Subclinical Cardiomyopathy in Miyoshi Myopathy Detected by Late Gadolinium Enhancement Cardiac Magnetic Resonance Imaging

نویسندگان

چکیده

Dysferlin is a sarcolemmal protein present in muscle cells. It responsible for membrane repair. gene (DYSF) mutation, resulting deficiency this protein, termed dysferlinopathy. Clinically, it manifests as early adulthood onset of weakness with markedly elevated creatine kinase levels. The main phenotypes are limb-girdle muscular dystrophy type 2B (LGMD2B), affecting proximal muscles, and Miyoshi myopathy (MM), distal muscles. also cardiomyocytes, case reports have emerged cardiac abnormalities While routine methods screening, namely, electrocardiography or echocardiography, convenient noninvasive, they often exhibit insufficient diagnostic sensitivity detecting subclinical remodeling during stages cardiomyopathy. Cardiac magnetic resonance imaging though can provide accurate assessment chamber sizes function. With gadolinium administration, detect areas myocardial scarring fibrosis. Early diagnosis neuromuscular disease-related cardiomyopathy clinical significance, appropriate treatment retard fibrosis, delaying progression. We patient MM incidentally diagnosed concomitant

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ژورنال

عنوان ژورنال: International Heart Journal

سال: 2021

ISSN: ['1349-3299', '1349-2365']

DOI: https://doi.org/10.1536/ihj.20-354