Neuromuscular disorders associated with apical hypertrophic cardiomyopathy.

摘要

BACKGROUND: Cardiomyopathy is a frequent manifestation of neuromuscular disorders. AIMS AND OBJECTIVES: The aim of this study was to investigate how often apical hypertrophic cardiomyopathy (AHC) is associated with neuromuscular disorders (NMDs), which types of NMDs are associated with AHC, and if the outcome of patients with NMD and AHC is different from those with AHC but without a NMD. METHOD: Literature review by means of a Medline (PubMed) search for the terms AHC, apical hypertrophy, NMD, myopathy, muscular dystrophy, skeletal muscle, and cardiac involvement. RESULTS: AHC is only rarely associated with NMDs. So far,AHC has been described in patients with limb girdle muscular dystrophy, glycogen storage disease, mitochondrial disorder, metabolic myopathy, myopathy of unknown significance, eosinophilia-myalgia syndrome, or arthrogryposis multiplex congenita. The outcome of these patients was not at variance from those with AHC but without a NMD. CONCLUSIONS: AHC is only rarely associated with NMDs,such as limb girdle muscular dystrophy, glycogen storage disease, metabolic myopathy, myopathy of unknown significance, or eosinophilia-myalgia syndrome. The rare association of NMDs with AHC might be due to absence of systematic neurologic investigations of patients with AHC and vice versa.