运动神经元病的神经电生理学研究
发布时间：2018-07-28 09:52
【摘要】：第一部分F波在肌萎缩侧索硬化和肯尼迪病诊断和鉴别诊断的意义研究背景：肌萎缩侧索硬化(amyotrophic lateral sclerosis, ALS)是一种选择性累及大脑皮质脑干和脊髓运动神经元的快速进展性神经系统变性疾病,主要临床表现包括进行性肌肉无力萎缩、延髓麻痹和锥体束征。目前ALS病因尚未明确且缺乏有效的治疗办法,患者预后不良。ALS诊断需要上、下运动神经元同时受累的证据,目前尚缺乏特异性的生物学标记物。ALS病情进展隐匿,临床表现具有异质性,早期容易误诊漏诊,例如一些ALS患者上运动神经元受累体征出现于病程晚期或无上运动神经元受累体征,临床上有时难以与肯尼迪病(Kennedy disease, KD)鉴别。既往研究显示ALS和KD在感觉神经传导、针级肌电图、皮层兴奋性测试、血清肌酸激酶水平和临床表现上存在差异。至今缺乏关于ALS和KD的F波特点比较的研究。目的：本研究拟通过比较ALS患者和正常受试者尺神经F波参数差异并分析ALS患者上、下运动神经元功能异常对F波参数的影响来探讨ALS中F波的特点；并试图通过分析和比较ALS患者和KD患者正中神经、尺神经、胫神经和腓神经F波特点的差异,探讨F波在ALS和KD诊断和鉴别诊断中的价值。方法：连续入组自2013年9月至2014年7月期间就诊于我院神经科门诊的82：名ALS患者,所有患者建立病案数据库,记录患者姓名、性别、年龄、病程、发病部位、症状、体征及所检测肌肉的MRC肌力分级等。50名年龄和性别匹配的健康志愿者作为正常对照组。以受试者尺神经为研究对象,对ALS患者和正常对照组行尺神经运动神经传导和F波检测。比较ALS患者与正常对照组尺神经F波参数差异,并使用Logistic回归分析评估ALS患者尺神经F波参数与上肢锥体束征和小指展肌MRC肌力分级(MRC5级、MRC4级和MRC3级)的关系。连续入组自2013年9月至2014年12月就诊于我院神经科门诊的37名以下运动神经元受累为主的男性ALS患者和同时期就诊于我院神经科门诊的32名KD患者；30名男性健康志愿者作为正常对照组。比较ALS患者、KD患者和正常对照组双侧正中神经、尺神经、胫神经和腓神经F波参数差异。结果：ALS患者与正常对照组尺神经F波参数的比较研究发现,与正常对照组相比,ALS患者尺神经平均(P=0.040)和最大(P)0.001)F波波幅、平均(P0.001)和最大(P0.001)F/M波幅比、重复神经元指数(P0.001)、重复F波指数(P0.001)和巨大F波出现率(P0.001)增高,F波时间离散度(P0.001)、最短F波潜伏期(P0.001)延长,F波时限(P=0.004)缩短,F波出现率(P0.001)降低,差异均有统计学意义。ALS患者尺神经F波最短潜伏期(P0.001)和F波出现率(P0.001)与小指展肌MRC肌力分级显著相关；F波平均(P0.001)和最大波幅(P=0.002)与上肢锥体束征显著相关；平均(MRC,P0.001;锥体束征,P0.001)和最大(MRC,P=0.001;锥体束征,P=0.002)F／M波幅比、F波时限(MRC,P0.001;锥体束征,P=0.047)、重复神经元指数(MRC,P0.001;锥体束征,P=0.009)和重复F波指数(MRC,P0.001；锥体束征,P=0.002)与小指展肌MRC肌力分级及上肢锥体束征显著相关。ALS患者与KD患者F波参数的比较研究发现,与ALS患者和正常对照组相比,KD患者正中神经、尺神经、胫神经和腓神经最大F波波幅、巨大F波出现率和出现巨大F波受试者比率均增高,差异有统计学意义。与ALS患者相比,巨大F波可出现于KD患者多条神经且倾向于对称分布。在所测F波参数中,出现巨大F波神经数量是区分ALS和KD最有效参数,曲线下面积为0.908(95%可信区间：0.835-0.982)。受试者≥3条神经出现巨大F波区分ALS和KD敏感性和特异性较高,分别为85%和81%。ALS患者(r=0.107,P=0.529)和KD患者(r=0.162,P=0.418)巨大F波总出现率与病程相关性无统计学意义。结论：F波参数受上、下运动神经元功能影响,可反映脊髓运动神经元池的完整性和兴奋性。F波有助于鉴别ALS和KD,不受患者病程影响。对于下运动神经元受累为主的男性ALS患者,如果巨大F波出现率显著增加,尤其是在正中神经和腓神经,以及多条神经记录到巨大F波(三3条)或巨大F波对称分布于左右侧相同神经需考虑KD诊断,建议行KD基因检测。第二部分肌萎缩侧索硬化分裂手现象的电生理学研究和机制探讨研究背景：肌萎缩侧索硬化(amyotrophic lateral sclerosis, ALS)分裂手现象是指拇短展肌(abductor pollicis brevis, APB)和第一骨间肌受累重于小指展肌(abductor digiti minimi, ADM),是ALS的特异性临床表现。既往研究显示中枢机制和周围轴索机制可能参与了ALS分裂手现象的发生,尚无研究显示支配APB和ADM的脊髓运动神经元池功能异常是否符合ALS分裂手现象。目的：本研究拟通过F波检测探讨ALS中支配APB和ADM的脊髓运动神经元池功能异常是否符合ALS分裂手现象,参与ALS分裂手现象的发生。方法：研究纳入2013年9月至2014年3月在北京协和医院神经科门诊就诊的40名ALS患者,根据ALS患者手部肌肉受累情况分为两组,一组为手部肌肉萎缩无力明显的ALS患者,一组为手部肌肉无明显萎缩无力的ALS患者。20名健康志愿者作为正常对照组。对受试者正中神经和尺神经进行运动神经传导检测和F波测定。比较APB和ADM记录的F波参数。分析的F波参数包括F波潜伏期、F波出现率、F波波幅F/M波幅比、重复神经元指数和重复F波指数。结果：正常受试者APB和ADM记录的F波参数相比,F波出现率(P0.05)、重复神经元指数(P0.001)和重复F波指数(P0.001)存在差异,有统计学意义。ALS患者无明显肌肉无力萎缩的上肢APB记录的F波与正常对照组APB记录的F波相比,F波出现率显著降低(P0.001)、重复神经元指数(P0.001)和重复F波指数(P0.001)显著增高,差异有统计学意义,而ALS患者ADM记录的F波参数相对正常。ALS患者出现肌肉萎缩无力临床表现的上肢APB与ADM记录的F波参数比较,平均F波波幅(P0.05)、F/M波幅比(P0.05)、F波出现率(P0.001)、重复神经元指数(P0.05)和重复F波指数(P0.05)存在差异,有统计学意义。APB和ADM脊髓运动神经元池功能异常的电生理学差异是ALS的特异性表现。APB记录的F波出现率(P=0.002)、重复神经元指数(P0.001)和重复F波指数(P0.001)有助于区分ALS患者和正常受试者,诊断价值优于ADM/APB复合肌肉动作电位波幅比。结论：正常受试者和ALS患者APB和ADM记录的F波参数差异符合分裂手现象,可能脊髓运动神经元池功能异常参与了ALS分裂手现象的发生。F波有助于发现ALS患者脊髓前角细胞的亚临床病变,有助于ALS的诊断和鉴别诊断。第三部分肌萎缩侧索硬化、肯尼迪病、远端肌萎缩型颈椎病和平山病电生理特点的比较研究背景：肌萎缩侧索硬化(amyotrophic lateral sclerosis, ALS)是快速进展的神经系统变性病,临床诊断需要上、下运动神经元同时受累的证据。但是在疾病早期,肌肉无力萎缩可能局限于ALS患者手部小肌肉,为了早期明确诊断需注意排除有类似临床表现的一些疾病如远端肌萎缩型颈椎病(cervical spondylotic amyotrophy, CSA)、平山病(Hirayama disease, HD)和肯尼迪病(Kennedy disease, KD)。目的：研究旨在探讨ALS、远端型CSA、 HD和KD患者神经传导检查的特点以及手部肌肉受累的差异。方法：回顾性分析北京协和医院肌电图室2000年至2014年符合ALS诊断标准的患者(200例)、符合远端型CSA诊断标准的患者(95例)、符合HD诊断标准的患者(88例)和符合KD诊断标准的患者(43例)的病历资料,收集患者临床资料和上肢神经传导检查结果。150名健康志愿者作为正常对照组。结果：ALS患者小指展肌／拇短展肌(abductor digiti minimi/abductor pollicis brevis, ADM/APB)复合肌肉动作电位(compound muscle action potential, CMAP)波幅比(3.52±0.60,P0.001)高于正常对照组(1.00±0.24),差异有统计学意义。远端型CSA患者(0.93±0.77,P0.001)和HD患者(0.63±0.52,P0.001)ADM/APB CMAP波幅比低于正常对照组,差异有统计学意义。远端型CSA患者APB CMAP波幅(9.91±5.05mV,P=0.005)低于HD患者(12.07±4.88mV),差异有统计学意义。HD患者ADM/APB CMAP波幅比低于远端型CSA,差异有统计学意义(P0.001)。KD患者ADM/APB CMAP波幅比(1.06±0.40,P=0.862)与正常对照组相比差异无统计学意义。APB CMAP消失或异常增高的ADM/APB CMAP波幅比(≥4.5)仅见于ALS患者。ALS患者、远端型CSA和HD患者正中神经和尺神经的感觉神经动作电位波幅和感觉神经传导速度均在正常范围。KD患者感觉神经动作电位波幅异常率为81%,感觉神经传导速度异常率为9.3%。结论：ALS患者、远端型CSA患者、HD患者和KD患者手部小肌肉萎缩的差异反映了疾病不同的病理生理机制。神经传导检查特别是ADM/APB CMAP波幅比有助于诊断和鉴别诊断ALS及与ALS有类似临床表现的疾病。
[Abstract]:Part 1 the significance of F wave in the diagnosis and differential diagnosis of amyotrophic lateral sclerosis and Kennedy's disease: amyotrophic lateral sclerosis (amyotrophic lateral sclerosis, ALS) is a rapid progressive neurodegenerative disease involving the cerebral cortex and spinal motor neurons of the cerebral cortex. The main clinical manifestations include progressive disease. Weak muscle atrophy, medulla paralysis and pyramidal tract sign. The etiology of ALS is not yet clear and lack of effective treatment. The patient has poor prognosis,.ALS diagnosis needs, the evidence of the simultaneous involvement of the lower motor neurons, the lack of specific biological markers,.ALS, is inactive, the clinical manifestation is heterogeneous, and early misdiagnosis. Missed diagnosis, for example, the signs of motor neuron involvement in some ALS patients appear at the late stage of the course of the disease or the sign of no upper motor neuron, and it is sometimes difficult to identify with Kennedy's disease (Kennedy disease, KD). Previous studies showed that ALS and KD were in sensory nerve conduction, needle level electromyography, cortical excitability test, serum creatine kinase level and clinical level. There is a difference in performance. So far, there is a lack of research on the comparison of the characteristics of the F wave of ALS and KD. Objective: To explore the characteristics of F wave in ALS by comparing the differences in the F wave parameters of the ulnar nerve in ALS patients and normal subjects and analyzing the effects of the dysfunction of the lower motor neurons on the F wave parameters in the ALS patients, and to try to analyze and compare the AL of the ALS. The difference between the median nerve, the ulnar nerve, the tibial nerve and the F wave of the peroneal nerve in S patients and KD patients, and to explore the value of the F wave in the diagnosis and differential diagnosis of ALS and KD. Methods: 82 consecutive patients from September 2013 to July 2014 were enrolled in the neurology department of our hospital, named ALS patients, the patient established a database of medical records, and recorded the name of the patient. Different age, age, course of disease, location, symptoms, signs, and MRC muscular strength classification of.50 were used as normal control group. The ulnar nerve conduction and F wave test were performed on the ulnar nerve in the subjects of the subjects and the ALS patients and the normal control group. Compared with the normal control group, the patients with ALS were compared with the normal control group. The parameter difference of nerve F wave and Logistic regression analysis were used to evaluate the relationship between the F wave parameters of the ulnar nerve in ALS patients and the pyramidal tract sign of the upper limb and the MRC muscle strength classification of the small finger abductor muscle (MRC5, MRC4 and MRC3). From September 2013 to December 2014, the group of 37 men with motor neuron involvement in the Department of Neurology Department of our hospital were diagnosed as the male ALS. 32 KD patients and 30 male healthy volunteers were treated in the Department of Neurology in our hospital as a normal control group. The F wave parameters of the median nerve, ulnar nerve, tibial nerve and peroneal nerve of the patients with ALS, KD and normal control were compared. Results: the comparison of the F wave parameters of the ulnar nerve between the ALS patients and the normal control group was compared with the normal control group. Compared with the normal control group, the average (P=0.040) and the maximum (P) 0.001) F wave amplitude of the ALS patients, the average (P0.001) and the maximum (P0.001) F/M amplitude ratio, the repeat neuron index (P0.001), the repetition of the F wave index (P0.001) and the large F wave occurrence rate (P0.001) increased. P=0.004) shortened and F wave occurrence rate (P0.001) decreased. The difference was statistically significant in the shortest latency (P0.001) and F wave occurrence rate (P0.001) of the ulnar nerve in.ALS patients and the MRC muscle strength classification of the small finger abductor muscle; F wave mean (P0.001) and maximum amplitude (P=0.002) were significantly related to the pyramidal tract of the upper limb. 1) and the maximum (MRC, P=0.001; pyramidal sign, P=0.002) F / M amplitude ratio, F wave time limit (MRC, P0.001; pyramidal tract sign, P=0.047), repeat neuron index (MRC, P0.001; pyramidal tract sign, P=0.009) and repeated exponential wave index (pyramidal tract sign) Compared with the ALS patients and the normal control group, the maximum F wave amplitude of the median nerve, the ulnar nerve, the tibial nerve and the peroneal nerve in the patients with KD was compared with that of the normal control group. The rate of the huge F wave and the ratio of the huge F wave subjects increased, and the difference was statistically significant. Compared with the ALS patients, the huge F wave could appear in the multiple nerves of KD patients and tend to be inclined to the KD patients. In the measured F wave parameters, the number of large F wave nerves appeared to be the most effective parameter to distinguish between ALS and KD. The area under the curve was 0.908 (95% confidence interval: 0.835-0.982). The subjects with more than 3 neurons showed great F waves to distinguish between ALS and KD sensitivity and specificity, respectively, 85% and 81%.ALS (r=0.107, P=0.529) and KD patients, respectively. 0.418) there is no statistical significance in the correlation between the total emergence rate of the huge F wave and the course of the disease. Conclusion: the parameters of the F wave and the function of the lower motor neurons can reflect the integrity of the spinal cord motor neuron pool and the excitatory.F wave, which can help identify the ALS and KD, and are not affected by the patient's course. For the male ALS patients who are mainly involved in the involvement of the lower transport neurons, if they are huge The incidence of large F waves increased significantly, especially in the median and peroneal nerves, as well as the multiple nerve recording of the huge F wave (three 3) or the huge F wave symmetrically distributed on the left and right side of the KD diagnosis. KD gene detection was recommended. The electrophysiological study and mechanism research background of the second part of the amyotrophic lateral sclerosis Amyotrophic lateral sclerosis (ALS) cleft hand phenomenon refers to the specific clinical manifestations of the abductor pollicis (abductor pollicis brevis, APB) and the first interosseous muscle (abductor digiti minimi, ADM). Previous studies have shown that the central mechanism and the peripheral axonal mechanism may be involved in the division. No study has shown whether the dysfunction of the spinal motor neuron pool dominating APB and ADM conforms to the ALS cleavage phenomenon. Objective: To investigate whether the dysfunction of the spinal motor neuron pool in the spinal cord motor neurons, which dominates APB and ADM in ALS, is conformed to the phenomenon of ALS splitting hand and to participate in the occurrence of ALS split hand phenomenon by F wave detection. 40 ALS patients in the Peking Union Medical College Hospital neurology department from September 2013 to March 2014 were divided into two groups according to the muscle involvement of the hands of the ALS patients. A group of ALS patients with weak muscle atrophy in hand, and a group of.20 healthy volunteers for ALS patients with no atrophy and weakness of the hand muscles as normal controls Motor nerve conduction detection and F wave measurement were performed on the median nerve and ulnar nerve in the subjects. F wave parameters recorded by APB and ADM were compared. The F wave parameters included the F wave incubation period, the F wave occurrence rate, the F/M amplitude ratio of F wave amplitude, the repeat neuron index and the repeated F wave index. The occurrence rate (P0.05), the repeated neuron index (P0.001) and the repeated F wave index (P0.001) were different. The F wave of the APB recorded in the upper limb of the.ALS patients with no obvious muscle weakness was significantly lower than the F wave recorded in the normal control group APB, and the F wave rate was significantly decreased (P0.001), and the repeated neuron index (P0.001) and repeated exponential wave index were observed. The difference was statistically significant, while the F wave parameters in the ADM records of the ALS patients were compared with the F wave parameters of the APB and ADM records of the upper limbs of the normal.ALS patients with atrophy and weakness of the muscles, the average F wave amplitude (P0.05), the F/M amplitude ratio (P0.05), the F wave occurrence rate, the repeated neuron index and the repeated exponential wave index. In the difference, the electrophysiological difference between.APB and ADM spinal motor neuron pool dysfunction is the specificity of ALS, the F wave occurrence rate of the.APB record (P=0.002), the repeat neuron index (P0.001) and the repeated F wave index (P0.001) help to distinguish between ALS and normal subjects, and the diagnostic value is better than the ADM/APB complex muscle movement. The amplitude ratio of potential wave amplitude. Conclusion: the difference of F wave parameters recorded by APB and ADM in normal subjects and ALS patients is in accordance with the split hand phenomenon. The abnormal involvement of the spinal motor neuron pool function in the occurrence of.F wave of the ALS split hand phenomenon is helpful to the detection of subclinical pathological changes of the spinal cord anterior horn cells in ALS patients, which is helpful for the diagnosis and differential diagnosis of ALS. Third parts are helpful. Comparative study on the electrophysiological characteristics of amyotrophic lateral sclerosis, Kennedy's disease, distal amyotrophic cervical spondylosis and Pingshan disease: amyotrophic lateral sclerosis (ALS) is a rapid progressive neurodegenerative disease, clinical diagnosis needs, and the evidence of simultaneous involvement of the lower transport neurons. But in the early stages of the disease, Muscle weakness may be limited to small hand muscles in ALS patients. For early diagnosis, attention should be paid to eliminating some of the diseases with similar clinical manifestations such as cervical spondylotic amyotrophy (CSA), Hirayama disease, HD, and Kennedy's disease (Kennedy disease, KD). S, the characteristics of nerve conduction examination in patients with distal CSA, HD and KD, and the difference in the involvement of the hand muscles. Methods: a retrospective analysis of the patients (200 cases) that met the ALS diagnostic criteria from 2000 to 2014 of Peking Union Medical College Hospital, which conforms to the distal CSA diagnostic criteria (95 cases), patients with HD diagnostic criteria (88 cases) and accords with KD diagnosis. The patient's medical records of 43 patients (43 cases), the patient's clinical data and the results of the upper limb nerve conduction examination were used as the normal control group. Results: the ALS patient's small finger abductor / pollicis abductor (abductor digiti minimi/abductor pollicis brevis, ADM/APB) complex muscle action potential (compound muscle action potent) Ial, CMAP) amplitude ratio (3.52 + 0.60, P0.001) was higher than that of normal control group (1 + 0.24), and the difference was statistically significant. The CMAP amplitude ratio of ADM/APB in distal CSA patients (0.93 + 0.77, P0.001) and HD patients (0.63 + 0.52, P0.001) was lower than that in normal control group, and the difference was statistically significant. The difference was statistically significant in patients (12.07 4.88mV). The amplitude ratio of ADM/APB CMAP in.HD patients was lower than that of distal CSA, and the difference was statistically significant (P0.001) the CMAP amplitude ratio of ADM/APB CMAP (1.06 + 0.40, P=0.862) was not statistically significant compared with that of the normal control group. The sensory nerve action potential wave amplitude and sensory nerve conduction velocity of the median nerve and the ulnar nerve in patients with.ALS, distal CSA and HD were 81%, and the abnormal rate of sensory nerve conduction velocity in.KD patients was 9.3%.: ALS patients, distal CSA patients, HD patients and KD patients hands. The difference in small muscle atrophy reflects the different pathophysiological mechanisms of the disease. The nerve conduction examination, especially the amplitude ratio of ADM/APB CMAP, helps to diagnose and differentiate between ALS and the disease that has similar clinical manifestations with ALS.
【学位授予单位】：北京协和医学院
【学位级别】：博士
【学位授予年份】：2016
【分类号】：R744.8
本文编号：2149711
[Abstract]:Part 1 the significance of F wave in the diagnosis and differential diagnosis of amyotrophic lateral sclerosis and Kennedy's disease: amyotrophic lateral sclerosis (amyotrophic lateral sclerosis, ALS) is a rapid progressive neurodegenerative disease involving the cerebral cortex and spinal motor neurons of the cerebral cortex. The main clinical manifestations include progressive disease. Weak muscle atrophy, medulla paralysis and pyramidal tract sign. The etiology of ALS is not yet clear and lack of effective treatment. The patient has poor prognosis,.ALS diagnosis needs, the evidence of the simultaneous involvement of the lower motor neurons, the lack of specific biological markers,.ALS, is inactive, the clinical manifestation is heterogeneous, and early misdiagnosis. Missed diagnosis, for example, the signs of motor neuron involvement in some ALS patients appear at the late stage of the course of the disease or the sign of no upper motor neuron, and it is sometimes difficult to identify with Kennedy's disease (Kennedy disease, KD). Previous studies showed that ALS and KD were in sensory nerve conduction, needle level electromyography, cortical excitability test, serum creatine kinase level and clinical level. There is a difference in performance. So far, there is a lack of research on the comparison of the characteristics of the F wave of ALS and KD. Objective: To explore the characteristics of F wave in ALS by comparing the differences in the F wave parameters of the ulnar nerve in ALS patients and normal subjects and analyzing the effects of the dysfunction of the lower motor neurons on the F wave parameters in the ALS patients, and to try to analyze and compare the AL of the ALS. The difference between the median nerve, the ulnar nerve, the tibial nerve and the F wave of the peroneal nerve in S patients and KD patients, and to explore the value of the F wave in the diagnosis and differential diagnosis of ALS and KD. Methods: 82 consecutive patients from September 2013 to July 2014 were enrolled in the neurology department of our hospital, named ALS patients, the patient established a database of medical records, and recorded the name of the patient. Different age, age, course of disease, location, symptoms, signs, and MRC muscular strength classification of.50 were used as normal control group. The ulnar nerve conduction and F wave test were performed on the ulnar nerve in the subjects of the subjects and the ALS patients and the normal control group. Compared with the normal control group, the patients with ALS were compared with the normal control group. The parameter difference of nerve F wave and Logistic regression analysis were used to evaluate the relationship between the F wave parameters of the ulnar nerve in ALS patients and the pyramidal tract sign of the upper limb and the MRC muscle strength classification of the small finger abductor muscle (MRC5, MRC4 and MRC3). From September 2013 to December 2014, the group of 37 men with motor neuron involvement in the Department of Neurology Department of our hospital were diagnosed as the male ALS. 32 KD patients and 30 male healthy volunteers were treated in the Department of Neurology in our hospital as a normal control group. The F wave parameters of the median nerve, ulnar nerve, tibial nerve and peroneal nerve of the patients with ALS, KD and normal control were compared. Results: the comparison of the F wave parameters of the ulnar nerve between the ALS patients and the normal control group was compared with the normal control group. Compared with the normal control group, the average (P=0.040) and the maximum (P) 0.001) F wave amplitude of the ALS patients, the average (P0.001) and the maximum (P0.001) F/M amplitude ratio, the repeat neuron index (P0.001), the repetition of the F wave index (P0.001) and the large F wave occurrence rate (P0.001) increased. P=0.004) shortened and F wave occurrence rate (P0.001) decreased. The difference was statistically significant in the shortest latency (P0.001) and F wave occurrence rate (P0.001) of the ulnar nerve in.ALS patients and the MRC muscle strength classification of the small finger abductor muscle; F wave mean (P0.001) and maximum amplitude (P=0.002) were significantly related to the pyramidal tract of the upper limb. 1) and the maximum (MRC, P=0.001; pyramidal sign, P=0.002) F / M amplitude ratio, F wave time limit (MRC, P0.001; pyramidal tract sign, P=0.047), repeat neuron index (MRC, P0.001; pyramidal tract sign, P=0.009) and repeated exponential wave index (pyramidal tract sign) Compared with the ALS patients and the normal control group, the maximum F wave amplitude of the median nerve, the ulnar nerve, the tibial nerve and the peroneal nerve in the patients with KD was compared with that of the normal control group. The rate of the huge F wave and the ratio of the huge F wave subjects increased, and the difference was statistically significant. Compared with the ALS patients, the huge F wave could appear in the multiple nerves of KD patients and tend to be inclined to the KD patients. In the measured F wave parameters, the number of large F wave nerves appeared to be the most effective parameter to distinguish between ALS and KD. The area under the curve was 0.908 (95% confidence interval: 0.835-0.982). The subjects with more than 3 neurons showed great F waves to distinguish between ALS and KD sensitivity and specificity, respectively, 85% and 81%.ALS (r=0.107, P=0.529) and KD patients, respectively. 0.418) there is no statistical significance in the correlation between the total emergence rate of the huge F wave and the course of the disease. Conclusion: the parameters of the F wave and the function of the lower motor neurons can reflect the integrity of the spinal cord motor neuron pool and the excitatory.F wave, which can help identify the ALS and KD, and are not affected by the patient's course. For the male ALS patients who are mainly involved in the involvement of the lower transport neurons, if they are huge The incidence of large F waves increased significantly, especially in the median and peroneal nerves, as well as the multiple nerve recording of the huge F wave (three 3) or the huge F wave symmetrically distributed on the left and right side of the KD diagnosis. KD gene detection was recommended. The electrophysiological study and mechanism research background of the second part of the amyotrophic lateral sclerosis Amyotrophic lateral sclerosis (ALS) cleft hand phenomenon refers to the specific clinical manifestations of the abductor pollicis (abductor pollicis brevis, APB) and the first interosseous muscle (abductor digiti minimi, ADM). Previous studies have shown that the central mechanism and the peripheral axonal mechanism may be involved in the division. No study has shown whether the dysfunction of the spinal motor neuron pool dominating APB and ADM conforms to the ALS cleavage phenomenon. Objective: To investigate whether the dysfunction of the spinal motor neuron pool in the spinal cord motor neurons, which dominates APB and ADM in ALS, is conformed to the phenomenon of ALS splitting hand and to participate in the occurrence of ALS split hand phenomenon by F wave detection. 40 ALS patients in the Peking Union Medical College Hospital neurology department from September 2013 to March 2014 were divided into two groups according to the muscle involvement of the hands of the ALS patients. A group of ALS patients with weak muscle atrophy in hand, and a group of.20 healthy volunteers for ALS patients with no atrophy and weakness of the hand muscles as normal controls Motor nerve conduction detection and F wave measurement were performed on the median nerve and ulnar nerve in the subjects. F wave parameters recorded by APB and ADM were compared. The F wave parameters included the F wave incubation period, the F wave occurrence rate, the F/M amplitude ratio of F wave amplitude, the repeat neuron index and the repeated F wave index. The occurrence rate (P0.05), the repeated neuron index (P0.001) and the repeated F wave index (P0.001) were different. The F wave of the APB recorded in the upper limb of the.ALS patients with no obvious muscle weakness was significantly lower than the F wave recorded in the normal control group APB, and the F wave rate was significantly decreased (P0.001), and the repeated neuron index (P0.001) and repeated exponential wave index were observed. The difference was statistically significant, while the F wave parameters in the ADM records of the ALS patients were compared with the F wave parameters of the APB and ADM records of the upper limbs of the normal.ALS patients with atrophy and weakness of the muscles, the average F wave amplitude (P0.05), the F/M amplitude ratio (P0.05), the F wave occurrence rate, the repeated neuron index and the repeated exponential wave index. In the difference, the electrophysiological difference between.APB and ADM spinal motor neuron pool dysfunction is the specificity of ALS, the F wave occurrence rate of the.APB record (P=0.002), the repeat neuron index (P0.001) and the repeated F wave index (P0.001) help to distinguish between ALS and normal subjects, and the diagnostic value is better than the ADM/APB complex muscle movement. The amplitude ratio of potential wave amplitude. Conclusion: the difference of F wave parameters recorded by APB and ADM in normal subjects and ALS patients is in accordance with the split hand phenomenon. The abnormal involvement of the spinal motor neuron pool function in the occurrence of.F wave of the ALS split hand phenomenon is helpful to the detection of subclinical pathological changes of the spinal cord anterior horn cells in ALS patients, which is helpful for the diagnosis and differential diagnosis of ALS. Third parts are helpful. Comparative study on the electrophysiological characteristics of amyotrophic lateral sclerosis, Kennedy's disease, distal amyotrophic cervical spondylosis and Pingshan disease: amyotrophic lateral sclerosis (ALS) is a rapid progressive neurodegenerative disease, clinical diagnosis needs, and the evidence of simultaneous involvement of the lower transport neurons. But in the early stages of the disease, Muscle weakness may be limited to small hand muscles in ALS patients. For early diagnosis, attention should be paid to eliminating some of the diseases with similar clinical manifestations such as cervical spondylotic amyotrophy (CSA), Hirayama disease, HD, and Kennedy's disease (Kennedy disease, KD). S, the characteristics of nerve conduction examination in patients with distal CSA, HD and KD, and the difference in the involvement of the hand muscles. Methods: a retrospective analysis of the patients (200 cases) that met the ALS diagnostic criteria from 2000 to 2014 of Peking Union Medical College Hospital, which conforms to the distal CSA diagnostic criteria (95 cases), patients with HD diagnostic criteria (88 cases) and accords with KD diagnosis. The patient's medical records of 43 patients (43 cases), the patient's clinical data and the results of the upper limb nerve conduction examination were used as the normal control group. Results: the ALS patient's small finger abductor / pollicis abductor (abductor digiti minimi/abductor pollicis brevis, ADM/APB) complex muscle action potential (compound muscle action potent) Ial, CMAP) amplitude ratio (3.52 + 0.60, P0.001) was higher than that of normal control group (1 + 0.24), and the difference was statistically significant. The CMAP amplitude ratio of ADM/APB in distal CSA patients (0.93 + 0.77, P0.001) and HD patients (0.63 + 0.52, P0.001) was lower than that in normal control group, and the difference was statistically significant. The difference was statistically significant in patients (12.07 4.88mV). The amplitude ratio of ADM/APB CMAP in.HD patients was lower than that of distal CSA, and the difference was statistically significant (P0.001) the CMAP amplitude ratio of ADM/APB CMAP (1.06 + 0.40, P=0.862) was not statistically significant compared with that of the normal control group. The sensory nerve action potential wave amplitude and sensory nerve conduction velocity of the median nerve and the ulnar nerve in patients with.ALS, distal CSA and HD were 81%, and the abnormal rate of sensory nerve conduction velocity in.KD patients was 9.3%.: ALS patients, distal CSA patients, HD patients and KD patients hands. The difference in small muscle atrophy reflects the different pathophysiological mechanisms of the disease. The nerve conduction examination, especially the amplitude ratio of ADM/APB CMAP, helps to diagnose and differentiate between ALS and the disease that has similar clinical manifestations with ALS.
【学位授予单位】：北京协和医学院
【学位级别】：博士
【学位授予年份】：2016
【分类号】：R744.8
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