肌肉特异性受体酪氨酸激酶抗体阳性重症肌无力

目的 探讨不同血清抗体重症肌无力(MG)的临床特征.方法 用荧光免疫沉淀法(FIPA)和荧光免疫细胞染色法(CBA)检测119例MG患者血清乙酰胆碱受体抗体(AChR-Ab)和肌肉特异性受体酪氨酸激酶抗体(MuSK-Ab)水平.比较AChR-Ab阳性、MuSK-Ab阳性、血清抗体阴性MG的临床特征.结果 纳入119例患者中,90例AChR-Ab阳性(75.6%),29例阴性:其中5例MuSK-Ab阳性(17.2%),24例血清抗体阴性(82.8%).AChR-Ab阳性、MuSK-Ab阳性和血清抗体阴性MG 3组比较,男女比例和平均发病年龄差异均无统计学意义.3例MuSK-Ab阳性的患者主要表现为延髓肌受累;79.2%(19/24)的血清抗体阴性MG患者表现为美国MG协会(MGFA)Ⅰ型;2例MuSK-Ab阳性的患者MGFA≥Ⅲ型;MuSK-Ab滴度水平与患者病情严重程度相关(r=0.941,P=0.014);MuSK-Ab阳性的患者均未发现有胸腺的异常.结论 MuSK-Ab仅出现在AChR-Ab阴性患者的血清中.MuSK-Ab阳性的患者主要表现为延髓肌受累,病情较重且不伴有胸腺的病变.MuSK-Ab阳性的MG可能是不同于血清AChR-Ab阳性的MG的又一亚型.     

伴肌萎缩重症肌无力4例分析并文献复习

目的探讨伴肌萎缩重症肌无力(myasthenia gravis,MG)患者的临床特征、抗体及电生理特点,提高对此少见疾病的认识。方法回顾性分析作者医院2003—2016年收治的伴肌萎缩MG患者4例的临床资料,分析其临床特征、抗体、神经电生理检查、治疗及预后特点。结果 4例患者病程中出现不同程度的肌肉萎缩,1例为手肌(左侧大鱼际肌、小鱼际肌及第一骨间肌)萎缩,3例为舌肌萎缩(其中1例同时伴面肌萎缩);抗体检查显示乙酰胆碱受体抗体(AChR-Ab)阳性3例,1例为抗骨骼肌特异性受体酪氨酸激酶抗体(MuSK-Ab)阳性。4例患者低频重复神经刺激波幅均可递减,高频刺激波幅未见明显递增。伴手肌萎缩MG患者针极肌电图检查结果提示神经源性损害。结论伴肌肉萎缩MG较罕见,MuSK-Ab、AChR-Ab阳性MG患者均可出现肌肉萎缩,其机制有待进一步明确;早期行神经电生理检查、AChR-Ab及MuSK-Ab测定有助于明确诊断。     

血清乙酰胆碱受体抗体、肌肉特异性酪氨酸激酶抗体浓度与重症肌无力患者临床分型和疾病转归的关系

目的探索血清乙酰胆碱受体抗体(AchR-Ab)、肌肉特异性酪氨酸激酶抗体(MuSK-Ab)浓度与重症肌无力(MG)患者临床分型和疾病转归的关系。方法收集2014年5月-2015年5月在本院神经内科住院治疗的MG患者43例(MG组)和同期在本院健康体检的正常人43例(N组),采用酶联免疫吸附(ELISA)法检测血清AChR-Ab、MuSK-Ab浓度,并计算MG组患者治疗前后的肌无力绝对评分,分析不同临床分型的MG患者治疗前的血清AChR-Ab、MuSK-Ab浓度,并对治疗前后肌无力绝对评分和治疗前后血清AChR-Ab、MuSK-Ab浓度变化进行相关性分析。结果 (1)治疗前,MG组和N组的血清AChR-Ab浓度分别为0.786±0.237ng·mL~(-1)、0.298±0.113ng·mL~(-1),差异有统计学意义(均P0.05),血清Mu SK-Ab浓度分别为0.322±0.128ng·mL~(-1)、0.281±0.123ng·mL~(-1),差异无统计学意义(均P0.05);(2)血清AChR-Ab和MuSK-Ab在不同临床分型MG患者中的浓度均没有明显差异(均P0.05);(3)治疗后肌力绝对评分和血清AChR-Ab浓度均显著下降(均P0.05),而血清MuSK-Ab浓度在治疗前后没有显著变化(P0.05),并且治疗后的血清AChR-Ab浓度和治疗后的肌无力绝对评分呈正相关(r=0.797,P0.05)。结论血清AChR-Ab和MuSK-Ab的浓度与MG的临床分型无明显相关性,血清AChR-Ab对疾病的转归有一定的预示作用。     

不同抗体分型的重症肌无力特点分析

目的探讨不同抗体分型重症肌无力(myasthenia gravis,MG)患者的临床特点。方法收集114例临床确诊MG患者的临床资料。使用酶联免疫吸附法检测患者血清中的抗乙酰胆碱受体抗体(acetylcholine receptors antibody,AChR Ab)、肌肉特异性激酶抗体(antibody to muscle-specific kinase, MuSK Ab)、抗联接蛋白抗体(titin antibody, Titin Ab)和抗兰尼碱受体抗体(ryanodine receptors antibody, RyR Ab)。根据致病抗体AChR Ab、MuSK Ab的表达情况将患者分为AChR Ab阳性组,MuSK Ab阳性组和双抗体阴性(double serum negative, DSN)组,对3组的临床表现、实验室检查和胸腺病理进行分析;根据疾病严重程度相关抗体Titin Ab和RyR Ab的表达情况,分为Titin Ab阳性组和Titin Ab阴性组,RyR Ab阳性组和RyR Ab阴性组,并比较两种抗体分型的阳性组和阴性组间的临床表现、实验室检查和胸腺病理,以及Titin Ab阳性MG和RyR Ab阳性MG组的受累肌群。结果 AChR Ab(+)组76例(66.7%)、MuSK Ab(+)组9例(7.9%)、DSN组29例(25.4%),三组间甲状腺功能(100.0%vs. 33.3%vs. 58.6%)和自身抗体异常发生率(63.2%vs. 33.3%vs. 37.9%)以及胸腺病理患者比例(胸腺增生/萎缩48.0%vs.33.3%vs. 71.4%,胸腺瘤52.0%vs.66.7%vs.28.6%)差异均有统计学意义(P0.05);组间两两比较,仅AChR Ab(+)MG甲状腺功能异常率高于MuSK Ab(+)MG及DSN MG组(均P0.05),余差异均无统计学意义(均P0.05)。三组间不同临床分型(眼肌型与全身型)患者比例、球部症状和肌无力危象发生率差异均无统计学意义(均P0.05)。Titin Ab阳性组甲状腺功能异常率高于Titin Ab阴性组(100.0%vs. 33.3%,P0.05),自身抗体、胸腺病理、球部症状及肌无力危象患者比例差异两组间无统计学意义(均P0.05)。RyR Ab阳性组胸腺瘤、肌无力危象发生率高于阴性组,自身抗体异常率低于阴性组(分别为66.7%vs. 32.1%、55.3%vs. 21.4%、30.0%vs. 57.1%,均P0.05);甲状腺功能异常率和球部症状患者比例两组间差异无统计学意义(P0.05)。Titin Ab(+)MG较RyR Ab (+)MG患者易出现眼外肌在受累(100.0%vs. 60.0%,P0.05),而RyR Ab (+)MG更易出现球部肌肉(66.7%vs.25.0%,P0.05)及呼吸肌受累(53.3%vs 20.8%,P0.05)。结论 AChR Ab、MuSK Ab不同表达情况下,AChR Ab(+)MG易出现甲状腺功能异常,MuSK Ab(+)MG易合并胸腺瘤,而双阴性患者病情温和,胸腺病理多呈良性。RyR Ab(+)MG与Titin Ab(+)MG比较,RyR Ab(+)MG易有球部症状和呼吸肌受累,RyR Ab阳性MG病情更严重。     

不同重症肌无力患者人群血清抗体检测的临床意义

目的探讨不同重症肌无力患者人群中血清抗乙酰胆碱受体(AChR)抗体、抗连接素(Titin)抗体、抗Rvanodine受体(RyR)抗体和抗肌肉特异性受体酪氨酸激酶(MuSK)抗体的表达特点。方法采用酶联免疫吸附法(ELISA)检测65例重症肌无力患者血清AChR抗体、Titin抗体、RyR抗体和MuSK抗体水平,分析不同病程、胸腺情况以及美国重症肌无力协会(MGFA)临床分型MG患者各种抗体表达情况。结果患者血清AChR抗体、Titin抗体、RyR抗体和MuSK抗体阳性率分别为47.70%、43.10%、46.20%和12.30%。不同病程MG患者中AChR抗体水平差异无统计学意义(P=0.305),Titin抗体、RyR抗体和MuSK抗体水平差异有统计学意义(分别P=0.000,P=0.001,P=0.004);胸腺瘤组和胸腺正常组间4种血清抗体阳性率和抗体水平差异无统计学意义(均P0.05);MGFA临床分型各亚组中AChR抗体水平差异有统计学意义(P=0.012),重度全身型(Ⅳ型)AChR抗体水平远高于眼肌型(Ⅰ型)和轻度全身型(Ⅱ型)(均P0.05),而Titin抗体、RyR抗体和MuSK抗体水平差异无统计学意义(均P0.05)。结论 MG患者中Titin抗体、RyR抗体和MuSK抗体水平与病程有关,AChR抗体水平病情严重程度有关。     

全身型重症肌无力的临床和电生理特点分析

目的 探讨不同亚型全身型重症肌无力(Myasthenia gravis,MG)临床和电生理特点之间的区别,并分析电生理结果与疾病严重程度的相关性。方法 回顾性分析2016年7月至2020年7月在香港大学深圳医院神经内科就诊或住院的101例全身型MG患者的临床和电生理资料,按照其临床特点,分为早发乙酰胆碱受体(Acetylcholine receptor,AchR)抗体阳性全身型MG组(52例)、晚发AchR抗体阳性全身型MG组(23例)、胸腺瘤型MG组(22例)和肌肉特异性受体酪氨酸激酶(Muscle-specific receptor tyrosine kinase,MuSK)抗体阳性型MG组(4例),比较不同亚组MG患者的人口学特点、重症肌无力评分(Quantitative Myasthenia Gravis score,QMGs)、美国重症肌无力协会(MG Foundation America,MGFA)临床分级、重复神经电刺激(Repetitive nerve stimulation,RNS)结果,分析RNS结果与QMGs之间的相关性。结果 非MuSK抗体阳性全身型MG中,早发AchR抗体阳性全身型MG女性患者所占比率最高(71. 2%vs 65. 2%vs 36. 4%,P 0. 05),胸腺瘤型MG呼吸肌/球部肌肉受累为主患者所占比率最高(28. 8%vs 43. 5%vs 63. 6%,P 0. 05),早发AchR抗体阳性全身型MG被检肌肉RNS均为阳性患者所占比率最高(44. 2%vs 17. 4%vs 22. 7%,P 0. 05),QMGs与RNS最大波幅下降比率、RNS总波幅下降比率呈明显正相关关系(P 0. 01); MuSK抗体阳性全身型MG,其中重度受累患者所占比率、呼吸肌/球部肌肉受累为主患者所占比率均较高(均为75%),而被检肌肉RNS均为阳性患者所占比率较低(0%)。结论 不同亚型全身型MG的人群分布、性别占比、主要受累肌肉、受累肌肉受累程度及分布范围均具有显著的差别,这在全身型MG的分型诊断方面具有很大的参考价值;其RNS结果与疾病严重程度具有高度相关性,提示RNS在全身型MG的病情评估方面具有重要临床价值。     

Osserman V型重症肌无力5例临床分析及文献复习

目的对Osserman V型重症肌无力(MG)的临床特点及诊治体会进行回顾性总结分析,以提高对此少见疾病中少见类型的认识。方法对作者医院2004-11—2006-11期间收治的491例MG患者中所有OssermanV型患者的临床特点和血清学检测结果进行回顾性分析。结果491例MG患者中5例为Osserman V型,在病程中先后出现不同程度舌肌、颞肌或上肢远端小肌肉萎缩。其中4例肌电图检查重频电刺激低频递减,血清乙酰胆碱受体抗体(AChR-Ab)阳性,另1例抗骨骼肌特异性受体酪氨酸激酶抗体(MuSK-Ab)阳性。5例患者均对溴吡斯的明对症治疗反应差,但对糖皮质激素和静脉丙种球蛋白(IVIG)治疗反应较好。半年后随访肌肉萎缩有不同程度改善。结论Osserman V型MG很罕见,肌肉萎缩的发病机制尚不清楚,部分患者可能与MuSK-Ab有关。血清学检测有助于对Osserman V型MG确诊、避免误诊及改善预后。     

联合检测重症肌无力的三种血清抗体诊断

目的对重症肌无力(MG)患者的乙酰胆碱受体抗体(Ach R-Ab)、连接素抗体(Titin-Ab)和肌肉特异性酪氨酸激酶抗体(Mu SK-Ab)评价该3种血清抗体联合检测对MG的诊断价值。方法选择MG组患者112例、其他疾病对照组53例和健康对照组56例,采用放射免疫法测定Ach R-Ab,ELISA法分别测定Titin-Ab和Mu SK-Ab,并对结果进行分析。结果 MG患者Ach R-Ab、Titin-Ab和Mu SK-Ab的阳性率分别为68.8%、54.5%和5.4%,明显高于对照组(P<0.05)。三种抗体联合检测,其敏感性和特异性分别为91.1%和89%。其中,Mu SK-Ab仅见于Ach R-Ab阴性的MG患者,主要表现为延髓肌受累,病情较重且不伴有胸腺病变。Titin-Ab多见于MG合并胸腺瘤(MGT)患者,其MGT组的阳性率高于未合并胸腺瘤的MG(NTMG)患者(P<0.05)。将MG患者根据Osserman评分进行分型,Titin-Ab阳性率与病情严重程度有关(P<0.05)。结论 Ach R-Ab、Titin-Ab、Mu SK-Ab三种抗体是MG的诊断性标记抗体,联合检测可以避免因单项检测出现的漏诊和误诊,此外,Titin-Ab对MG合并胸腺瘤具有显著相关性,还可作为判断MG病情严重程度的指标。     

Osserman Ⅴ型重症肌无力5例临床分析及文献复习

目的 对Osserman Ⅴ型重症肌无力（MG）的临床特点及诊治体会进行回顾性总结分析，以提高对此少见疾病中少见类型的认识。方法 对作者医院2004-11-2006-11期间收治的491例MG患者中所有Osserman Ⅴ型患者的临床特点和血清学检测结果进行回顾性分析。结果 491例MG患者中5例为Osserman Ⅴ型，在病程中先后出现不同程度舌肌、颞肌或上肢远端小肌肉萎缩。其中4例肌电图检查重频电刺激低频递减，血清乙酰胆碱受体抗体（AChR-Ab）阳性，另1例抗骨骼肌特异性受体酪氨酸激酶抗体（MuSK-Ab）阳性。5例患者均对溴吡斯的明对症治疗反应差，但对糖皮质激素和静脉丙种球蛋白（IVIG）治疗反应较好。半年后随访肌肉萎缩有不同程度改善。结论 Osserman Ⅴ型MG很罕见，肌肉萎缩的发病机制尚不清楚，部分患者可能与MuSK-Ab有关。血清学检测有助于对Osserman Ⅴ型MG确诊、避免误诊及改善预后。     

Tfh细胞在重症肌无力患者胸腺中的表达及作用机制

目的探讨Tfh细胞在重症肌无力(MG)患者胸腺中乙酰胆碱受体抗体(AChR-Ab)产生中的作用及机制。方法收集28例MG患者、9例无胸腺异常的先天性心脏病患者和9例未合并MG的胸腺增生患者。采用流式细胞术检测胸腺中滤泡辅助T(Tfh)细胞和B细胞的比例,分别通过RT-PCR和Western-Blotting技术检测胸腺中Th1、Th2和Tfh细胞特异性转录因子T-bet、GATA-3、Bcl-6 mRNA水平和细胞因子干扰素γ(IFN-γ)、白细胞介素4(IL-4)、IL-21表达;采用HE和多标免疫荧光染色观察胸腺中生发中心以及Tfh细胞和B细胞之间的组织结构关系;采用放射免疫沉淀技术(RIA)检测MG患者胸腺和血清中AChR-Ab滴度;免疫磁珠提取胸腺中Tfh细胞和B细胞共培养,检测培养上清中IL-21和AChR-Ab滴度水平。结果 MG患者胸腺中Tfh细胞和B细胞比例及Tfh细胞相关转录因子Bcl-6mRNA水平和细胞因子IL-21表达均较对照组增高(P0.01);MG患者胸腺中抗AChR-Ab滴度较对照组增高(P0.01);MG患者胸腺中存在异位生发中心,且在生发中心内Tfh细胞和B细胞存在共定位;MG患者胸腺Tfh和B细胞共培养上清中IL-21和AChR-Ab水平较对照组增多(P0.01),且AChR-Ab产生可以被IL-21中和抗体阻断。结论 Tfh细胞通过在MG患者胸腺中辅助B细胞形成异位生发中心及促进抗AChR-Ab产生参与MG的发生和发展。     

斜方肌肌电图在肌萎缩侧索硬化早期诊断中的应用

目的 探讨斜方肌肌电图在检测肌萎缩侧索硬化(ALS)延髓部下运动神经元损害中的价值.方法 对100例ALS患者、80例颈椎病患者和100名健康志愿者进行斜方肌肌电图检测,AKS组和颈椎病组同时进行胸锁乳突肌、第一骨间肌、腹直肌、胫前肌肌电图检测,比较3组肌电图的差异.颈椎病组中43例患者进行手术前后斜方肌肌电图比较.结果 ALS组中,病程≤8个月的患者斜方肌肌电图自发电位的出现率高于病程>8个月者[分别为21/30(70%)和28/70(40%),X~2=7.56,P=0.004];斜方肌肌电图与胸锁乳突肌肌电图异常率比较差异无统计学意义.ALS组[波幅(1086.9±152.6)μV,时限(17.2±6.5)ms,多相波23.6%±3.4%]与对照组[波幅(606.7±82.7)μV,时限(11.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±2.0)ms,多相波13.5%±2.4%]间运动单位动作电位(MUAP)各参数比较,差异有统计学意义(q=8.32、4.25、4.23,均P<0.01).颈椎病患者手术前后斜方肌肌电图MUAP各参数无明显变化,5例术后发现少量自发电位.结论 斜方肌肌电图可作为检测ALS延髓部下运动神经元损害的辅助手段,特别是早期可见自发电位增多. 1.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±2.0)ms,多相波13.5%±2.4%]间运动单位动作电位(MUAP)各参数比较,差异有统计学意义(q=8.32、4.25、4.23,均P< .01).颈椎病患者手术前后斜方肌肌电图MUAP各参数无明显变化,5例术后发现少量自发电位.结论 斜方肌肌电图可作为检测ALS延髓部下运动神经元损害的辅助手段,特别是早期可见自发电位增多. 1.6±1.8)ms,多相波12.8%±2.2%;q=9.27、4.57、4.12,均P<0.01]、ALS组与颈椎病组[术前,波幅(615.7±90.3)μV,时限(12.1±     

A forty-five-years-old woman suffered from Sj?gren syndrome with progressive tetraparesis]

We herein report the finding of a 45-year-old woman suffered from Sj?gren syndrome with progressive tetraparesis, who later developed systemic muscle atrophy and respiratory failure with a one-year clinical history. Neurological examinations revealed progressive tetraparesis with absent deep tendon reflexes, whereas no upper motor neuron signs were observed. The motor and sensory nerve conduction velocity and sensory nerve action potential (SNAP) were both completely normal, but the prolongation of distal motor latency in the median nerve and a decrease in the compound muscle action potential (CMAP) amplitude were observed. We ascertained that a spontaneous discharge was detected in her upper and lower limbs on electromyography (EMG). Her neurological findings as well as the EMG findings closely correlated with those of motor neuron disease; however, she showed a motor paralytic bladder and also demonstrated a serum antibody reaction with 50 kDa spinal cord protein of the rat. A lumbar MR image showed an increased signal intensity of the cauda equina on a gadolinium-enhanced T1 weighted image. We consider the immune-mediated impairment of the motor nerve associated with Sj?gren syndrome to be the cardinal pathogenesis of the present patient, even though treatment with oral corticosteroids did not ameliorate her symptoms.     

卒中后肩痛周围神经电生理学研究

目的 通过对卒中后肩痛患者上肢进行神经传导速度检查及针极肌电图检查，观察卒中后肩痛患者周围神经电生理指标的变化。方法 选择符合入选标准的卒中住院患者40例，根据数字疼痛评分法（Numerical Pain Rating Scale，NPRS）分为肩痛组（26例）与无肩痛组（14例）。分别进行双侧上肢神经传导速度检查和针极肌电图（electromyography，EMG）检查。结果 肩痛组患侧腋神经、肌皮神经、正中神经复合肌肉动作电位（compound muscle action potential，CMAP）波幅较无肩痛组患侧降低，差异有显著性（P=0.000，0.001，0.000）；无肩痛组患侧尺神经CMAP波幅较同组健侧降低，差异有显著性（P=0.000）；肩痛组患侧尺神经感觉神经动作电位（sensory nerve action potential，SNAP）波幅较无肩痛组患侧降低，差异有显著性（P=0.000）。三角肌、肱二头肌自发电位出现率，肩痛组较无肩痛组增高，差异具有显著性（P=0.044，0.044）。结论 卒中后肩痛患者伴有上肢周围神经的损伤，且肩痛的发生可能与运动神经损伤有关。     

Clinical utility of trapezius muscle studies in the evaluation of amyotrophic lateral sclerosis.

Needle electromyography (EMG) and determining the motor evoked potential (MEP) of the genioglossus (tongue) are difficult to perform in evaluation of the craniobulbar region in patients with amyotrophic lateral sclerosis (ALS). Needle EMG and MEP determination in the upper trapezius were carried out in 17 consecutive ALS patients. The needle EMG parameters recorded included abnormal spontaneous activity and motor unit action potential morphology. An upper motor neuron lesion was presumed when either response to cortical stimulation was absent, or the central conduction time was delayed (>mean + 2 SD). Of the 12 patients with limb-onset ALS, using needle EMG, 11 were found to have abnormalities in the upper trapezius, and only five in the tongue. Three of the six patients with isolated limb involvement had abnormal MEP findings. In conclusion, electrophysiological studies of the upper trapezius are useful in ALS patients without bulbar symptoms.     

Muscular fatigue and decremental response to repetitive nerve stimulation in X-linked spinobulbar muscular atrophy

Background and Purpose:  We report decremental responses to repetitive nerve stimulation (RNS) in 11 patients diagnosed with X-linked spinobulbar muscular atrophy (X-SBMA).
Methods:  The compound muscle action potential (CMAP) of the right abductor digiti minimi (ADM) and trapezius (TZ) in response to a 3-Hz stimulation of the ulnar nerve at the wrist and accessory nerve at the neck were recorded by surface electrodes.
Results:  A decremental response to RNS was observed in 90.9% of the TZ muscle and 27.2% in the ADM muscle of patients with X-SBMA.
Conclusion:  These electrophysiological features of X-SBMA are considered to be useful for diagnosis of X-SBMA. Furthermore, the waning phenomena that mostly appeared in the TZ muscle and increment of CMAP in RNS after the exercise also suggest a unique manifestation in X-SBMA.     

Evaluation of neuromuscular junction disorders in the electromyography laboratory

Neuromuscular junction (NMJ) disorders may be demonstrated using repetitive nerve stimulation (RNS) testing and single-fiber electromyography (SFEMG). RNS testing with low frequency stimulation reduces the safety factor of neuromuscular transmission (NMT) and may elicit decrementing compound muscle action potential (CMAP) responses. Exercise or tetanic nerve stimulation may potentiate acetylcholine release in presynaptic NMT disorders with CMAP facilitation. SFEMG is a selective recording technique assessing MFAPs within the same motor unit. Jitter is increased in NMJ disorders, and is the temporal variability between these MFAPs. Impulse blocking reflects failure of NMT. RNS and SFEMG findings in NMJ disorders are reviewed.     

多点刺激法进行运动单位数目估计及其正常值探讨

目的 采用多点刺激法进行运动单位数目估计(MUNE)检测,并探寻其国人正常值.方法 对知情同意的80名健康志愿者进行多点刺激法MUNE:刺激电极分别于腕、腕上6 cm、肘、肘上6 cm共4点刺激正中神经或尺神经,以超强刺激诱发最大波幅M波,然后从0 mA认刺激开始逐渐增加刺激强度,直到出现可辨认的低波幅的单个运动单位电位,稳定后保存,每次记录3个递增的M波,再逐渐增大刺激强度,共获取12个递增的M波.重复做2次.结果 健康志愿者拇短展肌MUNE结果为230.0±35.7,小指展肌242.5±30.2.多点刺激法2次测验所得的MUNE的相关系数为0.88～0.91,变异系数13.20%～15.24%,差异无统计学意义.结论 多点刺激法能测定运动单位数目,可重复性良好.     

Pathologic classification of a late-onset peripheral neuropathy in a spontaneous Labrador retriever dog model

Late-onset peripheral neuropathy (LPN) is a heritable canine neuropathy commonly found in Labrador retrievers and is characterized by laryngeal paralysis and pelvic limb paresis. Our objective was to establish canine LPN as a model for human hereditary peripheral neuropathy by classifying it as either an axonopathy or myelinopathy and evaluating length-dependent degeneration. We conducted a motor nerve conduction study of the sciatic and ulnar nerves, electromyography (EMG) of appendicular and epaxial musculature, and histologic analysis of sciatic and recurrent laryngeal nerves in LPN-affected and control dogs. LPN-affected dogs exhibited significant decreases in compound muscle action potential (CMAP) amplitude, CMAP area, and pelvic limb latencies. However, no differences were found in motor nerve conduction velocity, residual latencies, or CMAP duration. Distal limb musculature showed greater EMG changes in LPN-affected dogs. Histologically, LPN-affected dogs exhibited a reduction in the number of large-diameter axons, especially in distal nerve regions. In conclusion, LPN in Labrador retrievers is a common, spontaneous, length-dependent peripheral axonopathy that is a novel animal model of age-related peripheral neuropathy that could be used for fundamental research and clinical trials.     

神经电生理检查在肯尼迪病和进行性肌萎缩鉴别诊断中的价值

目的 通过观察神经电生理改变特点,协助鉴别肯尼迪病(KD)和进行性肌萎缩(PMA).方法 收集13例KD和12例PMA患者,对两组患者运动和感觉神经传导、常规肌电图、单纤维肌电图结果进行比较.结果 同侧肢体正中神经(拇短展肌记录)和尺神经(小指展肌记录)远端复合肌肉动作电位(CMAP)波幅比值在KD组和PMA组分别为1.11±0.40、0.57±0.29(t=4.760,P=0.000).6例KD患者感觉神经传导测定显示波幅下降,传导速度正常.PMA患者感觉传导测定均正常.单纤维肌电图检测可见,KD组和PMA组平均颤抖(jitter)分别为(56.3±24.2)、(97.1±35.3)μs(t=2.696,P=0.015),阻滞百分比中位数(M_(50))分别为0、32.5%(Z=3.168,P=0.002).两组肌电图检查均表现为广泛神经源性损害.结论 KD和PMA进行鉴别时,如运动传导显示正中神经CMAP波幅大于尺神经波幅,感觉神经传导波幅下降,单纤维肌电图颤抖相对正常,可以支持KD的诊断.     

Interrelationship among nerve conduction velocity, amplitudes of compound muscle and compound nerve action potentials in diabetic neuropathy]

In order to clarify the relationship among amplitudes of compound nerve action potential (CNAP), compound muscle action potential (CMAP) and nerve conduction velocity parameters, data of nerve conduction studies were analyzed in 102 patients with diabetes mellitus. In motor conduction studies CMAP amplitudes after stimulations at the distal nerve trunk, and the polyneuropathy index (PNI), a mean percentage of normal for 12 indices from 4 nerves concerning to the velocity or long distance latency, were evaluated. CNAP was recorded in the median and ulnar nerves from an intrafascicularly inserted microelectrode at the elbow after wrist stimulation. CMAP amplitudes were high in the median and ulnar nerves, and were reduced in the tibial and peroneal nerves. A close relationship was found between PNI and CNAP amplitudes. Among CMAP amplitude parameters tibial nerve, not median or ulnar nerves, had a good correlation with PNI and CNAP amplitude. Along with the progression of diabetic neuropathy, neuropathic signs or symptoms become conspicuous, and nerve conduction velocity drops as is expressed by the PNI level, which reflects the change in nerve conduction velocity in the upper and lower limbs. At the same time CNAP amplitude or CMAP amplitude in the tibial nerve decreases, but in nerves of the upper limb CMAP amplitude doesn't always decrease. So, tibial nerve is best among CMAP amplitude parameters in evaluating the degree of diabetic neuropathy. It is necessary to judge the degree of diabetic neuropathy after due consideration of these facts.