目的　报道一家系 2例遗传性压迫易感性神经病(HNPP) ,以提高对本病的认识及诊断水平。方法　2例均行详细肌电图、运动及感觉神经传导速度、运动神经远端潜伏期测定。1例行腓肠神经活检 ,标本分别在光镜或电镜下观察。结果　例 1临床表现为反复的压迫或牵拉后肢体无力和麻木。例 2为例 1父亲 ,临床无发病 ,查体有周围神经病表现。 2例电生理检查示广泛性神经传导速度减慢 ,特别是周围神经嵌压部位运动传导速度减慢更明显 ,运动神经远端潜伏期延长 ,包括临床未受累的神经。腓肠神经活检电镜见大多数有髓纤维髓鞘增厚 ,有的髓鞘向轴索内突出 ,轴索未见异常。无葱皮样改变。无髓纤维未见显变。结论　神经电生理检查是诊断HNPP重要的筛选手段 ,确诊有赖于腓肠神经活检的典型病理表现 Objective To report a family of 2 cases of hereditary compression of susceptibility neuropathy (HNPP), in order to improve the understanding of the disease and the level of diagnosis. Methods Two patients underwent detailed electromyography, motor and sensory nerve conduction velocity, motor nerve distal latency determination. A routine sural nerve biopsy specimens were observed under light or electron microscopy. Results of the clinical manifestations of 1 cases of repeated compression or traction after hind limb weakness and numbness. Example 2 as an example 1 father, clinical no disease, physical examination showed peripheral neuropathy. Two cases of electrophysiological examination showed that the general nerve conduction velocity slowed down, especially the peripheral nerve entrapment site movement conduction velocity slowed down more obvious, prolonged motor nerve distal latency, including the clinical involvement of the nerves. Sural nerve biopsy electron microscopy showed the majority of myelinated fiber myelin thickening, and some myelin protruding to the axon, axonal no abnormalities. No skin change onions. No changes in myelinated fibers. Conclusion Neuroelectrophysiological examination is an important screening tool for the diagnosis of HNPP. The diagnosis depends on the typical pathological findings of sural nerve biopsy