脊髓延髓性肌萎缩症的肌电生理特点

目的：探讨脊髓延髓性肌萎缩症（SBMA）的电生理特点。方法：总结并回顾分析了7例SBMA患者的神经传导和针极肌电图的特点，并以肌萎缩侧索硬化（ALS）患者作为对照组，与SBMA组进行比较。结果：（1）SBMA组复合肌肉动作电位（CMAP）异常的患者和神经的出现率均明显低于ALS组（P〈0.01）。（2）7例SBMA患者均存在感觉神经传导异常，且上肢感觉神经动作电位（SNAP）异常的出现率高于下肢（P〈0.05）。（3）SBMA组自主电位的出现率显著低于ALS组，而巨大电位的出现率显著高于ALS组（P〈0.01）。结论：SBMA的神经肌肉电生理改变具有特征性，可以作为鉴别诊断的重要依据。

Electrophysiological Features of Spinal-bulbar Muscular Atrophy

Objective：To observe the electrophysiological characteristics of spinal-bulbar muscular atrophy （SBMA）. Method：7 cases of SBMA were observed and analyzed on their nerve conduction studies and needle EMG retrospectively.The SBMA cases were taken as test group and ALS patients were taken as control.Result：（1）Incidence of abnormal amplitudes of compound muscle action potentials（CMAPs）was lower in SBMA group than that in ALS cases with statistical significance.（2）All of 7 SBMA patients presented abnormal sensory conduction. Among them,abnormalities of sensory nerve action potential（SNAP）in upper limbs were higher than in lower limbs（P〈0.05）.（3）Spontaneous activities were less often seen in SBMA cases than in ALS cases,while motor unit action potentials with large amplitude （〉5 mV）were more often in SBMA group（P〈0.01）. Conclusion：The electrophysiological feature of SBMA may help the diagnosis and differential diagnosis in clinical practice.