RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164068418 T1 Duchenne Muscular Dystrophy T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accesspediatrics.mhmedical.com/content.aspx?aid=1164068418 RD 2024/10/07 AB Inherited and progressive myopathy affecting boys. Affected individuals present with muscular, respiratory, and cardiac disease. The cardiac and orthopedic complications (ie, scoliosis) are present in most cases. The presence of myotonia/hypotonia usually begins around the age of four in boys and worsens rapidly. The muscle groups affected are typically upper legs and pelvis followed by the upper arms. Young boys affected are unable to walk by the age of 12 years. Death usually occurs in the twenties due to respiratory muscle weakness or cardiomyopathy. Intellectual disability is often associated. Females may show mild symptoms but maintain a normal life expectancy.