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脊髓小脑性共济失调3型的周围神经损害

发布时间:2018-07-16 10:30
【摘要】:[目的]探讨脊髓小脑性共济失调3型(spinocerebellar ataxia type 3 SCA3)的周围神经损害特点,为基因检测提供更加明确的依据。[方法]依据设定的SCA3病例组和健康对照组的纳入和排除标准,收集2014年至2016年在昆明医科大学第一附属医院神经内科就诊并经基因检查确诊的SCA3患者共26例为病例组,随机选取与病例组年龄、性别相匹配健康人群26例为健康对照组,病例组和对照组均完善右侧肢体神经传导检测,比较病例组与对照组是否有差异,进而分析病例组的神经传导速度与病程的相关性、动作电位波幅与病程的相关性、神经传导速度与CAG重复次数的相关性和动作电位波幅与CAG重复次数的相关性,分析病程、CAG重复次数与电生理结果的相关性,寻找SCA3的周围神经损害的相关因素。病例组还完善针极肌电图检查,了解SCA3是否存在肌源性损害,了解电生理异常是否早于周围神经损害的临床症状出现。数据经过SPSS17.0统计软件包进行统计学分析。[结果](1)病例组正中神经、尺神经、胫后神经、腓总神经运动传导速度慢于正常对照组(p0.0001,p=0.002,p=0.001,p=0.006);(2)病例组正中神经、尺神经、胫后神经、腓总神经远端运动潜伏期较对照组延长(p0.0001,p0.0001,p=0.01,p=0.001);(3)病例组正中神经、尺神经、胫后神经CMAP波幅低于正常对照组(p0.0001,p=0.003,p=0.024):病例组腓总神经CMAP波幅与正常对照组的差异无统计学意义(p=0.443);(4)病例组正中神经、尺神经、胫后神经、腓肠神经感觉传导速度慢于正常对照组(p0.0001,p0.0001,p=0.002,p=0.001);(5)病例组正中神经、尺神经、胫后神经、腓肠神经远端感觉潜伏期较正常对照组延长(p0.0001,p0.0001,p=0.004,p=0.001);(6)病例组正中神经、尺神经、胫后神经、腓肠神经SNAP波幅低于正常对照组(p0.0001,p=0.001,p0.0001,p=0.001);(7)病例组正中神经、尺神经、胫后神经、腓总神经运动传导速度与病程无直线相关(r=-0.071,p=0.729;r=-0.020,p=0.922;r=0.108,p=0.599;r=-0.01,p=0.959);(8)病例组正中神经、尺神经、胫后神经、腓总神经CMAP波幅与病程无直线相关(r=-0.113,p=0.583;r=0.085,p=0.681;r=-0.045,p=0.827;r==0.114,p=0.581);(9)病例组正中神经、尺神经、胫后神经、腓总神经感觉传导速度与病程无直线相关(r=-0.089,p=0.666;r=-0.151,p=0.460;r=0.028,p=0.892;r=-0.02,p=0.924);(10)病例组正中神经、尺神经、胫后神经、腓总神经SNAP波幅与病程无直线相关(r=-0.117,p=0.570;r=-0.007,p=0.971;r=-0.137,p=0.504;r=-0.223,p=0.273);(11)病例组正中神经、尺神经、胫后神经、腓总神经运动传导速度与CAG重复次数无直线相关(r=-0.376,p=0.058;r=-0.216,p=0.290;r=-0.148,p=0.472;r=-0.281,p=0.165);(12)病例组正中神经、尺神经、胫后神经、腓总神经CMAP波幅与CAG重复次数无直线相关(r=-0.109,p=0.596;r=-0.054,p=0.792;r=-0.240,p=0.237;r=-0.154,p=0.450);(13)病例组正中神经、尺神经感觉传导速度与CAG重复次数呈负相关(r=-0.546,p=0.004;r=-0.464,p=0.017);病例组胫后神经、腓肠神经感觉传导速度与CAG重复次数无直线相关(r=-0.279,p=0.168;r=-0.288,p=0.154);(14)病例组正中神经、尺神经、胫后神经、腓总神经SNAP波幅与CAG重复次数无直线相关(r=-0.09,p=0.622;r=-0.372,p=0.061;r=-0.175,p=0.394;r=-0.240,p=0.238);(15)电生理结果与病程、CAG重复次数无相关关系(r=:0.137,p=0.504;r=0.059,p=0.776);(16)神经传导检测出现异常(81%),其中混合性感觉运动神经异常(58%),单纯性感觉神经异常(11%);单纯运动神经异常(12%);(17)神经传导速度和动作电位波幅均有异常(65%),单纯动作电位波幅异常(12%),单纯神经传导速度异常(4%)。(18)38.46%的患者无感觉异常的症状和体征但感觉神经传导检测异常,57.69%的患者无肌力减弱和肌萎缩但运动神经传导检测异常;运动神经传导阳性率为69.23%,EMG阳性率为38.46%,运动神经传导检测阳性率高于EMG阳性率(p0.0001)。[结论]SCA3存在明显的周围神经损害,包括混合性感觉运动神经损害、单纯性运动神经损害、单纯性感觉神经损害,其中以混合性感觉运动神经损害为主。正中神经、尺神经的感觉传导速度与CAG重复次数呈负相关。神经传导检测异常的比例高,轴索损害和脱髓鞘并存多见。对运动神经损害的检查中,运动神经传导检测较EMG敏感。电生理检测可以发现周围神经亚临床损害。SCA3这些电生理特点可为基因检测提供更加明确的依据。
[Abstract]:[Objective] to explore the characteristics of peripheral nerve damage of the spinal cord ataxia type 3 (spinocerebellar ataxia type 3 SCA3), and provide a more clear basis for gene detection. [Methods] according to the inclusion and exclusion criteria of the set SCA3 case group and the healthy control group, the nerve was collected from 2014 to 2016 at the First Affiliated Hospital of Kunming Medical University. A total of 26 cases of SCA3 patients diagnosed by internal medical examination and gene examination were selected as case group. The age of the case group was selected randomly and 26 cases of the healthy people were matched to the healthy control group. Both the case group and the control group improved the right limb nerve conduction detection, and compared the difference between the case group and the control group, and then analyzed the nerve conduction velocity in the case group. The correlation with the course of the disease, the correlation between the amplitude of action potential and the course of the disease, the correlation between the nerve conduction velocity and the repetition of CAG, the correlation between the amplitude of action potential and the repetition of CAG, analysis of the correlation between the duration of the disease, the repetition of CAG and the electrophysiological results, in search of the related factors of the damage of the peripheral deity around the SCA3. The case group also perfected the needle pole electromyography To see if SCA3 had myogenic damage and whether electrophysiological abnormalities were earlier than the clinical symptoms of peripheral nerve damage. Data were statistically analyzed by SPSS17.0 software package. [results] (1) the median nerve, ulnar nerve, posterior tibial nerve, and peroneal nerve were slower than the normal control group (P0.0001, p=0.0). 02, p=0.001, p=0.006); (2) the median nerve, ulnar nerve, the posterior tibial nerve, the distal tibial nerve and the distal peroneal nerve were prolonged (P0.0001, P0.0001, p=0.01, p=0.001) in the case group. (3) the median nerve, ulnar nerve, and the posterior tibial nerve were lower than the normal control group (P0.0001, p=0.003, p=0.024): the CMAP wave amplitude and the positive amplitude of the common peroneal nerve in the case group. There was no significant difference in the control group (p=0.443). (4) the median nerve, ulnar nerve, the posterior tibial nerve and the gastrocnemius nerve conduction velocity were slower than the normal control group (P0.0001, P0.0001, p=0.002, p=0.001) in the case group (5) the median nerve, ulnar nerve, the posterior tibial nerve and the sural nerve were longer than the normal control group (P0.0001 P0.0001, p=0.004, p=0.001); (6) the median nerve, ulnar nerve, posterior tibial nerve, and the gastrocnemius nerve SNAP wave amplitude was lower than the normal control group (P0.0001, p=0.001, P0.0001, p=0.001) in the case group; (7) the median nerve, ulnar nerve, the posterior tibial nerve, the peroneal nerve movement conduction velocity had no linear correlation with the course of the disease (r=-0.071, p=0.729; r=-0.020, p=0.922; p=0.922; P=0.599; r=-0.01, p=0.959); (8) the median nerve, ulnar nerve, posterior tibial nerve, and the CMAP wave of the peroneal nerve in the case group have no linear correlation with the course of the disease (r=-0.113, p=0.583; r=0.085, p=0.681; r=-0.045, p=0.827; p=0.581); (9) there is no linear phase between the sensory conduction velocity of the ulnar nerve, the posterior tibial nerve and the general peroneal nerve in the case group. (r=-0.089, p=0.666; r=-0.151, p=0.460; r=0.028, p=0.892; r=-0.02, p=0.924); (10) there is no linear correlation between the median nerve, the ulnar nerve, the posterior tibial nerve and the common peroneal nerve in the case group (r=-0.117, p=0.570; r=-0.007, 11); (11) the case group is the median nerve, the ulnar nerve, the tibial nerve, and the peroneal nerve. There was no linear correlation between the total nerve conduction velocity and the number of CAG repetitions (r=-0.376, p=0.058; r=-0.216, p=0.290; r=-0.148, p=0.472; r=-0.281, p=0.165); (12) the median nerve, the ulnar nerve, the posterior tibial nerve and the CMAP wave amplitude of the common peroneal nerve were not directly related to the CAG repetitions. 0.154, p=0.450); (13) the median nerve of the case group was negatively correlated with the frequency of the sensory conduction of the ulnar nerve (r=-0.546, p=0.004; r=-0.464, p=0.017). There was no linear correlation between the posterior tibial nerve, the conduction velocity of the gastrocnemius nerve and the number of CAG repetition (r= -0.279, p=0.168; r=-0.288, p=0.154); (14) the median nerve and ulnar nerve in the case group. There was no linear correlation between the SNAP wave amplitude of the posterior tibial nerve and the common peroneal nerve (r=-0.09, p=0.622; r=-0.372, p=0.061; r=-0.175, p=0.394; r=-0.240, p=0.238); (15) there was no correlation between the electrophysiological results and the course of the disease, CAG repetition (r=:0.137, 81%), and (81%), in which the mixture was mixed. Abnormal sensorimotor nerve (58%), simple sensory nerve abnormality (11%), simple motor nerve abnormality (12%), and (17) abnormal nerve conduction velocity and action potential amplitude (65%), simple action potential amplitude (12%) and simple nerve conduction velocity (4%). (18) 38.46% patients had no symptoms and signs but sensory nerve transmission. There was no abnormality of muscle strength and muscular atrophy in 57.69% of patients, but motor nerve conduction detection was abnormal, the positive rate of motor nerve conduction was 69.23%, the positive rate of EMG was 38.46%, and the positive rate of motor nerve conduction detection was higher than that of EMG (P0.0001). [conclusion]SCA3 has obvious peripheral nerve damage, including mixed sensory motor nerve damage. Simple motor nerve damage, simple sensory nerve damage, mainly mixed sensorimotor nerve damage. The sensory conduction velocity of median nerve and ulnar nerve is negatively correlated with CAG repetition. The ratio of abnormal nerve conduction detection is high, axonal damage and demyelination are more common. The transmission detection is more sensitive than EMG. Electrophysiological detection can detect subclinical lesion of peripheral nerve.SCA3. These electrophysiological characteristics can provide a more clear basis for gene detection.
【学位授予单位】:昆明医科大学
【学位级别】:硕士
【学位授予年份】:2016
【分类号】:R744.7

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