脊髓小腦性共濟(jì)失調(diào)3型的周?chē)窠?jīng)損害
發(fā)布時(shí)間：2018-07-16 10:30
【摘要】：[目的]探討脊髓小腦性共濟(jì)失調(diào)3型(spinocerebellar ataxia type 3 SCA3)的周?chē)窠?jīng)損害特點(diǎn),為基因檢測(cè)提供更加明確的依據(jù)。[方法]依據(jù)設(shè)定的SCA3病例組和健康對(duì)照組的納入和排除標(biāo)準(zhǔn),收集2014年至2016年在昆明醫(yī)科大學(xué)第一附屬醫(yī)院神經(jīng)內(nèi)科就診并經(jīng)基因檢查確診的SCA3患者共26例為病例組,隨機(jī)選取與病例組年齡、性別相匹配健康人群26例為健康對(duì)照組,病例組和對(duì)照組均完善右側(cè)肢體神經(jīng)傳導(dǎo)檢測(cè),比較病例組與對(duì)照組是否有差異,進(jìn)而分析病例組的神經(jīng)傳導(dǎo)速度與病程的相關(guān)性、動(dòng)作電位波幅與病程的相關(guān)性、神經(jīng)傳導(dǎo)速度與CAG重復(fù)次數(shù)的相關(guān)性和動(dòng)作電位波幅與CAG重復(fù)次數(shù)的相關(guān)性,分析病程、CAG重復(fù)次數(shù)與電生理結(jié)果的相關(guān)性,尋找SCA3的周?chē)窠?jīng)損害的相關(guān)因素。病例組還完善針極肌電圖檢查,了解SCA3是否存在肌源性損害,了解電生理異常是否早于周?chē)窠?jīng)損害的臨床癥狀出現(xiàn)。數(shù)據(jù)經(jīng)過(guò)SPSS17.0統(tǒng)計(jì)軟件包進(jìn)行統(tǒng)計(jì)學(xué)分析。[結(jié)果](1)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓總神經(jīng)運(yùn)動(dòng)傳導(dǎo)速度慢于正常對(duì)照組(p0.0001,p=0.002,p=0.001,p=0.006);(2)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓總神經(jīng)遠(yuǎn)端運(yùn)動(dòng)潛伏期較對(duì)照組延長(zhǎng)(p0.0001,p0.0001,p=0.01,p=0.001)；(3)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)CMAP波幅低于正常對(duì)照組(p0.0001,p=0.003,p=0.024)：病例組腓總神經(jīng)CMAP波幅與正常對(duì)照組的差異無(wú)統(tǒng)計(jì)學(xué)意義(p=0.443)；(4)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓腸神經(jīng)感覺(jué)傳導(dǎo)速度慢于正常對(duì)照組(p0.0001,p0.0001,p=0.002,p=0.001)；(5)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓腸神經(jīng)遠(yuǎn)端感覺(jué)潛伏期較正常對(duì)照組延長(zhǎng)(p0.0001,p0.0001,p=0.004,p=0.001)；(6)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓腸神經(jīng)SNAP波幅低于正常對(duì)照組(p0.0001,p=0.001,p0.0001,p=0.001)；(7)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓總神經(jīng)運(yùn)動(dòng)傳導(dǎo)速度與病程無(wú)直線(xiàn)相關(guān)(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)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓總神經(jīng)CMAP波幅與病程無(wú)直線(xiàn)相關(guān)(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)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓總神經(jīng)感覺(jué)傳導(dǎo)速度與病程無(wú)直線(xiàn)相關(guān)(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)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓總神經(jīng)SNAP波幅與病程無(wú)直線(xiàn)相關(guān)(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)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓總神經(jīng)運(yùn)動(dòng)傳導(dǎo)速度與CAG重復(fù)次數(shù)無(wú)直線(xiàn)相關(guān)(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)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓總神經(jīng)CMAP波幅與CAG重復(fù)次數(shù)無(wú)直線(xiàn)相關(guān)(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)病例組正中神經(jīng)、尺神經(jīng)感覺(jué)傳導(dǎo)速度與CAG重復(fù)次數(shù)呈負(fù)相關(guān)(r=-0.546,p=0.004;r=-0.464,p=0.017)；病例組脛后神經(jīng)、腓腸神經(jīng)感覺(jué)傳導(dǎo)速度與CAG重復(fù)次數(shù)無(wú)直線(xiàn)相關(guān)(r=-0.279,p=0.168；r=-0.288,p=0.154)；(14)病例組正中神經(jīng)、尺神經(jīng)、脛后神經(jīng)、腓總神經(jīng)SNAP波幅與CAG重復(fù)次數(shù)無(wú)直線(xiàn)相關(guān)(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)電生理結(jié)果與病程、CAG重復(fù)次數(shù)無(wú)相關(guān)關(guān)系(r=：0.137,p=0.504;r=0.059,p=0.776)；(16)神經(jīng)傳導(dǎo)檢測(cè)出現(xiàn)異常(81%),其中混合性感覺(jué)運(yùn)動(dòng)神經(jīng)異常(58%),單純性感覺(jué)神經(jīng)異常(11%)；單純運(yùn)動(dòng)神經(jīng)異常(12%)；(17)神經(jīng)傳導(dǎo)速度和動(dòng)作電位波幅均有異常(65%),單純動(dòng)作電位波幅異常(12%),單純神經(jīng)傳導(dǎo)速度異常(4%)。(18)38.46%的患者無(wú)感覺(jué)異常的癥狀和體征但感覺(jué)神經(jīng)傳導(dǎo)檢測(cè)異常,57.69%的患者無(wú)肌力減弱和肌萎縮但運(yùn)動(dòng)神經(jīng)傳導(dǎo)檢測(cè)異常；運(yùn)動(dòng)神經(jīng)傳導(dǎo)陽(yáng)性率為69.23%,EMG陽(yáng)性率為38.46%,運(yùn)動(dòng)神經(jīng)傳導(dǎo)檢測(cè)陽(yáng)性率高于EMG陽(yáng)性率(p0.0001)。[結(jié)論]SCA3存在明顯的周?chē)窠?jīng)損害,包括混合性感覺(jué)運(yùn)動(dòng)神經(jīng)損害、單純性運(yùn)動(dòng)神經(jīng)損害、單純性感覺(jué)神經(jīng)損害,其中以混合性感覺(jué)運(yùn)動(dòng)神經(jīng)損害為主。正中神經(jīng)、尺神經(jīng)的感覺(jué)傳導(dǎo)速度與CAG重復(fù)次數(shù)呈負(fù)相關(guān)。神經(jīng)傳導(dǎo)檢測(cè)異常的比例高,軸索損害和脫髓鞘并存多見(jiàn)。對(duì)運(yùn)動(dòng)神經(jīng)損害的檢查中,運(yùn)動(dòng)神經(jīng)傳導(dǎo)檢測(cè)較EMG敏感。電生理檢測(cè)可以發(fā)現(xiàn)周?chē)窠?jīng)亞臨床損害。SCA3這些電生理特點(diǎn)可為基因檢測(cè)提供更加明確的依據(jù)。
[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.
【學(xué)位授予單位】：昆明醫(yī)科大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2016
【分類(lèi)號(hào)】：R744.7
本文編號(hào)：2126100
[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.
【學(xué)位授予單位】：昆明醫(yī)科大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2016
【分類(lèi)號(hào)】：R744.7
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