male presented to hospital with progressive dysphasia, on a background of Wiskott-Aldrich syndrome (WAS) and end-stage renal failure secondary to IgA nephropathy. The diagnosis of WAS was established in childhood, based on thrombocytopaenia with recurrent epistaxis; severe T cell deficiency and marginal zone B cell deficiency with recurrent infections; atopic eczema; and two affected brothers. Eight months prior to the current presentation, replacement intravenous immunoglobulin therapy was commenced for management of sinopulmonary infections and bronchiectasis. Six months prior to presentation, the patient developed T10 dermatomal zoster and a course of acyclovir was completed. Four months later the patient presented with right-sided hemiplegia due to an acute left basal ganglia infarct, in the context of recent tranexamic acid therapy for epistaxis. Multiple new cerebral microhaemorrhages were visualized on an MRI scan of the brain. The patient's motor symptoms resolved but he developed progressive language deficits with difficulty reading, writing and naming objects. There was no new focal neurology, or signs of meningism. Neuroimaging by MRI, CT angiogram and cerebral angiography demonstrated a stenosing vasculopathy involving the proximal large intracranial vessels, with extensive pial-pial collateralization and evidence of progression over two weeks. New ischaemic changes were demonstrated in the left temporal and occipital white matter. Varicella zoster virus (VZV) DNA was detected in cerebrospinal fluid, in keeping with VZV vasculopathy. Treatment with three weeks of intravenous acyclovir and one week of high dose corticosteroids, led to clinical resolution. Long-term prophylactic valacyclovir was recommended. This is a rare case of VZV cerebral vasculopathy presenting four months after reactivation of VZV infection in an immunocompromised patient. VZV is implicated in a widening spectrum of neurovascular diseases. Clinicians should be aware of these unusual VZV manifestations in immunodeficient patients, as prompt diagnosis and antiviral therapy can result in favourable clinical outcomes.