Said G, Slama G, Selva J
Brain. 1983 Dec;106 ( Pt 4):791-807. doi: 10.1093/brain/106.4.791.
A clinicopathological study is presented of 5 patients with a pseudosyringomyelic dissociation of sensory loss and severe autonomic disturbances related to diabetic polyneuropathy. Sensory loss which followed a pattern that suggested a length-related degeneration of fibres was associated with spontaneous pains in 3 patients. Plantar ulcers and neurogenic arthropathies were also present in 3 patients. The pathological changes in sural nerve biopsies included a severe loss of unmyelinated and small myelinated axons which appeared to be involved earlier than larger myelinated fibres. Distal degeneration of single fibres with subsequent axonal sprouting from the proximal axon was demonstrated in all patients. This severe axonal neuropathy was associated with primary and secondary segmental demyelination. Our observations are consistent with a progressive centripetal degeneration of axons in predominantly sensory diabetic polyneuropathy.
本文报告了5例患有与糖尿病性多发性神经病相关的假性脊髓空洞症感觉丧失和严重自主神经功能障碍患者的临床病理研究。感觉丧失呈纤维长度相关变性模式，3例患者伴有自发疼痛。3例患者还出现足底溃疡和神经源性关节病。腓肠神经活检的病理变化包括无髓鞘和小髓鞘轴突严重丧失，这些轴突似乎比大髓鞘纤维更早受累。所有患者均显示单纤维远端变性，随后近端轴突发芽。这种严重的轴索性神经病与原发性和继发性节段性脱髓鞘有关。我们的观察结果与主要为感觉性糖尿病性多发性神经病中轴突进行性向心性变性一致。
