0000000001068600
AUTHOR
Marc Klein
MUSCLE BIOENERGETIC ABNORMALITY IN MYOTONIC-DYSTROPHY - A SECONDARY MITOCHONDRIAL DISORDER
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 obs…
MITOCHONDRIAL DISORDER SECONDARY TO INFLAMMATION IN POLYMYOSITIS - 2 CASES
National audience; Abstract: Two cases of polymyositis were followed using phosphorus nuclear magnetic resonance spectroscopy. The spectra recorded during remission were normal, but those collected from the gastrocnemius muscle during the active phase of the diseases showed an increased inorganic phosphate level or a decreased phosphocreatine content. The intracellular pH was normal. These findings may be related to an impairment in mitochondrial metabolism secondary to the inflammatory process. Moreover, the fact that the abnormalities observed disappeared after treatment suggests that phosphorus NMR spectroscopy could be used as a non-invasive method in the follow-up of polymyositis, but …