报告罕见的遗传性感觉神经根神经病4例及一个家系的调查结果。讨论诊断依据与鉴别、肢端毁损的机理、遗传方式、病因和发病机理。有以下特点和创见:(1)例3、例4除肢端毁损外,还有舌尖无痛性溃烂导致舌尖缺损;例3左眼角膜无痛性疱疹导致云翳;以往文献均未见报道。从基因遗传与个体发生的角度分析其病位应在与脊髓后根神经节相当的半月神经节,发病机理也与肢端感觉缺失一样。(2)本病的遗传方式据文献只有常显与常隐两 Report rare genetic hereditary sensory nerve root neuropathy in 4 cases and a pedigree findings. Discuss the diagnosis basis and identification, the mechanism of limb deformity, the way of inheritance, etiology and pathogenesis. There are the following characteristics and innovative findings: (1) Example 3, Example 4 In addition to the damaged limbs, there tongue painless ulceration leading to tongue tip defect; Example 3 Left corneal painless herpes cause cloud clam; none of the previous literature has been reported. From the perspective of genetic genetics and the occurrence of individual analysis of its position should be in the spinal ganglion corresponding to the ganglion ganglion, the pathogenesis of acral sensory loss is also the same. (2) The genetic mode of the disease, according to the literature only often and often hidden two