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髓鞘少突胶质细胞糖蛋白抗体相关性疾病的诊断:国际 MOGAD 专家组提出的标准。

Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria.

机构信息

Division of Child Neurology, Children's Hospital of Philadelphia, Department of Neurology and Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, PA, USA.

Departments of Neurology and Ophthalmology, Programs in Neuroscience and Immunology, University of Colorado School of Medicine, Anschutz Medical Campus, Aurora, CO, USA.

出版信息

Lancet Neurol. 2023 Mar;22(3):268-282. doi: 10.1016/S1474-4422(22)00431-8. Epub 2023 Jan 24.

Abstract

Serum antibodies directed against myelin oligodendrocyte glycoprotein (MOG) are found in patients with acquired CNS demyelinating syndromes that are distinct from multiple sclerosis and aquaporin-4-seropositive neuromyelitis optica spectrum disorder. Based on an extensive literature review and a structured consensus process, we propose diagnostic criteria for MOG antibody-associated disease (MOGAD) in which the presence of MOG-IgG is a core criterion. According to our proposed criteria, MOGAD is typically associated with acute disseminated encephalomyelitis, optic neuritis, or transverse myelitis, and is less commonly associated with cerebral cortical encephalitis, brainstem presentations, or cerebellar presentations. MOGAD can present as either a monophasic or relapsing disease course, and MOG-IgG cell-based assays are important for diagnostic accuracy. Diagnoses such as multiple sclerosis need to be excluded, but not all patients with multiple sclerosis should undergo screening for MOG-IgG. These proposed diagnostic criteria require validation but have the potential to improve identification of individuals with MOGAD, which is essential to define long-term clinical outcomes, refine inclusion criteria for clinical trials, and identify predictors of a relapsing versus a monophasic disease course.

摘要

血清髓鞘少突胶质细胞糖蛋白(MOG)抗体存在于获得性中枢神经系统脱髓鞘综合征患者中,这些疾病与多发性硬化症和水通道蛋白 4 阳性视神经脊髓炎谱系障碍不同。基于广泛的文献回顾和结构化的共识过程,我们提出了 MOG 抗体相关疾病(MOGAD)的诊断标准,其中 MOG-IgG 的存在是核心标准。根据我们提出的标准,MOGAD 通常与急性播散性脑脊髓炎、视神经炎或横贯性脊髓炎相关,较少与大脑皮质脑炎、脑干表现或小脑表现相关。MOGAD 可以表现为单相或复发病程,MOG-IgG 细胞检测对诊断准确性很重要。需要排除多发性硬化症等诊断,但并非所有多发性硬化症患者都需要进行 MOG-IgG 筛查。这些拟议的诊断标准需要验证,但有可能提高 MOGAD 患者的识别能力,这对于确定长期临床结局、细化临床试验纳入标准以及识别复发与单相病程的预测因素至关重要。

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