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Symmetrical weakness started in the proximal lower limb muscles, and gradually upper limb muscles also became affected. Early contractures of the spine were absent. Contractures of elbows and Achilles tendons were either minimal or late. Serum creatine kinase activity was normal to moderately elevated. Electromyogram and muscle biopsy were consistent with a mild muscular dystrophy. Cardiological abnormalities, found in more than one\u2010half the patients, included dysrhythmias and atrioventricular (AV) conduction disturbances presenting as bradycardia, syncopal attacks necessitating pacemaker implantation, and sudden cardiac death. There was a significant relation between the severity of AV conduction disturbances and age. In nearly all patients, neuromuscular symptomatology preceded cardiological involvement. The early recognition of this previously not described, autosomal dominant LGMD with life\u2010threatening cardiac involvement offers an opportunity for therapeutic intervention.<\/jats:p>","DOI":"10.1002\/ana.410390513","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T16:36:26Z","timestamp":1104597386000},"page":"636-642","source":"Crossref","is-referenced-by-count":97,"title":["A newly recognized autosomal dominant limb girdle muscular dystrophy with cardiac involvement"],"prefix":"10.1002","volume":"39","author":[{"given":"A. J.","family":"van der Kooi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T. M.","family":"Ledderhof","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W. G.","family":"DeVoogt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. C. 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