视神经脊髓炎谱系病40例临床特点分析  

The Clinical Features of Neuromyelitis Optica Spectrum Disorders

作　　者：陈雪娇 陈柏龄 陈跃鸿 黄伟平 CHEN Xuejiao;CHEN Bailing;CHEN Yuehong

机构地区：[1]福建医科大学附属漳州市医院,福建漳州363000

出　　处：《中外医学研究》2018年第28期5-8,共4页CHINESE AND FOREIGN MEDICAL RESEARCH

摘　　要：目的:探讨视神经脊髓炎谱系病(neuromyelitis optica spectrum disorders,NMOSD)患者的临床特点,提高对本病的认识。方法:回顾性分析40例诊断明确的NMOSD患者的临床资料。结果:40例患者中,女32例,男8例,发病年龄15~68岁。合并自身免疫性疾病者20例(50%)。首次发病累及视神经、脊髓、视神经/脊髓、最后区、大脑分别为14例(35.0%)、14例(35.0%)、4例(10.0%)、7例(17.5%)、1例(2.5%);全部患者检测血清水通道蛋白4(AQP4)抗体,阳性者34例(85.0%),阴性者6例(15.0%)。18例(45.0%)与33例(82.5%)患者分别存在颅脑及脊髓病灶。表现为单相病程11例(27.5%),复发缓解29例(72.5%)。所有患者均使用糖皮质激素冲击治疗,24例(60%)遗留有不同程度后遗症。结论:视神经脊髓炎谱系疾病好发于青壮年女性,最后区综合征起病者占比较大,且常合并自身免疫性疾病,复发率及致残率均较高,通过临床核心症状及影像学表现,结合血清AQP4抗体检测,可早期诊断并进行合理治疗,减少复发及致残。Objective：To investigate the clinical features of neuromyelitis optica spectrum disorders（NMOSD） to help physicians have a deeper understanding of the disease.Method：The features of forty NMOSD patients were analyzed retrospectively.Result：Forty patients were enrolled,consisting of 32 females and 8 males.The onset age ranged from 15 to 68 years old.Among them,20 patients（50%） were combined with autoimmune diseases.The first onset involved optic nerve in 14 cases （35.0%）,spinal cord in 14 cases（35.0%）,optic nerve/spinal cord in 4 cases（10.0%）,area postrema in 7（17.5%）,cerebrum in only 1（2.5%）.All cases were tested with Aquaporin 4（AQP4） antibody,34 cases（85.0%） were positive and 6 cases（15.0） were negative.Brain and spinal cord abnormalities were found in 18 cases（45.0%） and 33 cases（82.5%）.11 cases（27.5%） appeared to be monophasic and the rest of 29 cases（72.5%） belonged to eased relapsing.All patients were treated with high dosage of glucocorticoid,and 24 patients（60%） had different degrees of sequelae.Conclusion： NMOSD frequently occurs in young women,and have high rate of disease relapse and disability.NMOSD is often combined with autoimmune diseases,and has high proportion of patients with area postrema syndrome as onset.By means of clinical core symptoms and imaging,combined with detection of AQP4 antibody,we can make early diagnosis and reasonable treatment in order to reduce the rate of disease relapse and disability.

关 键 词：视神经脊髓炎谱系病 自身免疫性疾病 水通道蛋白4 磁共振成像 最后区综合征 临床特点 

分 类 号：R744.52[医药卫生—神经病学与精神病学]