심근을 침범한 MELAS 증후군 1예

Abstract

MELAS syndrome is characterized by mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes, and cardiac involvement also frequently occurs. A 42-year-old female was admitted because of stroke like episodes and dyspnea. She had short status and hearing difficulty, and she complainded of general weakness. Brain MR revealed cortical sweeling and edema in temporal, parietal lobe and thalamus, which was not compatibe with stroke. Echocardiography revelaed hypertrophic left ventricular myocardium, resembling hypertrophic cardiomyopathy. Endomyocardial Biopsy confirmed myocardial involvement with MELAS syndrome.