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髓鞘少突胶质细胞糖蛋白抗体和 N-甲基-D-天冬氨酸受体抗体重叠综合征:来自最近病例报告的见解。

Myelin oligodendrocyte glycoprotein antibody and N-methyl-d-aspartate receptor antibody overlapping syndrome: insights from the recent case reports.

机构信息

Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.

Department of Neurology, Zhejiang Hospital, Hangzhou, China.

出版信息

Clin Exp Immunol. 2024 Jan 9;215(1):27-36. doi: 10.1093/cei/uxad109.

Abstract

The overlapping of two or more types of neural autoantibodies in one patient has increasingly been documented in recent years. The coexistence of myelin oligodendrocyte glycoprotein (MOG) and N-methyl-d-aspartate receptor (NMDAR) antibodies is most common, which leads to a unique condition known as the MOG antibody and NMDAR antibody overlapping syndrome (MNOS). Here, we have reviewed the pathogenesis, clinical manifestations, paraclinical features, and treatment of MNOS. Forty-nine patients with MNOS were included in this study. They were young males with a median onset age of 23 years. No tumors were observed in the patients, and 24 of them reported prodromal symptoms. The most common clinical presentations were psychiatric symptoms (35/49) and seizures (25/49). Abnormalities on magnetic resonance imaging involved the brainstem (11/49), cerebellum (9/49), and parietal lobe (9/49). Most patients mostly responded to immunotherapy and had a good long-term prognosis. However, the overall recurrence rate of MNOS was higher than that of mono antibody-positive diseases. The existence of concurrent NMDAR antibodies should be suspected in patients with MOG antibody-associated disease having psychiatric symptoms, seizures, movement disorders, or autonomic dysfunction. Similarly, serum MOG antibody testing should be performed when patients with anti-NMDAR encephalitis present with atypical clinical manifestations, such as visual impairment and limb weakness, and neuroradiological findings, such as optic nerve, spinal cord, or infratentorial involvement or meningeal enhancement. Early detection of the syndrome and prompt treatment can be beneficial for these patients, and maintenance immunosuppressive therapy is recommended due to the high overall recurrence rate of the syndrome.

摘要

近年来,越来越多的文献报道了同一患者同时存在两种或以上神经自身抗体的重叠现象。其中以髓鞘少突胶质细胞糖蛋白(MOG)抗体和 N-甲基-D-天冬氨酸受体(NMDAR)抗体的共存最为常见,由此导致一种独特的综合征,即 MOG 抗体和 NMDAR 抗体重叠综合征(MNOS)。本文就 MNOS 的发病机制、临床表现、辅助检查特征和治疗进行了综述。共纳入 49 例 MNOS 患者,均为年轻男性,中位发病年龄 23 岁,均无肿瘤,24 例患者有前驱症状,最常见的临床表现为精神症状(35/49)和癫痫发作(25/49),磁共振成像异常部位包括脑干(11/49)、小脑(9/49)和顶叶(9/49),大多数患者对免疫治疗反应良好,预后较好,但总体复发率高于单抗体阳性疾病。当 MOG 抗体相关疾病患者出现精神症状、癫痫发作、运动障碍或自主神经功能障碍时,应怀疑同时存在 NMDAR 抗体;当抗 NMDAR 脑炎患者出现视神经、脊髓、颅后窝受累或脑膜强化等不典型神经影像学表现,或出现视力障碍和肢体无力等不典型临床表现时,应进行血清 MOG 抗体检测。早期发现该综合征并及时治疗可能对患者有益,鉴于该综合征总体复发率较高,建议维持免疫抑制治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4ee6/10776248/c85347773a7f/uxad109_fig5.jpg

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