CAJ | 학술논문

目的通过观察神经电生理改变特点,协助鉴别肯尼迪病(KD)和进行性肌萎缩(PMA).方法收集13例KD和12例PMA患者,对两组患者运动和感觉神经传导、常规肌电图、单纤维肌电图结果进行比较.结果同侧肢体正中神经(拇短展肌记录)和尺神经(小指展肌记录)远端复合肌肉动作电位(CMAP)波幅比值在KD组和PMA组分别为1.11±0.40、0.57±0.29(t=4.760,P=0.000).6例KD患者感觉神经传导测定显示波幅下降,传导速度正常.PMA患者感觉传导测定均正常.单纤维肌电图检测可见,KD组和PMA组平均颤抖(jitter)分别为(56.3±24.2)、(97.1±35.3)μs(t=2.696,P=0.015),阻滞百分比中位数(M_(50))分别为0、32.5%(Z=3.168,P=0.002).两组肌电图检查均表现为广泛神经源性损害.结论 KD和PMA进行鉴别时,如运动传导显示正中神经CMAP波幅大于尺神经波幅,感觉神经传导波幅下降,单纤维肌电图颤抖相对正常,可以支持KD的诊断.
목적 통과관찰신경전생리개변특점,협조감별긍니적병(KD)화진행성기위축(PMA).방법 수집13례KD화12례PMA환자,대량조환자운동화감각신경전도、상규기전도、단섬유기전도결과진행비교.결과 동측지체정중신경(무단전기기록)화척신경(소지전기기록)원단복합기육동작전위(CMAP)파폭비치재KD조화PMA조분별위1.11±0.40、0.57±0.29(t=4.760,P=0.000).6례KD환자감각신경전도측정현시파폭하강,전도속도정상.PMA환자감각전도측정균정상.단섬유기전도검측가견,KD조화PMA조평균전두(jitter)분별위(56.3±24.2)、(97.1±35.3)μs(t=2.696,P=0.015),조체백분비중위수(M_(50))분별위0、32.5%(Z=3.168,P=0.002).량조기전도검사균표현위엄범신경원성손해.결론 KD화PMA진행감별시,여운동전도현시정중신경CMAP파폭대우척신경파폭,감각신경전도파폭하강,단섬유기전도전두상대정상,가이지지KD적진단.
Objective To assess the utility of electrophysiological studies in differential diagnosis of Kennedy's disease(KD)and progressive muscular atrophy(PMA).Methods Thirteen patients with KD and 12 patients with PMA were recruited.The characteristic of onset and duration of disease,motor nerve conduction studies,sensory nerve conduction studies,electromyography and single fiber electromyography were compared between KD and PMA.Results Eleven patients with KD and 1 patient with PMA presented weakness in both lower limbs at onset.The mean compound muscle action potential(CMAP)amplitude ratio of abductor pollicis brevis/abductor digiti minimi in the same side were 1.11±0.40 in KD and 0.57±0.29in PMA(t=4.760,P=0.000).Decreased amplitude of sensory nerve action potential was detected in 6 patients with KD,with bilateral median and ulnar nerve involved in 3 patients,unilateral ulnar nerve involved in 1 patient,bilateral median,ulnar,tibial and peroneal nerve involved in 2 patients.The sensory nerve conduction studies were normal in all patients with PMA.Single fiber electromyography showed that mean jitter was(56.3±24.2)μs in KD and(97.1±35.3)μs in PMA(t=2.696,P=0.015),M_(50) of the percentage of block wag 0 in KD and 32.5% in PMA(Z=3.168,P=0.002).All patients with KD and PMA had extensive neumgenic changes in electromyography study.Conclusions When patient presents symmetric proximal weakness in bilateral lower limbs at onset,nerve conduction studies show CMAP amplitude ratio of abductor pollicis brevis/abductor digiti minimi in the same side is more than 1,and decreased amplitude of sensory nerve action potential with normal conduction velocity.single fiber electromyography shows normal or mild increased mean jitter,the diagnosis of KD but not PMA should be considered.