AbstractA case study is reported regarding a 32‐year‐old man with classic clinical and electrophysiologic features of hereditary motor and sensory neuropathy, type I (HMSN I), a slowly progressive autosomal dominant condition marked by slow motor and sensory velocities and generalized segmental demyelination. Another clinically similar autosomal dominant neuropathy (HMSN II) is distinguished from HMSN I by nearly normal nerve conduction velocity. Acquired demyelinating neuropathy may occasionally resemble HMSN I clinically, but the former demonstrates electrophysiologic features not seen in the latter such as conduction block, dispersed compound muscle action potentials, and differential slowing of conduction velocity. Neuropathologic studies of HMSN I suggest that both neuronal and Schwann cell distrubances play a role in pathogene
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机译：摘要报道了一例32岁男性的病例，该男性具有遗传性运动和感觉神经病变（HMSN I）的典型临床和电生理学特征，这是一种缓慢进展的常染色体显性遗传病，其特征是运动和感觉速度缓慢以及全身节段性脱髓鞘。另一种临床相似的常染色体显性遗传性神经病变 （HMSN II） 与 HMSN I 的区别在于神经传导速度接近正常。获得性脱髓鞘性神经病变在临床上可能偶尔类似于 HMSN I，但前者表现出后者所不见的电生理特征，例如传导阻滞、分散的复合肌肉动作电位和传导速度的差异性减慢。HMSN I 的神经病理学研究表明，神经元和雪旺细胞分布在发病机制中起作用
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