Fitting J W, Bischoff A, Regli F, De Crousaz G
J Neurol Neurosurg Psychiatry. 1979 Mar;42(3):193-202. doi: 10.1136/jnnp.42.3.193.
Three cases of neuropathy with monoclonal gammopathy and amyloid deposits in peripheral nerves are described. They appeared to present a benign gammopathy because of the duration of the neuropathy in the absence of any clinical or biological sign of myeloma or macroglobulinaemia. The pathological abnormality found in the sural nerves of the three patients was characterised by a marked loss of myelinated and unmyelinated nerve fibres because of active axonal degeneration with Wallerian degeneration. The most striking feature in all three cases was the finding of deposits identified as an accumulation of microfibrils.
本文描述了3例伴有单克隆丙种球蛋白病和周围神经淀粉样沉积物的神经病变。由于神经病变持续存在,且无骨髓瘤或巨球蛋白血症的任何临床或生物学迹象,这些病例似乎呈现出良性丙种球蛋白病。在3例患者的腓肠神经中发现的病理异常表现为,由于沃勒变性导致的轴突主动变性,有髓和无髓神经纤维明显缺失。所有3例中最显著的特征是发现沉积物被鉴定为微原纤维的积聚。