摘要
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（M5。）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。
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. 29 in 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）μ s in KD and （97. 1 ±35.3） μs in PMA（t =2. 696,P =0. 015）, M50 of the percentage of block was 0 in KD and 32. 5% in PMA（Z =3. 168, P =0. 002）. All patients with KD and PMA had extensive neurogenic 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.
出处
《中华神经科杂志》
CAS
CSCD
北大核心
2010年第3期204-206,共3页
Chinese Journal of Neurology
关键词
肌萎缩
脊髓性
肌电描记术
神经传导
诊断
鉴别
Muscular atrophy, spinal
Eleetromyography
Neural conduction
Diagnosis, differential
