17例AQP4抗体阳性视神经脊髓炎谱系疾病的临床分析  

Clinical analysis of 17 cases with AQP4 antibody positive Neuromyelitis optica spectrum disorders

作　　者：张灯儿 史琴[2] Zhang Denger;Shi Qin(Ningxia Medical University,Yinchuan,Ningxia,750000,China;General Hospital of Ningxia Medical University,Yinchuan,Ningxia,750000,China)

机构地区：[1]宁夏医科大学,宁夏银川750000 [2]宁夏医科大学总医院,宁夏银川750000

出　　处：《当代医学》2020年第13期115-119,共5页Contemporary Medicine

摘　　要：目的探讨AQP4抗体阳性视神经脊髓炎谱系疾病的临床资料、受累部位、临床表现、辅助检查,为临床早期诊断提供借鉴,以降低该病的误诊率、漏诊率。方法选取宁夏医科大学总医院2012年1月至2018年7月诊断为视神经脊髓炎谱系疾病且血清AQP4抗体阳性的患者17例作为研究对象,回顾性分析患者的性别、年龄、首次发病年龄、发病诱因、发病次数等临床资料,临床表现及眼科检查、脑脊液、神经电生理等辅助检查结果。结果17例患者中,女性例数明显高于男性,青壮年多见,复发率高,部分病例合并自身免疫性疾病,首次受累部位最常见于视神经64.7%(11/17),视神经和脊髓均易受累88.2%(15/17),且累及脊髓者多见于颈髓86.7%(13/15)、胸髓80%(12/15)。有93.3%(14/15)的脊髓病灶≥3个椎体节段。有41.2%(7/17)的病例脑脊液(cerebrospinal fluid,CSF)白细胞数升高,有17.6%(3/17)的病例脑脊液蛋白质升高。脑脊液寡克隆区带(oligoclonal bands,OB)阳性占28.6%(2/7)。视觉诱发电位异常者82.4%(14/17),其中潜伏期延长者52.6%(10/19)。结论AQP4抗体阳性的视神经脊髓炎谱系疾病病例女性多见,青壮年多发,疾病复发率高,可合并自身免疫性疾病,首次受累部位常见于视神经,易累及视神经和脊髓,脊髓病变多数≥3个椎体节段,且病灶常位于颈髓和胸髓,该病可导致脑脊液白细胞、蛋白数可升高,诱发电位敏感性较高,可协助早期诊断。Objective To explore the general situation,affected site,clinical manifestation and auxiliary examination of AQP4 antibody positive optic neuromyelitis pedigree disease,so as to provide reference for clinical early diagnosis and reduce the misdiagnosis rate and missed diagnosis rate of the disease.Methods From January 2012 to July 2018,17 cases of patients diagnosed with neuromyelitis optica pedigree diseases and serum AQP4 antibody positive in Ningxia Medical University General Hospital were retrospectively analyzed.The patients'general conditions such as sex,age,age of first onset,predisposing factors of onset,frequency of onset as well as clinical manifestations,ophthalmologic examination,cerebrospinal fluid,neuroelectrophysiology and other auxiliary examination results were investigated and summarized.Results In 17 cases of this study,the incidence of female was obviously higher than that of male.The disease was most common in young adults and has high recurrence rate.Some of cases had autoimmune diseases.64.7%(11/17)of the optic nerves were affected for the first time.During the course of the disease,both optic nerve and spinal cord were easily involved 88.2%(15/17),93.3%(14/15)of spinal cord lesions were≥3 vertebral segments.In 41.2%(7/17)of the cases,the number of leukocytes in cerebrospinal fluid was increased.In 17.6%(3/17)of the cases,the protein in cerebrospinal fluid was increased(CSF).The positive rate of oligoclonal bands(OB)in cerebrospinal fluid were 28.6%(2/7).Abnormal visual evoked potentials were found in 82.4%(14/17)cases,of which 52.6%(10/19)with prolonged latency was the most common.Conclusion Positive AQP4 antibody of neuromyelitis optica spectrum disorder was more likely to occur in women and young adults.The disease had high recurrence rate and may be associated with autoimmune diseases.The first affected part was most common in optic nerve.The optic nerve and spinal cord were most likely to be involved in the course of the disease.≥3 vertebral segments were involved in spinal cord le

关 键 词：视神经脊髓炎谱系疾病 水通道蛋白4抗体 受累部位 临床表现 辅助检查 

分 类 号：R744.52[医药卫生—神经病学与精神病学]