肯尼迪病的临床及肌电图分析

目的总结肯尼迪病的临床特征及肌电图表现,为早期诊断提供依据。方法回顾性分析2016年6月至2019年4月于中国医科大学附属盛京医院就诊的5例肯尼迪病患者的资料,所有患者均行肌电图检查。结果本组病例于就诊时均伴有肢体无力表现,但首发症状不尽相同。多数病例于就诊时临床症状主要表现为蹲起不能、上楼费力、行走困难、双上肢上举困难等。肌电图检查:4例患者出现感觉神经不同程度的波幅降低甚至不能引出,速度正常或轻度减慢。1例患者感觉神经传导速度及波幅正常。4例患者存在运动神经传导波幅降低,速度正常或轻度减慢。1例患者出现正中神经、胫神经F波出现率降低。5例患者四肢肌肉静止时可见少量自发电位,轻收缩及大力募集时存在广泛神经源性损害。肌电图检查时神经传导出现以感觉神经波幅受累为主,肌电图检查自发电位少,伴有肌肉神经源性损害时,结合相关临床表现,考虑肯尼迪病的可能。结论肌电图检查是一项方便、快捷的检查,对肯尼迪病可以起到很好的诊断及鉴别诊断的作用。

Clinical and electromyographic analysis of Kennedy's disease

ObjectiveTo summarize the clinical and electromyographic features of Kennedy’s disease to provide evidence for early diagnosis. MethodsA retrospective analysis was performed on five patients with Kennedy’s disease who were treated at Shengjing Hospital Affiliated to China Medical University from June 2016 to April 2019. All patients underwent electromyography. ResultsThe patients in this group were accompanied by limb weakness at the time of presentation, but the initial symptoms were different. In most cases, the clinical symptoms at the time of presentation are mainly inability to lift, difficulty in going upstairs, difficulty in walking, and difficulty in lifting the upper limbs. Electromyographic analysis showed that in four patients, the amplitude of the sensory nerves decreased or cannot lead out in different level, and the speed was normal or slightly slowed down. There was no abnormal abnormality in sensory nerve conduction velocity and amplitude in one patient. Four patients had a decrease in motor nerve conduction amplitude, and the rate was normal or slightly slowed. In one patient, the incidence of F-waves in the median nerve and tibial nerve was reduced. Five patients had a little bit of spontaneous activity when the muscles of the limbs were at rest, and large motor unit action potentials during light contraction and heavy contraction. When the above clinical and electromyographic manifestations are present, the possibility of Kennedy’s disease should be considered. ConclusionElectromyography is a convenient and rapid examination and can play an appreciated role in the diagnosis and differential diagnosis of Kennedy’s disease.