title: [Abnormalities of the muscular bioenergetics in Steinert's disease].
abstract: The thenar muscles and gastrocnemius of a patient with myotonic dystrophy were investigated, at rest, by phosphorus nuclear magnetic resonance spectroscopy. A decrease in phosphocreatine level and an increase in inorganic phosphate and phosphodiester levels were found in the gastrocnemius, which was clinically spared, whilst the thenar muscles, which were wasted and affected by myotonia, exhibited only an increased inorganic phosphate level and an elevated pH. These findings were comparable with those found in other muscular disorders, such as Duchenne's and Becker's dystrophies, as well as in limb girdle dystrophy. They suggested that the abnormalities observed were unrelated to myotonia or wasting, and the possibility of a secondary mitochondrial disorder in myotonic dystrophy, is to be considered.
authors.affiliation: Service de Médecine J, Centre Hospitalier Universitaire de Nancy, Hôpitaux de Brabois.
authors.forename: P
authors.initials: P
authors.lastname: Kaminsky
authors.forename: B
authors.initials: B
authors.lastname: Robin-Lherbier
authors.forename: P
authors.initials: P
authors.lastname: Fener
authors.forename: P
authors.initials: P
authors.lastname: Jouanny
authors.forename: P
authors.initials: P
authors.lastname: Walker
authors.forename: M
authors.initials: M
authors.lastname: Klein
authors.forename: J M
authors.initials: JM
authors.lastname: Escanyé
authors.forename: J
authors.initials: J
authors.lastname: Pourel
authors.forename: M
authors.initials: M
authors.lastname: Duc
mesh_terms: D000328:Adult; D004734:Energy Metabolism; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008931:Mitochondria, Muscle / Q000378:metabolism; D009132:Muscles / Q000378:metabolism; D009223:Myotonic Dystrophy / Q000378:metabolism*; D010758:Phosphorus
chemical_list: D010758:Phosphorus
vernacular_title: Anomalies de la bioénergétique musculaire dans une maladie de Steinert.