| 赵莹莹，张依，梁文野，徐慧珍，李尧，王瑞金，张拥波.视神经脊髓炎谱系疾病临床特点分析及治疗进展[J].神经损伤功能重建,2018,13(4):177-180 |
| 视神经脊髓炎谱系疾病临床特点分析及治疗进展 |
| Clinical Characteristics Analysis and Treatment Progression of Neuromyelitis Optica SpectrumDisorders |
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| DOI： |
| 中文关键词: 视神经脊髓炎谱系疾病 临床特点 治疗 |
| 英文关键词: neuromyelitis optica spectrum disorders clinical characteristics treatment |
| 基金项目:国家自然科学基金
（No. 8167050067） |
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| 摘要点击次数: 3811 |
| 全文下载次数: 4770 |
| 中文摘要: |
| 目的：研究视神经脊髓炎谱系疾病（NMOSD）的临床特点，探讨治疗进展。方法：回顾性分析13例
NMOSD患者的临床表现、辅助检查结果、治疗及预后，并进行分析。结果：13例NMOSD患者中男女比例
1∶3.3，平均（45±14）岁。急性脊髓炎11例，视神经炎9例，延髓最后区综合征2例，伴发皮肤瘙痒2例，肢体痉
挛性疼痛3例。9例患者血和/或脑脊液AQP4-IgG阳性，3例抗体阴性患者中1例MOG-IgG阳性。11例MRI
表现为脊髓长节段脱髓鞘信号伴强化，1例延髓背侧异常信号，1例视神经异常信号伴强化。大部分患者急
性期给予大剂量激素冲击或免疫球蛋白或血浆置换治疗，部分患者给予硫唑嘌呤预防复发。11 例患者
（84.6%）好转，1例患者（7.7%）无好转，1例患者（7.7%）自行缓解。8例（61.5%）呈复发病程，5例（38.5%）为
单相病程。结论：NMOSD临床表现多种多样，除了典型的核心症状以外，还有很多非典型症状。大剂量激
素冲击、血浆置换和免疫球蛋白治疗仍是目前急性期首选治疗方案，硫唑嘌呤、吗替麦考酚酯和利妥昔单抗
是预防发作的一线用药。 |
| 英文摘要: |
| To investigate the clinical characteristics and treatment progression of neuromyelitis
optica spectrum disorders(NMOSD). Methods: The clinical manifestations, auxiliary examination results,
treatment, and prognosis of 13 NMOSD patients were retrospectively analyzed. Results: The proportion of men
to women in the 13 NMOSD cases was 1∶3.3, and the average age was (45±14) years. There were 11 cases of
acute myelitis, 9 cases of optic neuritis, 2 cases of area postrema syndrome, 2 cases of pruritus, and 3 cases of
spastic pain in limbs. There were 9 cases positive for AQP4-IgG in blood and/or cerebrospinal fluid, and of the 3
cases negative for antibody, 1 was MOG-IgG positive. MRI showed demyelinating signal with intensification in
the long segment of the spinal cord in 11 cases, abnormal signals on the dorsum of the medulla in 1 case, and
optic nerve abnormal signals with strengthening in 1 case. Most patients in acute stage were given high-dose
corticosteroid, immunoglobulin, or plasma exchange treatment, and some patients received azathioprine in
prevention of recurrence. Eleven patients (84.6% ) showed improvement, 1 patient (7.7% ) showed no
improvement, and 1 patient (7.7% ) showed spontaneous improvement. Eight patients (61.5% ) had a course of
recurrence, and 5 patients (38.5% ) had a monophasic course. Conclusion: The clinical manifestations of
NMOSD are varied. Beside the typical core symptoms, there are also many atypical symptoms. Current treatment
during the acute phase includes high-dose glucocorticoid, plasma exchange, and immunoglobulin therapy.
Azathioprine, mycophenolate mofetil, and rituximab are first-line drugs for prevention. |
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