Ikeda S, Yanagisawa N, Hongo M, Ito N
J Neurol Sci. 1987 Jun;79(1-2):129-39. doi: 10.1016/0022-510x(87)90267-x.
To clarify the cause of gastrointestinal disorders in systemic amyloidosis we made pathologic and morphometric studies of vagus nerves, celiac ganglia, stomach and rectum in three autopsied cases with type 1 familial amyloid polyneuropathy (FAP) and two with nonhereditary generalized amyloidosis (AL-amyloidosis). The gastric and rectal walls in all cases were affected in the same way by amyloid deposition. On the other hand, there was a great difference between the two diseases in the severity of vagus nerve and celiac ganglion lesions: the vagus nerves in FAP showed very extensive endoneurial deposition of amyloid with severe loss of myelinated nerve fibers, but in AL-amyloidosis there was no loss of myelinated nerve fibers and only slight amyloid deposition in the endoneurium. Similarly, in the celiac ganglion, intraganglionic deposition of amyloid was prominent in FAP and slight in AL-amyloidosis. It is known that bowel symptoms frequently occur in type I FAP and are less prominent in AL-amyloidosis. This study demonstrated that the gastrointestinal autonomic nerves were more markedly disturbed by amyloid in the former than in the latter, and disorder in neural control of the digestive tract may be responsible for the bowel symptoms in systemic amyloidosis, especially in type I FAP.
为明确系统性淀粉样变性中胃肠道紊乱的病因，我们对3例1型家族性淀粉样多神经病（FAP）尸检病例和2例非遗传性全身性淀粉样变性（AL淀粉样变性）病例的迷走神经、腹腔神经节、胃和直肠进行了病理及形态计量学研究。所有病例的胃壁和直肠壁受淀粉样蛋白沉积的影响方式相同。另一方面，两种疾病在迷走神经和腹腔神经节病变的严重程度上存在很大差异：FAP中的迷走神经显示神经内膜有非常广泛的淀粉样蛋白沉积，有髓神经纤维严重丧失，但在AL淀粉样变性中，有髓神经纤维没有丧失，神经内膜中只有轻微的淀粉样蛋白沉积。同样，在腹腔神经节中，神经节内淀粉样蛋白沉积在FAP中很突出，而在AL淀粉样变性中很轻微。众所周知，肠道症状在I型FAP中经常出现，而在AL淀粉样变性中则不那么突出。本研究表明，胃肠道自主神经在前一种疾病中比在后一种疾病中更明显地受到淀粉样蛋白的干扰，消化道神经控制紊乱可能是系统性淀粉样变性，尤其是I型FAP中肠道症状的原因。
