中华医学杂志
中華醫學雜誌
중화의학잡지
National Medical Journal of China
2015年
19期
1522-1525
,共4页
肯尼迪病%上运动神经元%经颅磁刺激%三重刺激技术
肯尼迪病%上運動神經元%經顱磁刺激%三重刺激技術
긍니적병%상운동신경원%경로자자격%삼중자격기술
Kennedy disease%Upper motor neuron%Transcranial magnetic stimulation%Triple stimulation technique
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.
目的 對肯尼迪病(KD)患者進行三重刺激技術(TST)檢測,探索其上運動神經元(UMN)的亞臨床損害.方法 分析2013年7月至2014年7月于北京大學第三醫院神經內科就診的肯尼迪病患者20例.對其進行臨床查體、基因檢測、神經電生理檢測,主要觀察TST檢測(包括經顱磁刺激、外週神經刺激、對遲技術等),計算TSTtest/TSTcontrol波幅比,判斷其上運動神經元功能狀況.結果 20例患者的臨床錶現、基因檢測[雄激素受體基因第1外顯子CAG重複序列(44±5)箇]、常規肌電圖結果均符閤KD.TST檢測結果:13例TST、中樞運動傳導時間(CMCT)均正常,其中1例臨床存在腱反射活躍,餘12例腱反射減低;7例患者TST結果異常,其中4例CMCT正常,5例臨床查體有不同程度的腱反射亢進或病理徵,其中2例閤併腔隙性腦梗死,2例閤併脊髓型頸椎病,3例為KD原髮性TST異常.結論 肯尼迪病可以存在上運動神經元的亞臨床損害,同時必需排除錐體束損害的其他原因.
목적 대긍니적병(KD)환자진행삼중자격기술(TST)검측,탐색기상운동신경원(UMN)적아림상손해.방법 분석2013년7월지2014년7월우북경대학제삼의원신경내과취진적긍니적병환자20례.대기진행림상사체、기인검측、신경전생리검측,주요관찰TST검측(포괄경로자자격、외주신경자격、대충기술등),계산TSTtest/TSTcontrol파폭비,판단기상운동신경원공능상황.결과 20례환자적림상표현、기인검측[웅격소수체기인제1외현자CAG중복서렬(44±5)개]、상규기전도결과균부합KD.TST검측결과:13례TST、중추운동전도시간(CMCT)균정상,기중1례림상존재건반사활약,여12례건반사감저;7례환자TST결과이상,기중4례CMCT정상,5례림상사체유불동정도적건반사항진혹병리정,기중2례합병강극성뇌경사,2례합병척수형경추병,3례위KD원발성TST이상.결론 긍니적병가이존재상운동신경원적아림상손해,동시필수배제추체속손해적기타원인.
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.
