摘要
目的分析伴有瘙痒视神经脊髓炎谱系疾病(NMOSD)的临床特点。方法回顾性收集2007年6月至2015年6月本院神经内科确诊的54例伴有瘙痒NMOSD患者的临床资料,分析患者的临床表现、免疫学指标、脑脊液指标、寡克隆区带(OCB)、抗水通道蛋白4(AQP4)抗体及颅脑脊髓MRI影像学特征。结果54例患者中,女47例,占比87.04%;年龄(40.38±14.97)岁,发病年龄(37.00±14.38)岁;病程1.65(0.80,4.00)年。临床症状以呃逆呕吐(44.44%)、二便异常(44.44%)、视力(68.52%)、运动(66.67%)、感觉(72.22%)、神经性疼痛(38.89%)为多见。以瘙痒首发(35.19%),上肢(31.48%)、下肢(24.07%)、颈部(20.37%)、前胸(18.52%)、后背(24.07%)、肩部(11.11%)为瘙痒常见发生部位。抗体检查结果:IgAQP4抗体阳性率为90.48%,抗核抗体(ANA)阳性率为30.00%;Sjogren氏综合征A(SSA)抗体阳性率为22.58%;Sjogren氏综合征B(SSB)抗体阳性率为12.50%。血清免疫学结果:IgG为(11.76±3.34)g/L,IgA为(2.13±0.72)g/L,IgM为(1.25±0.62)g/L,补体C3为(1.01±0.28)g/L,补体C4为0.22(0.16,0.26)g/L,总补体活性(CH50)为(42.72±9.74)g/L。脑脊髓MRI影像学结果:累及延髓(32.65%)、延髓至上颈髓(24.49%)、含C1长节段脊髓损伤(48.99%)、侧脑室周围(32.65%)、脑叶(51.02%)、不含C1长节段脊髓损伤(51.01%)。结论伴有瘙痒NMOSD女性较多,瘙痒可首发症状,上肢、下肢、颈部、前胸、后背、肩部是瘙痒常见发生部位,呃逆呕吐、二便异常、视力、运动、感觉、神经性疼痛是患者最常见症状,延髓及延髓至上颈髓、含与不含C1长节段脊髓损伤、侧脑室周围、脑叶是其主要累及部位。
Objective To analyse the clinical characteristics of neuromyelitis optica spectrum disorders(NMOSD)with pruritus.Methods The clinical data of 54 patients with pruritus NMOSD diagnosed in the department of Neurology in our hospital from June 2007 to June 2015 were retrospectively collected,the clinical manifestations,immunological indexes,cerebrospinal fluid indexes,oligoclonal bands(OCB)and anti aquaporin-4(AQP4)antibodies and craniocerebral spinal cord MRI imaging features of the patients were analyzed.Results Among the 54 patients,47 were female,accounting for 87.04%;the age(40.38±14.97)years,onset age(37±14.38)years,and the course of disease 1.65(0.80,4.00)years.The most common clinical symptoms were hiccup or vomiting(44.44%),bowel or bladder dysfunction(44.44%),visual acuity(68.52%),movement disorders(66.67%),sensory disturbances(72.22%),neuropathic pain(38.89%).The patients with pruritus(35.19%)were the initial symptoms,and the common pruritus occurred in the upper limbs(31.48%),lower limbs(24.07%),neck(20.37%),chest(18.52%),back(24.07%),shoulder(11.11%)was the common site of affection.Antibody examination:the positive rate of anti-AQP4 antibody was 90.48%,the positive rate of anti-nuclear antibody(ANA)was 30.00%,the positive rate of Sjogren's syndrome A(SSA)was 22.58%,the positive rate of Sjogren's syndrome B(SSB)was 12.50%.Serum immunological results:IgG was(11.76±3.34)g/L,IgA was(2.13±0.72)g/L,IgM was(1.25±0.62)g/L,complement C3 was(1.01±0.28)g/L,complement C4 was 0.22(0.16,0.26)g/L,and complement hemolytic activity(CH50)was(42.72±9.74)g/L.Cerebral and spinal cord MRI results showed that it involved the medulla oblongata(32.65%),the superior cervical medulla oblongata(24.49%),including C1 long segment spinal cord injury(48.99%),periventricular lateral injury(32.65%),lobar injury(51.02%),and excluding C1 long segment spinal cord injury(51.01%).Conclusion Female are the main patients of NMOSD with pruritus,pruritus would be the initial symptoms,upper limbs,lower limbs,neck,chest,back,shoulder are th
作者
武宁
李健
张付生
王玉鸽
王炎强
WU Ning;LI Jian;ZHANG Fusheng;WANG Yuge;WANG Yanqiang(Weifang Medical University,Weifang,Shandong,261031,China;The Second Department of Neurology,Affiliated Hospital of Weifang Medical University,Weifang,Shandong,261031,China;Department of Neurology,Shandong Provincial Hospital,Ji'nan,Shandong,250021,China;Department of Neurology,the Third Affiliated Hospital of Sun Yat-sen University,Guangzhou,Guangdong,510630,China)
出处
《当代医学》
2023年第21期122-125,共4页
Contemporary Medicine
基金
潍坊市科学技术发展计划项目(2019GX021)。
关键词
视神经脊髓炎谱系疾病
神经性瘙痒
抗水通道蛋白4
脑干
极后区
长节段脊髓炎
MRI
Neuromyelitis optica spectrum disorders
Pruritus
Aquaporin-4
Brainstem
Area postrema
Longitudinally extensive tranceverse myelitis
MRI
