Intravenous immunoglobulin and rituximab in a treatment-refractory COVID-19-associated central nervous system vasculitis: A case report and literature review

Abstract

Central nervous system (CNS) vasculitis is an uncommon inflammatory disease that affects the brain and spinal cord. It might present with headache, focal neurological deficits, seizures and encephalopathy. CNS vasculitis might be triggered by systemic vasculitides, connective tissue diseases, malignancies and infectious agents, including severe acute respiratory syndrome coronavirus 2. Diagnosis requires a combination of clinical presentation, serological and cerebral spinal fluid analysis, radiological findings (magnetic resonance imaging and conventional angiography), and brain biopsy. In the lack of randomized prospective studies, the treatment approach is based on retrospective cohort studies, anecdotal reports and therapeutic approaches derived from other vasculitides. In this report, we present a case of a 55-year-old man who presented with hemiparesthesia 39 days after coronavirus disease 2019 (COVID-19) infection. During the disease course, the patient developed right-sided hemiparesis and was referred to our center. Conventional angiogram suggested CNS vasculitis. After an extensive work-up, systemic vasculitis was excluded. Despite having been treated with pulse steroid and cyclophosphamide, the patient experienced relapses. As we administered intravenous immunoglobulin and rituximab, remission was achieved both in clinical and radiological aspects. In this study, we present the first case of treatment-refractory CNS vasculitis associated with COVID-19, which was treated successfully with intravenous immunoglobulin and rituximab. Second, we report a review of COVID-19-related CNS vasculitis articles and applied treatment strategies published in English. To elucidate the mechanism of COVID-19-related CNS vasculitis, further research is needed.