Dyck P James B, Windebank Anthony J
Peripheral Neuropathy Research Laboratory, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA.
Muscle Nerve. 2002 Apr;25(4):477-91. doi: 10.1002/mus.10080.
Diabetic lumbosacral radiculoplexus neuropathy (DLRPN) (also called diabetic amyotrophy) is a well-recognized subacute, painful, asymmetric lower-limb neuropathy that is associated with weight loss and type II diabetes mellitus. Nondiabetic lumbosacral radiculoplexus neuropathy (LRPN) has received less attention. Comparison of large cohorts with DLRPN and LRPN demonstrated that age at onset, course, type and distribution of symptoms and impairments, laboratory findings, and outcomes are similar. Both conditions are lumbosacral radiculoplexus neuropathies that are associated with weight loss and begin focally with pain but that evolve into widespread, bilateral paralytic disorders. Although both are monophasic illnesses, patients have prolonged morbidity from pain and weakness, and many patients become wheelchair-dependent. Although motor-predominant, there is unequivocal evidence that autonomic and sensory nerves are also involved. Cutaneous nerves from patients with DLRPN and LRPN show pathological evidence of ischemic injury (multifocal fiber loss, perineurial thickening and degeneration, neovascularization, microfasciculation, and swollen axons with accumulated organelles) and microvasculitis (mural and perivascular inflammation, separation and fragmentation of mural smooth muscle layers of microvessels and hemosiderin-laden macrophages). Controlled trials with immune-modulating therapies in DLRPN are in progress, and preliminary data suggest that such therapy may be beneficial in LRPN. It is likely that DLRPN and LRPN are immune-mediated neuropathies that should be separated from chronic inflammatory demyelinating polyneuropathy and from systemic necrotizing vasculitis.
糖尿病性腰骶神经根丛神经病（DLRPN）（也称为糖尿病性肌萎缩）是一种公认的亚急性、疼痛性、不对称性下肢神经病，与体重减轻和2型糖尿病相关。非糖尿病性腰骶神经根丛神经病（LRPN）受到的关注较少。对大量DLRPN和LRPN队列的比较表明，发病年龄、病程、症状和损伤的类型及分布、实验室检查结果和预后相似。这两种情况都是与体重减轻相关的腰骶神经根丛神经病，起初以局部疼痛开始，但会发展为广泛的双侧麻痹性疾病。虽然两者都是单相疾病，但患者因疼痛和无力而发病时间延长，许多患者需要依赖轮椅。虽然以运动为主，但有明确证据表明自主神经和感觉神经也受累。DLRPN和LRPN患者的皮神经显示出缺血性损伤（多灶性纤维丢失、神经束膜增厚和变性、新生血管形成、微束状改变以及含有细胞器聚集的轴突肿胀）和微血管炎（壁层和血管周围炎症、微血管壁层平滑肌层分离和破碎以及含铁血黄素巨噬细胞）的病理证据。针对DLRPN的免疫调节治疗的对照试验正在进行中，初步数据表明这种治疗可能对LRPN有益。DLRPN和LRPN可能是免疫介导的神经病，应与慢性炎症性脱髓鞘性多发性神经病和系统性坏死性血管炎区分开来。
