Wolf Andrew B, Palace Jacqueline, Bennett Jeffrey L
Department of Neurology, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Department of Neurology, John Radcliffe Hospital, Oxford, UK.
Curr Treat Options Neurol. 2023 Nov;25(11):437-453. doi: 10.1007/s11940-023-00776-1. Epub 2023 Nov 22.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare inflammatory disorder of the central nervous system that affects both adults and children. Neurologic disability is relapse-driven; therefore, early diagnosis and targeted treatment are critical for effective care. We review the new MOGAD diagnostic criteria and evidence for current acute and preventative therapies.
The International MOGAD Panel has released the first clinical, laboratory, and radiographic criteria for MOGAD diagnosis. These criteria set the stage for evaluating clinical investigations and designing future randomized clinical trials. Prior retrospective studies have evaluated multiple off-label agents for the acute care or prevention of MOGAD attacks, and prospective randomized clinical trials are now underway.
Acute MOGAD attacks are generally responsive to high-dose corticosteroids; however, early use of plasma exchange or intravenous immunoglobulin may be beneficial for severe attacks or cases lacking corticosteroid response. A slow corticosteroid taper may lower the risk of relapse. Preventative treatment has been typically limited to patients with a definitive relapsing disease. While there is no consensus on the choice or duration of treatment, multiple therapies have been retrospectively evaluated. Prospective placebo-controlled trials for interleukin-6 receptor inhibition and neonatal Fc receptor inhibition may open new frontiers for patient care.
髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）是一种罕见的中枢神经系统炎性疾病，可累及成人和儿童。神经功能障碍由复发驱动；因此，早期诊断和靶向治疗对有效治疗至关重要。我们综述了MOGAD的新诊断标准以及当前急性和预防性治疗的证据。
国际MOGAD专家组发布了首个MOGAD诊断的临床、实验室和影像学标准。这些标准为评估临床研究和设计未来的随机临床试验奠定了基础。先前的回顾性研究评估了多种用于MOGAD发作急性期治疗或预防的非标签药物，目前前瞻性随机临床试验正在进行中。
MOGAD急性发作通常对大剂量皮质类固醇有反应；然而，对于严重发作或对皮质类固醇无反应的病例，早期使用血浆置换或静脉注射免疫球蛋白可能有益。缓慢减少皮质类固醇剂量可能会降低复发风险。预防性治疗通常仅限于确诊为复发性疾病的患者。虽然在治疗的选择或持续时间上没有共识，但已对多种治疗方法进行了回顾性评估。针对白细胞介素-6受体抑制和新生儿Fc受体抑制的前瞻性安慰剂对照试验可能为患者治疗开辟新的途径。
