Julien J, Vallat J M, Vital C, Coiffu B, Legendre P, Vallat M
Rev Neurol (Paris). 1975 Apr;131(4):229-41.
The authors describe two typical cases of Portuguese amyloid neuropathy in immigrants. One of the patients had been ill for only a short time while the other's condition had been developing over more than ten years. The first patient's neuropathy was characterized by a perforating ulcer of the foot and loss of sensation. The neurological disorder as well as the effect on general health were much more serious in the second patient. Neuro-muscular biopsy was carried out on each of these patients and revealed excessive endoneural amyloid and very severe lesions of nerve parenchyma mainly secondary to Wallerian degeneration. The results were compared with the few existing ultrastructural studies on the peripheral nerve in amyloid neuropathies.
作者描述了两例移民葡萄牙淀粉样变神经病的典型病例。其中一名患者患病时间较短,而另一名患者的病情已发展了十多年。第一名患者的神经病变表现为足部穿通性溃疡和感觉丧失。第二名患者的神经疾病以及对整体健康的影响更为严重。对这两名患者均进行了神经肌肉活检,结果显示神经内膜淀粉样蛋白过多,神经实质有非常严重的病变,主要继发于华勒变性。将结果与现有的少数关于淀粉样变神经病周围神经的超微结构研究进行了比较。