Nadkarni N, Freimer M, Mendell J R
Department of Neurology, College of Medicine, Ohio State University, Columbus 43210, USA.
Muscle Nerve. 1995 Sep;18(9):1016-8. doi: 10.1002/mus.880180914.
A 62-year-old woman developed profound weakness secondary to a progressive myopathy associated with primary systemic amyloidosis. The characteristic apple-green birefringent amyloid deposits were demonstrated surrounding individual muscle fibers in Congo red stained sections. Electron microscopy demonstrated amyloid filaments in close apposition to muscle fibers exhibiting excessive corrugations of the sarcolemmal membrane. The pathological features of progressive amyloid myopathy associated with primary systemic amyloidosis are distinct from the intracellular amyloid deposits characteristic of sporadic inclusion body myositis and inherited inclusion body myopathy.
一名62岁女性因与原发性系统性淀粉样变性相关的进行性肌病而出现严重肌无力。在刚果红染色切片中，可见特征性的苹果绿色双折射淀粉样沉积物围绕着单个肌纤维。电子显微镜显示淀粉样细丝紧邻肌纤维，肌纤维的肌膜呈现过度的皱襞。与原发性系统性淀粉样变性相关的进行性淀粉样肌病的病理特征不同于散发性包涵体肌炎和遗传性包涵体肌病的细胞内淀粉样沉积物。
