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作者回顾了1960年1月～1994年12月经肌肉活检诊断的淀粉样变性的病人1596例,其中12例确诊为原发性系统性淀粉样变性,同时肌肉中有淀粉样蛋白的沉积,对这12例病人进行了随访。 12例病人全部做了肌电图检查。10例有肌病的证据。2例有周围神经病而无肌病。2例显示腕管综合征。3例既有肌病又有周围神经病的证据。12例肌肉活检显示:4例在血管内和间质有淀粉样蛋白的沉积。2例为血管和内皮的沉积。4例仅在血管沉积。
The authors reviewed 1596 patients with amyloidosis diagnosed by muscle biopsy from January 1960 to December 1994, 12 of whom were diagnosed with primary systemic amyloidosis with amyloid deposition in the muscle Twelve patients were followed up. All 12 patients had an EMG examination. 10 cases of myopathy evidence. 2 cases of peripheral neuropathy without myopathy. 2 cases showed carpal tunnel syndrome. 3 cases of both myopathy and peripheral neuropathy evidence. Twelve muscle biopsies showed that four had amyloid deposition in the blood vessels and in the stroma. 2 cases of vascular and endothelial deposition. Only 4 cases of vascular deposition.