Discrimination of spinal and bulbar muscular atrophy from amyotrophic lateral sclerosis using sensory nerve action potentials
Discrimination of spinal and bulbar muscular atrophy from amyotrophic lateral sclerosis using sensory nerve action potentials
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DOI：
10.1002/mus.22291
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发表时间：
2012-02-01
期刊：
影响因子：
3.4
通讯作者：
Sobue, Gen
中科院分区：
文献类型：
--
作者：
Hama, Tetsuo;Hirayama, Masaaki;Sobue, Gen
前言：脊髓和延髓肌萎缩症(SBMA)和肌萎缩侧索硬化症(ALS)是运动神经元病。有时会观察到感觉障碍，并已描述在SBMA患者的感觉神经中有电生理参与。我们假设感觉神经传导检查(NCS)可以用于区分SBMA和ALS。方法：我们比较了120例经遗传分析证实的SBMA患者、188例ALS患者和50例正常人的NCS结果。结果：SBMA组的感觉神经动作电位(SNAP)波幅明显低于ALS组和对照组。SNAP波幅的受试者-操作特征曲线分析表明，正中切断值为13.8 mU V，尺神经切断值为10.7 mV，腓肠神经切断值为9.9 mU V是区分SBMA和ALS的最佳分界值。结论：SBMA中SNAP波幅的特异性降低为运动神经元病的鉴别诊断提供了另一种有用的工具。肌肉神经45：169-174,2012
Introduction: Spinal and bulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS) are motor neuron diseases. Sensory impairment is sometimes observed, and electrophysiological involvement has been described in the sensory nerves of SBMA patients. We hypothesized that a sensory nerve conduction study (NCS) could be used to discriminate SBMA from ALS.Methods: We compared the results from NCSs in a total of 120 SBMA cases confirmed by genetic analysis, 188 ALS cases, and 50 normal subjects. Results: Sensory nerve action potential (SNAP) amplitudes of the SBMA group were significantly lower than in the ALS and control groups. In addition, receiver-operating characteristic curve analysis for SNAP amplitude showed that a cut-off value of 13.8 mu V for median, 10.7 mu V for ulnar, and 9.9 mu V for sural nerve best discriminated SBMA from ALS. Conclusions: The specific decrease of SNAP amplitude in SBMA provides another useful tool for the differential diagnosis of motor neuron diseases. Muscle Nerve 45: 169-174, 2012