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肯尼迪病的EMG特点及其诊断价值OA

EMG characteristics of Kennedy's disease and its diagnostic value

中文摘要英文摘要

目的 探讨肯尼迪病的EMG特点及EMG特异性对肯尼迪病的诊断价值.方法 选取 2018年6 月至2024 年5 月来我院就诊的29 例不同程度肌肉萎缩患者为研究对象.将通过基因检测确诊为肯尼迪病的7 例患者作为观察组(KD组),并与其余 12 例颈髓型颈椎病患者(CS组)、10 例运动神经元病患者(MND组)进行比较.所有入组患者均分别检测了正中神经、尺神经、胫神经、腓总神经、腓肠神经、腓浅神经的运动神经传导速度、波幅(CMAP),感觉神经传导速度、波幅(SNAP)和F波的潜伏期及巨大F波的出现率,并用针极EMG分别检测延髓段、颈段、胸段、腰段的所支配的舌骨肌、斜方肌等 9 组肌肉.对比三组患者EMG相关指标并进行统计学分析.结果 KD组、CS组及MND组肌无力、肢体麻木、性功能下降、鼻音重、肌肉颤搐、吞咽困难、乳房发育、脊柱MRI异常、肌酶异常比率比较,差异有统计学意义(均P<0.05).KD组正中神经、尺神经、腓浅神经、腓肠神经的SNAP明显低于CS组和MND组,MND组腓肠神经的SNAP明显高于CS组(均P<0.05).MND组正中神经的CMAP明显低于KD和CS组(均P<0.05).KD组尺神经的CMAP明显低于CS组和MND组(均P<0.05).KD组、MND组胫神经和腓总神经的CMAP均明显低于CS组(均P<0.05).KD组腓总神经的运动神经传导速度明显低于CS组和MND组(均P<0.05).KD组运动神经的巨大F波出现率明显大于CS组(P<0.05).CS组延髓段、胸段、腰段针极EMG的正常率明显高于KD组(均P<0.05).MND组延髓段、胸段针极EMG的活动性损害出现率明显高于KD组(均P<0.05).KD组颈段、胸段、腰段针极EMG的慢性损害出现率均为100%.KD组延髓段、胸段、腰段针极EMG的慢性损害出现率明显高于CS组(均P<0.05).KD组胸段针极EMG的慢性损害出现率明显高于MND组(P<0.05).结论 肯尼迪病的EMG具有其特异性,利用其特异性对肯尼迪病具有一定的筛查、诊断和鉴别诊断意义.

Objective To investigate the EMG characteristics of Kennedy's disease and the diagnostic value of the EMG specificity.Methods Twenty-nine patients with varying degrees of muscle atrophy who visited our hospital between June 2018 and May 2024 were selected as the study subjects.Seven patients diagnosed with Kennedy's disease via genetic testing served as the observation group(KD group),and compared with the remaining 12 patients with cervical spondylotic myelopathy(CS group)and 10 patients with motor neuron disease(MND group).The motor nerve conduction velocity,amplitude(CMAP),sensory nerve conduction velocity,amplitude(SNAP),and the latency of F wave and the appearance rate of giant F-waves of the median,ulnar,tibial,peroneal,sural and superficial peroneal nerves were tested in all recruited patients,respectively.The needle electrode EMG was used to detect nine groups of muscles including the hyoid muscle,trapezius muscle,etc.,which were dominated by bulbar,cervical,thoracic and lumbar segments,respectively.The EMG-related parameters of the three groups were compared and statistically analyzed.Results The differences of the rates of muscle weakness,limb numbness,decreased sexual function,hyponasality,muscle twitching,dysphagia,gynecomastia,spinal MRI abnormalities,and abnormal muscle enzymes among KD group,CS group and MND group were statistically significant(all P<0.05).The SNAP of median,ulnar,superficial peroneal and sural nerves in KD group were significantly lower than those in CS and MND groups,and the SNAP of sural nerve in MND group was significantly higher than that in CS group(all P<0.05).The CMAP of the median nerve in MND group was significantly lower than those in KD and CS groups(both P<0.05).The CMAP of the ulnar nerve in KD group was significantly lower than those in CS and MND groups(both P<0.05).The CMAP of the tibial,peroneal nerves in KD and MND groups were significantly lower than those in the CS group(all P<0.05).The motor nerve conduction velocity of the peroneal nerve in KD group was significantly lower than those in CS and MND groups(both P<0.05).The incidence of giant F-waves in the motor nerves of KD group was significantly higher than that in CS group(P<0.05).The normal rates of needle electrode EMG of the bulbar,thoracic and lumbar segments in CS group were significantly higher than those in KD group(all P<0.05).The active damage rates of needle electrode EMG of the bulbar and thoracic segments in MND group were significantly higher than those in KD group(all P<0.05).The chronic damage rates of needle electrode EMG of cervical,thoracic and lumbar segments in KD group were 100%,respectively.The chronic damage rates of needle electrode EMG of bulbar,thoracic and lumbar segments in KD group were significantly higher than those in CS group(all P<0.05).The chronic damage rate of needle electrode EMG of thoracic segment in KD group was significantly higher than that in MND group(P<0.05).Conclusion The EMG of Kennedy's disease has its specificity,and its specificity has a certain screening,diagnostic and differential diagnostic significance for Kennedy's disease.

孙成梅;戴月;吴倩倩;杨林丽;蒋瑞

223001 淮安市第一人民医院神经内科223001 淮安市第一人民医院神经电生理室223001 淮安市第一人民医院神经电生理室223001 淮安市第一人民医院神经电生理室223001 淮安市第一人民医院神经电生理室

医药卫生

肯尼迪病EMG特点诊断价值鉴别诊断

Kennedy's diseaseEMG characteristicsdifferential diagnosis

《临床神经病学杂志》 2026 (2)

109-113,5

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