Benstead T J, Kuntz N L, Miller R G, Daube J R
Section of Electromyography, Mayo Clinic, Rochester, Minnesota 55905.
Muscle Nerve. 1990 Jul;13(7):586-92. doi: 10.1002/mus.880130705.
Electrophysiologic studies in 11 patients with Dejerine-Sottas disease (hereditary motor and sensory neuropathy type III, HMSN III) showed median and ulnar motor nerve conduction velocities less than 6 m/sec in all but 1 patient. Marked temporal dispersion without conduction block was present in all patients. Uniform slowing in adjacent motor nerves was consistent with other studies of inherited neuropathies, although marked temporal dispersion may make HMSN III more difficult to distinguish from acquired neuropathies than other hereditary conditions. The electrophysiologic features of HMSN III patients were significantly different from a series of patients with other hereditary neuropathies chosen because of very slow nerve conduction velocity.
对11例德热里纳 - 索塔斯病（遗传性运动和感觉神经病III型，HMSN III）患者进行的电生理研究显示，除1例患者外，其余所有患者的正中神经和尺神经运动神经传导速度均低于6米/秒。所有患者均存在明显的时间离散但无传导阻滞。相邻运动神经的均匀减慢与其他遗传性神经病的研究结果一致，尽管明显的时间离散可能使HMSN III比其他遗传性疾病更难与获得性神经病相区分。HMSN III患者的电生理特征与一系列因神经传导速度非常慢而入选的其他遗传性神经病患者有显著差异。
