Cardiomyopathy and angiopathy in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes

摘要: Abstract In four patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) in which mutated deoxyribonucleic acid was seen, hypertrophic cardiomyopathy angiopathy demonstrated by echocardiography, dipyridamole stress scintigraphy, cardiac catheterization. On scintigraphy dipyridamole, three showed hypoperfusion the early image a “filling-in” pattern late image. However, coronary angiography did not demonstrate narrowing of large vessels these patients. Light electron microscopy endomyocardial biopsy specimens indicated abnormal mitochondria, marked increase number size mitochondria endothelium. Modified Gomori's trichrome staining biopsied revealed red-purple deposit similar appearance ragged-red fibers skeletal muscle, characteristic finding disease. Deterioration complex I transfer system, is widely observed various diseases, appeared muscle our patients, indicating deficiency some subunits I. These results indicate that diseases such as MELAS show only but also angiopathy. We speculate proliferation leads to lumen arterioles, might be responsible for ischemic findings scintigraphically.