肯尼迪病患者60例神经电生理研究  

The neurophysiological evaluation in 60 patients with Kennedy disease

作　　者：陈君逸[1] 徐迎胜[1] 张朔[1] 樊东升[1] Chen Junyi;Xu Yingsheng;Zhang Shuo;Fan Dongsheng(Department of Neurology,Peking University Third Hospital,Beijing 100191,China)

机构地区：[1]北京大学第三医院神经内科,北京100191

出　　处：《中华神经科杂志》2023年第8期871-875,共5页Chinese Journal of Neurology

摘　　要：目的对肯尼迪病患者进行神经电生理检测,了解其神经功能状况。方法对2010年11月至2022年11月北京大学第三医院门诊和病房收治的60例肯尼迪病患者(根据是否合并糖尿病分为单纯肯尼迪病组29例、肯尼迪病合并糖尿病组31例)及60例糖尿病周围神经病(DPN)患者,分别进行肌电图、神经传导、体感诱发电位(SEP)、接触性热痛诱发电位(CHEP)及三重刺激技术(TST)检测。采用t检验分别对组内及组间神经传导速度及动作电位波幅、SEP各波潜伏期和波间期、CHEP起始峰潜伏期等参数进行比较。结果与正常值比较,肯尼迪病组患者感觉神经动作电位(SNAP)波幅明显降低[正中神经(0.7±0.4)μV,尺神经(0.8±0.3)μV,腓肠神经(1.8±0.1)μV,波幅下降30%~80%],正中神经和尺神经波幅低于腓肠神经(t=2.43,P=0.010;t=2.40,P=0.010);SEP和CHEP的周围段异常(潜伏期延长115%~130%),SEP中枢段异常(潜伏期延长104%~115%),17例TST结果异常(TST_(测试)/TST_(对照)波幅比下降40%~60%)。DPN组患者SNAP波幅降低[正中神经(2.9±0.5)μV,尺神经(2.6±0.6)μV,腓肠神经(1.6±0.2)μV,波幅下降30%~50%],腓肠神经波幅低于正中神经和尺神经(t=2.52,P=0.006;t=2.47,P=0.007),SEP和CHEP周围段受累(潜伏期延长75%~112%),中枢段正常。与DPN组比较,肯尼迪病合并糖尿病组患者上肢SNAP波幅降低[正中神经(0.7±0.3)μV,t=3.18,P=0.001;尺神经(0.8±0.4)μV,t=3.20,P=0.001]。结论肯尼迪病患者感觉神经大、小纤维均受累,深感觉通路中枢段异常;除前角细胞受累外,锥体束功能可能受损。Objective To study the neurophysiological features of Kennedy disease(KD)and to figure out the function of the nervous system.Methods Subjects were recruited from the outpatient and the ward of Peking University Third Hospital from November 2010 to November 2022.Sixty patients with KD(29 patients with KD alone,31 KD cases with the complication of diabetes mellitus)and 60 patients with diabetic polyneuropathy(DPN)were included in this study.Electrophysiological tests were performed in all subjects,including electromyogram,nerve conduction study,somaosensory evoked potential(SEP),contact heat evoked potential(CHEP)and triple stimulation technique(TST).Student′s t-tests were conducted to compare differences intra or inter groups of nerve conduction velocity and action potential of nerve conduction study,latency and interphase of SEP,initial peak latency of CHEP and other parameters.Results Compared with the normative value,the amplitude of the sensory nerve action potential(SNAP)declined by 30%-80% in KD patients[median nerve(0.7±0.4)μV,ulnar nerve(0.8±0.3)μV,sural nerve(1.8±0.1)μV],the amplitude of the median and ulnar nerves was lower than the sural nerves(t=2.43,P=0.010;t=2.40,P=0.010).The conduction time of peripheral segments of SEP and CHEP was prolonged by 115%-130%,while that of the central segments was prolonged by 104%-115%in SEP.TST_(test)/TST_(control) declined by 40%-60%in 17 patients with KD.The amplitude of SNAP declined by 30%-50%in patients with DPN[median nerve(2.9±0.5)μV,ulnar nerve(2.6±0.6)μV,sural nerve(1.6±0.2)μV],the amplitude of the sural nerves was lower than the median and ulnar nerves(t=2.52,P=0.006;t=2.47,P=0.007).The conduction time of peripheral segments of SEP and CHEP was prolonged by 75%-112%,while that of the central segments was normal in both SEP and CHEP in DPN patients.Compared with DPN patients,the upper limb SNAP amplitude was lower in KD patients with the complication of diabetes mellitus[median nerve(0.7±0.3)μV,t=3.18,P=0.001;ulnar nerve(0.8±0.4)μV,t=3.20,P

关 键 词：脊髓延髓性肌萎缩 X连锁 肌电描记术 神经传导 诱发电位 躯体感觉 

分 类 号：R744.8[医药卫生—神经病学与精神病学]