Yoshita Mitsuhiro, Ishida Chiho, Yanase Daisuke, Yamada Masahito
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Kanazawa.
Intern Med. 2006;45(3):159-62. doi: 10.2169/internalmedicine.45.1151. Epub 2006 Mar 1.
Myasthenic symptoms and the echocardiographic findings of dilated cardiomyopathy are very rare in primary AL amyloidosis. We report a 59-year-old man with dyspnea on effort and weakness after exercise. His electrocardiogram showed ischemic heart disease and echocardiography indicated dilated cardiomyopathy. Muscle biopsy revealed amyloidosis with deposits of lambda light chain-derived amyloid within the vessel wall. Treatment with PGE1 resulted in improvement of the myasthenic symptoms. This patient indicates that myasthenic symptoms and dilated cardiomyopathy would be a unique syndrome associated with systemic AL amyloidosis involving mainly the small vessels, i.e., AL amyloid angiopathy, in the skeletal muscles and myocardium vessels.
肌无力症状和扩张型心肌病的超声心动图表现在原发性AL淀粉样变性中非常罕见。我们报告一名59岁男性，运动时出现呼吸困难和运动后乏力。他的心电图显示有缺血性心脏病，超声心动图提示扩张型心肌病。肌肉活检显示为淀粉样变性，血管壁内有λ轻链衍生的淀粉样蛋白沉积。使用前列腺素E1治疗后肌无力症状有所改善。该患者表明，肌无力症状和扩张型心肌病可能是一种与主要累及骨骼肌和心肌血管中小血管的系统性AL淀粉样变性相关的独特综合征，即AL淀粉样血管病。
