6533b839fe1ef96bd12a61b1
RESEARCH PRODUCT
MUSCLE BIOENERGETIC ABNORMALITY IN MYOTONIC-DYSTROPHY - A SECONDARY MITOCHONDRIAL DISORDER
Pierre Kaminsky Brigitte Robin P. Fener P. Jouanny Paul Walker Marc Klein Jean Marie Escanyé J. Pourel Michel Ducsubject
musculoskeletal diseases[ INFO.INFO-IM ] Computer Science [cs]/Medical ImagingINVIVO[INFO.INFO-IM] Computer Science [cs]/Medical Imaging: MAGNETIC-RESONANCE SPECTROSCOPYEXERCISEMETABOLISMmusculoskeletal systemMUSCULAR-DYSTROPHYbody regionsMYOPATHYRAGGED-RED FIBERSNMR-SPECTROSCOPY[INFO.INFO-IM]Computer Science [cs]/Medical ImagingSKELETAL-MUSCLEP-31-NMRdescription
International audience; 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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 1993-01-01 |