抗髓鞘相关糖蛋白抗体的检测及相关周围神经病研究  

Myelin-associated glycoprotein associated neuropathies:clinical features and antibody detection

作　　者：钱敏[1] 陈琳[1] 赵玉英[4] 李剑[2] 任海涛[1] 管宇宙[1] 邸茜[3] 王亚静[3] 关鸿志[1] 崔丽英[1,5] Qian Min;Chert Lin;Zhao Yuying;Li Jian;Ren Haitao;Guan Yuzhou;Di Qian;Wang Yajing;Guan Hongzhi;CuiLiying(Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijiing 100730,China)

机构地区：[1]中国医学科学院北京协和医院神经科,100730 [2]中国医学科学院北京协和医院血液科,100730 [3]中国医学科学院北京协和医院检验科,100730 [4]山东大学齐鲁医院神经内科 [5]中国医学科学院神经科学中心

出　　处：《中华神经科杂志》2018年第12期955-960,共6页Chinese Journal of Neurology

摘　　要：目的研究抗髓鞘相关糖蛋白(myelin—associated glyeoprotein,MAG)抗体相关周围神经病的临床及电生理特点、抗体检测方法以及治疗和预后,以提高对该病的认识。方法总结2014年4月至2018年2月北京协和医院和山东大学齐鲁医院共6例抗MAG抗体阳性的IgM型单克隆丙种球蛋白血症相关周围神经病患者病例资料,对其临床特点、电生理特征、辅助检查结果包括抗MAG抗体检、狈0结果进行分析,并随访其治疗和预后。结果6例患者中,男性5例,女性1例,发病年龄50—77岁,病程3个月~6年。6例均以肢体远端麻木起病,渐发展至肢体近端受累,其中3例出现运动受累(肢体肌力Ⅲ~Ⅳ级)。5例出现行走不稳,双足踩棉感,1例伴下肢远端过电样疼痛。5例出现四肢腱反射减低,6例均有长手套、袜套样深浅感觉减退。5例患者行腰椎穿刺,脑脊液蛋白0.75~1.33g/L。6例患者均发现血清单克隆蛋白,其中4例为IgM K型,2例为IgG K和IgM K双克隆型。原发血液病诊断:4例诊断为意义未明的单克隆免疫球蛋白血症,2例诊断为巨球蛋白血症。6例患者均行肌电图检测,提示四肢多发性周围神经病,脱髓鞘损害,感觉神经损害为主,下肢重;2例针极肌电图可见自发电位,运动单位时限增宽、波幅增高,主动收缩募集减少,提示继发慢性轴索损害。6例均行基于转染细胞和动物周围神经组织的间接免疫荧光检测抗MAG-IgM抗体,结果均阳性。4例在确诊前给予激素以及静脉注射免疫球蛋白治疗,症状仍逐渐进展。确诊后3例行RCD(利妥昔单抗+地塞米松+环磷酰胺)化疗,1例好转[治疗前/后改良Rankin量表(mRS)评分为3/2分,随诊3年],1例平稳(治疗前/后mRS评分为2/2分,随诊2年),1例随诊中;1例给予利妥昔单抗治疗,病情好转(治疗前/后mRS评分为3/1分,随诊1年)。结论抗MAG抗体相关周围神经病临床表现为缓慢进展的远端对称性周围神经病,感觉受累为主,可Objective To investigate the clinical and electrophysiological characteristics of antimyelin-associated glycoprotein 0VIAG)-associated peripheral neuropathy,as well as its antibody detection methods, treatment and prognosis.Methods Six cases of IgM paraproteinemia and anti-MAG antibody-associated peripheral neuropathy were summarized.All of the patients came from Peking Union Medical College Hospital and Qilu Hospital since April 2014 to February 2018.The clinical features,electrophysiological characteristics,and auxiliary examinations including anti-MAG antibody results were analyzed,and the treatment and prognosis were followed.Results Of the six patients,five were male and one was female.The age of onset was 50-77 years and the duration was three months to six years.All the six cases suffered from numbness of distal limbs and gradually progressed to the proximal limbs,of which three cases had muscleweakness.Walking instability occurred in five cases.One patient had electricity-like pain in the lower extremities. Reflexes in the four limbs decreased in five cases,and gloves and socks-like deep sensation decreased in six cases.Five patients underwent lumbar puncture and the cerebral spinal fluid protein ranged from 0.75 to 1.33g/L.Serum monoclonal proteins were found in six patients,of which four were IgM kappa and two were IgG kappa and IgM kappa biclonal.Four cases were diagnosed with monoclonal gammopathy of undetermined significance and two cases were diagnosed with Waldenstrom's macroglobulinaemia. Abnormal electromyography was detected in all the six cases,suggesting demyelinating peripheral nerve damage with secondary axonal damage,which was sensory predominant and more severe in lower limbs than upper limbs.In all the six cases,anti-MAG-IgM antibodies were all positive by indirect immunofluorescence assay based on transfected cells and peripheral nerve tissues.After the diagnosis,three patients underwent RCD (rituxima +dexamethasone +cyclophosphamide)chemotherapy.One patient improved (the modified Rankin

关 键 词：髓鞘相关糖蛋白抗体 周围神经系统疾病 免疫荧光测定 副蛋白血症 WALDENSTROM巨球蛋白血症 

分 类 号：R745[医药卫生—神经病学与精神病学]