Trotter J L, Engel W K, Ignaczak F I
Arch Neurol. 1977 Apr;34(4):209-14. doi: 10.1001/archneur.1977.00500160023003.
In ten previously undiagnosed patients, we have found erstwhile-"primary" nonhereditary amyloidosis as an overlooked cause of a predominately sensory, painful, and hyperesthetic distal neuropathy occurring in middle-age and older patients. These symptoms, associated with orthostatic hypotension, diarrhea or constipation, cardiac abnormality, and male impotence are virtually diagnostic (in the absence of diabetes mellitus). Tissue diagnosis is quickly made by crystal-violet metachromasia of amyloid in fresh-frozen sections of a muscle biopsy specimen. Immunoglobulin and bone marrow evidence of plasma cell dyscrasia in eight of the ten patients suggests that the neuropathy in this form of amyloidosis is actually secondary to a plasma-cell-originating dysproteinemia. Therapy with melphalan and prednisone was not of benefit.
在10例先前未确诊的患者中，我们发现既往被认为是“原发性”的非遗传性淀粉样变性病是中老年患者中一种被忽视的主要感觉性、疼痛性和感觉过敏的远端神经病的病因。这些症状与体位性低血压、腹泻或便秘、心脏异常以及男性阳痿相关，实际上具有诊断意义（在无糖尿病的情况下）。通过肌肉活检标本新鲜冰冻切片中淀粉样蛋白的结晶紫异染性可快速做出组织诊断。10例患者中有8例存在免疫球蛋白和浆细胞发育异常的骨髓证据，提示这种淀粉样变性病形式的神经病实际上继发于浆细胞源性的蛋白异常血症。美法仑和泼尼松治疗无效。
