@article{23e8c3a0117f11df803f000ea68e967b,
title = "Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy",
abstract = "OBJECTIVE: To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD). DESIGN: Prospective screening. SETTING: Neuromuscular Clinic and Department of Cardiology at Rigshospitalet. Patients One hundred one patients with LGMD2A-I and BMD and 29 patients with LGMD2 and no molecular diagnosis. MAIN OUTCOME MEASURES: Clinical investigation, echocardiography, and electrocardiographic findings. RESULTS: Cardiac involvement was present in 24 of 100 patients (24%) with LGMD2A-I and in 14 of 30 patients (47%) with BMD. Only a few patients with LGMD2A and unclassified LGMD2 had mild cardiac involvement, whereas 29% and 67% of patients with LGMD2I and LGMD2E, respectively, had cardiac involvement. Cardiac involvement was not correlated with age, muscle strength, or the level of dystrophic changes on muscle biopsy. CONCLUSIONS: This study demonstrates a high prevalence of cardiac involvement in patients with LGMD2I, LGMD2E, and BMD. Patients with LGMD2A, LGMD2D, and unclassified LGMD2 have a much lower and milder prevalence of cardiac involvement.",
author = "Marie-Louise Sveen and Thune, {Jens Jakob} and Lars K{\o}ber and John Vissing",
note = "Keywords: Adolescent; Adult; Aged; Cardiovascular Diseases; Female; Humans; Male; Middle Aged; Muscular Dystrophies, Limb-Girdle; Muscular Dystrophy, Duchenne; Prospective Studies",
year = "2008",
doi = "10.1001/archneur.65.9.1196",
language = "English",
volume = "65",
pages = "1196--201",
journal = "JAMA Neurology",
issn = "2168-6149",
publisher = "The JAMA Network",
number = "9",
}