Meier C, Roberts K, Steck A, Hess C, Miloni E, Tschopp L
Acta Neuropathol. 1984;64(4):297-307. doi: 10.1007/BF00690395.
A case of progressive polyneuropathy associated with Waldenström's macroglobulinaemia is reported. A monoclonal IgM-lambda gradient was detected in the serum and cerebro-spinal fluid. By electro-immunoblot analysis antibodies against myelin-associated glycoprotein were found in the serum and cerebro-spinal fluid. The motor and sensory conduction velocities of several peripheral nerves were markedly decreased, and examination of visual evoked potentials (VEPs) revealed pathological latencies. Sural nerve biopsies before and after treatment with chlorambucil and plasmapheresis showed nerve fibre loss and demyelination. In the pre-treatment biopsy, heavy accumulations of filamentous material were found which stained positively for IgM by immuno-cytochemistry. Such accumulations had disappeared in a biopsy performed after treatment. The morphological findings were correlated with an improvement of clinical and electro-physiological findings.
报告了1例与华氏巨球蛋白血症相关的进行性多神经病病例。在血清和脑脊液中检测到单克隆IgM-λ梯度。通过电免疫印迹分析，在血清和脑脊液中发现了抗髓鞘相关糖蛋白抗体。多条周围神经的运动和感觉传导速度明显降低，视觉诱发电位（VEP）检查显示潜伏期异常。用苯丁酸氮芥和血浆置换治疗前后的腓肠神经活检显示神经纤维丢失和脱髓鞘。在治疗前的活检中，发现丝状物质大量积聚，免疫细胞化学显示其IgM染色呈阳性。在治疗后的活检中，此类积聚物已消失。形态学发现与临床和电生理结果的改善相关。
