aqp -4阳性视神经脊髓炎伴MOG抗体病的诊治特点

V. E. Avdeeva, A. Kotov
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Antibodies to AQP-4 were found in 5 patients, antibodies to MOG were found in 9 patients. 89 % of patients with MOG antibody disease had the number of antibodies to MOG less than 50 pg/ml, determined by the Sandwich-type ELISA method; therefore, multiple sclerosis cannot be excluded (considering the oligoclonal IgG type 2 in three patients). To clarify the diagnosis, antibodies to MOG must be tested by more specific method of live cell-based assay in these patients.Based on this group of patients, it can be assumed that the younger the patient was, the earlier the diagnosis was made and treatment started, the better was prognosis. The prognosis was more favorable in patients with AQP-4-positive neuromyelitis in whom the disease debuted with optic neuritis. Patients with MOG antibody disease had a more favorable prognosis if the disease debuted with a supratentorial brain lesion; less favorable - when oligoclonal IgG type 2 was detected. 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引用次数: 0

摘要

背景。随着对多发性硬化知识的增加,人们对其他形式的脱髓鞘疾病产生了兴趣,其中可以区分神经脊髓炎视谱障碍和MOG(髓鞘少突胶质细胞糖蛋白)抗体疾病。目的:提高aqp -4阳性视神经脊髓炎合并MOG抗体疾病的诊治效率,评价其远期疗效。材料和方法。该研究包括14例患者:儿童、青少年、成人和老年人(女性9例,男性5例)。灾变持续时间1 ~ 6年。结果和讨论。5例检测到AQP-4抗体,9例检测到MOG抗体。采用夹心型ELISA法检测MOG抗体数小于50 pg/ml的MOG抗体病患者占89%;因此,不能排除多发性硬化症(考虑到3例患者的寡克隆IgG 2型)。为了明确诊断,必须在这些患者中采用更具体的活细胞检测方法检测MOG抗体。根据这组患者,可以认为患者年龄越小,诊断越早,治疗越早,预后越好。aqp -4阳性神经脊髓炎以视神经炎首发的患者预后较好。MOG抗体疾病患者如果首发时伴有幕上脑病变,预后较好;当检测到2型寡克隆IgG时,不太有利。性别对疾病的转归无影响。根据临床资料对这种疾病进行鉴别诊断实际上是不可能的。该研究是在一个小群体中进行的,因此很难将结果转化为患有视神经脊髓炎的患者群体。在治疗过程中,几乎所有患者在使用糖皮质激素、人免疫球蛋白制剂时都表现出积极的动态。血浆置换术对aqp -4阳性视神经脊髓炎无效,对部分MOG抗体疾病患者有阳性效果。细胞抑制剂治疗对aqp -4阳性视神经脊髓炎有效。利妥昔单抗对MOG抗体疾病患者的B细胞消耗治疗有效。干扰素制剂没有产生积极作用。
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Features of the diagnosis and treatment of patients with AQP-4-positive neuromyelitis optica and MOG antibody disease
Background. As the amount of knowledge about multiple sclerosis increases, there is an interest in other forms of demyelinating diseases, among which neuromyelitis optica spectrum disorder and MOG (myelin oligodendrocyte glycoprotein) antibody disease can be distinguished.Objective: to improve the efficiency of diagnosis and treatment, to assess the long-term outcome in patients with AQP-4-positive neuromyelitis optica and MOG antibody disease.Materials and methods. The study included 14 patients: children, adolescents, adults, and elderly (9 - female, 5 -male). The duration of catamnesis ranged from 1 year to 6 years.Results and discussion. Antibodies to AQP-4 were found in 5 patients, antibodies to MOG were found in 9 patients. 89 % of patients with MOG antibody disease had the number of antibodies to MOG less than 50 pg/ml, determined by the Sandwich-type ELISA method; therefore, multiple sclerosis cannot be excluded (considering the oligoclonal IgG type 2 in three patients). To clarify the diagnosis, antibodies to MOG must be tested by more specific method of live cell-based assay in these patients.Based on this group of patients, it can be assumed that the younger the patient was, the earlier the diagnosis was made and treatment started, the better was prognosis. The prognosis was more favorable in patients with AQP-4-positive neuromyelitis in whom the disease debuted with optic neuritis. Patients with MOG antibody disease had a more favorable prognosis if the disease debuted with a supratentorial brain lesion; less favorable - when oligoclonal IgG type 2 was detected. Gender had no influence on the outcome of the disease.Conclusion. Differential diagnosis of this diseases based on clinical data is practically impossible. The study was carried out in a small group, so it is difficult to translate the results to the population of patients with neuromyelitis optica spectrum disorders. During treatment, almost all patients show positive dynamics when using glucocorticosteroids, human immunoglobulin preparations. Plasmapheresis was ineffective in patients with AQP-4-positive neuromyelitis optica, in some patients with MOG antibody disease the positive effect was observed. Cytostatic therapy was effective in patients with AQP-4-positive neuromyelitis optica. B cell depletion therapy with rituximab was effective in patients with MOG antibody disease. Interferon preparations did not give a positive effect.
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