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中文摘要：
目的 对肯尼迪病（KD）患者进行三重刺激技术（TST）检测,探索其上运动神经元（UMN）的亚临床损害.方法 分析2013年7月至2014年7月于北京大学第三医院神经内科就诊的肯尼迪病患者20例.对其进行临床查体、基因检测、神经电生理检测,主要观察TST检测（包括经颅磁刺激、外周神经刺激、对冲技术等）,计算TSTtest/TSTcontrol波幅比,判断其上运动神经元功能状况.结果 20例患者的临床表现、基因检测[雄激素受体基因第1外显子CAG重复序列（44±5）个]、常规肌电图结果均符合KD.TST检测结果：13例TST、中枢运动传导时间（CMCT）均正常,其中1例临床存在腱反射活跃,余12例腱反射减低;7例患者TST结果异常,其中4例CMCT正常,5例临床查体有不同程度的腱反射亢进或病理征,其中2例合并腔隙性脑梗死,2例合并脊髓型颈椎病,3例为KD原发性TST异常.结论 肯尼迪病可以存在上运动神经元的亚临床损害,同时必需排除锥体束损害的其他原因.
英文摘要：
Objective To explore the presence of subclinical upper motor neuron （UMN） dysfunction in Kennedy disease （KD） patients with triple stimulation technique （TST）.Methods At our hospital from July 2013 to July 2014,a total of 20 KD patients with clinical manifestations,genetic testing and routine electrophysiological tests were examined by triple stimulation technique （TST） combining transcranial magnetic stimulation （TMS） of motor cortex with peripheral collision studies.And the results were expressed by TST amplitude ratio （TST test/TST control）.Results All patients had typical presentations and were genetically proved.The expansion size of polymorphic tandem cytosine-adenine-guanine （CAG） repeat in the first exon of androgen receptor gene was（44 ± 5）.Electromyography showed acute and chronic neurogenic damage.Sensory nerve action potentials declined in amplitude.TST amplitude ratio was normal for 13 KD patients and 7 cases were significantly altered.Tendon hyperreflexia was found in 1 patient with normal TST.In patients with abnormal TST,central motor conduction time （CMCT） was normal in 4 patients and tendon hyperreflexia was found in 5 patients.The possible reasons for 7 patients with abnormal TST were concurrent lacunar infaction （n =2）,concurrent spondylotic myelopathy （n =2） and KD alone （n =3）.Conclusion Subclinical involvement of UMN may be present in KD patients while other potential causes must be excluded.
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