Pellegrini G, Scarlato G, Moggio M, Bettinelli C, Gaini S M
Acta Neuropathol Suppl. 1981;7:255-7. doi: 10.1007/978-3-642-81553-9_75.
The authors describe an adult onset sensorimotor polyneuropathy in a woman with light chain K-type myeloma. Neurophysiological investigation showed a severe reduction of conduction velocity of the peripheral nerve to the four limbs. A muscle and sural nerve biopsy were performed. Sural nerve displayed a severe loss of myelinated and unmyelinated fibers. No amyloid deposit was observed in vessels, perineurium and endomysium by electron microscopy examination. No onion bulb formation was present in nerve tissue, but many demyelinated fibers were surrounded by circumferentially disposed Schwann cell processes without any interposition of collagen fibrils. Some fibers exhibit signs of active remyelination; axonal alterations were rarely seen. Collagen pockets were frequently observed among proliferated collagen fibrils. The mechanism of peripheral nerve lesions in various categories of multiple myeloma is till now an unsolved problem. In this case the unusual association with a light chain myeloma suggest that these immunoglobulins play a role in the pathogenesis of the polyneuropathy as supported by experimental studies.
作者描述了一名患有轻链K型骨髓瘤的成年女性发生的成人起病的感觉运动性多发性神经病。神经生理学检查显示四肢周围神经传导速度严重降低。进行了肌肉和腓肠神经活检。腓肠神经显示有髓和无髓纤维严重丢失。电子显微镜检查未在血管、神经束膜和肌内膜中观察到淀粉样沉积物。神经组织中不存在洋葱球形成，但许多脱髓鞘纤维被沿圆周排列的施万细胞突起包围，没有任何胶原纤维插入。一些纤维表现出活跃的髓鞘再生迹象；很少见到轴突改变。在增生的胶原纤维中经常观察到胶原小窝。各类多发性骨髓瘤中周围神经病变的机制至今仍是一个未解决的问题。在这种情况下，与轻链骨髓瘤的不寻常关联表明，这些免疫球蛋白在多发性神经病的发病机制中起作用，实验研究也支持这一点。
