547: CEREBRAL EDEMA IN MOG ANTIBODY DISEASE TREATED WITH INVASIVE MULTIMODAL NEUROMONITORING

Abstract

Introduction: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a neuroinflammatory disorder with a range of clinical phenotypes. MOGAD is typically steroid-responsive, with more refractory cases receiving intravenous immunoglobulin (IVIG) or plasmapheresis. Invasive multimodal neuromonitoring provides real-time measurements of intracranial pressure (ICP) and brain tissue oxygenation (PbtO2) and may be accomplished via insertion of an intraparenchymal probe, also known as a “bolt”. It is becoming an important strategy in the management of patients with traumatic brain injury but is less well described in other acute neurological conditions associated with increased ICP. We describe a case of MOGAD in a child that presented as life-threatening cerebral edema, in which invasive multimodal neuromonitoring with ICP- and PbtO2-directed therapy was employed. This child ultimately survived with some persistent neurological deficits but with normal mood and affect and the ability to eat, drink, and ambulate independently. Description: A 7-year old boy presented with headaches, nausea and somnolence and was found to have increased ICP of unclear etiology. He then progressed to develop exam findings consistent with herniation. He received emergent medical therapies including hypertonic (3%) saline, hyperventilation, and sedation. A bolt was placed for invasive neuromonitoring, which guided treatment based on real-time measurements of ICP and PbtO2. Workup for the etiology of his severe cerebral edema revealed serum MOG antibodies. He received immunomodulatory therapies including steroids, plasmapheresis, and tocilizumab. He was discharged two months later with expressive and receptive aphasia and mild gait imbalance, but with recovered bowel and bladder continence, independent ambulation, and the ability to eat, drink, and interact socially with a normal mood and affect. Discussion: We describe a child who presented with cerebral edema and herniation due to MOGAD. He was managed with invasive multimodal neuromonitoring in addition to standard immunomodulatory treatments and had a favorable outcome. While further data is needed, invasive multimodal neuromonitoring may have utility as an adjunct to standard therapy in children with MOGAD and severe cerebral edema.