Phosphorus magnetic resonance spectroscopy (31P MRS) in neuromuscular disorders.
Phosphorus magnetic resonance spectroscopy monitors muscle energy metabolism by recording the ratio of phosphocreatine to inorganic phosphate at rest, during exercise, and during recovery from exercise. In mitochondrial diseases, abnormalities may appear during some or all these phases. Low phosphocreatine-inorganic phosphate ratios at rest are not disease-specific, but can be increased by drug therapy in several myopathies. Phosphorus magnetic resonance spectroscopy can also record intracellular pH and thus identify disorders of glycogen metabolism in which the production of lactic acid is blocked during ischemic exercise. The measurements of accumulated sugar phosphate intermediates further delineate glycolytic muscle defects. Myophosphorylase deficiency responds to intravenous glucose administration with improved exercise bioenergetics, but no such response is seen in phosphofructokinase deficiency. The muscular dystrophies show no specific bioenergetic abnormality; however, elevation of phospholipids metabolites and phosphodiesters was detected in some cases. While phosphorus magnetic resonance spectroscopy remains primarily a research tool in metabolic myopathies, it will be clinically useful in identifying new therapies and monitoring their effects in a variety of neuromuscular disorders.
Argov
Z
Z
Department of Neurology, Hadassah University Hospital, Jerusalem, Israel.
Bank
W J
WJ
D004734:Energy Metabolism; D015444:Exercise; D005947:Glucose; D006003:Glycogen / Q000378:metabolism; D006019:Glycolysis; D006801:Humans; D006863:Hydrogen-Ion Concentration; D009682:Magnetic Resonance Spectroscopy; D008931:Mitochondria, Muscle / Q000201:enzymology; D009119:Muscle Contraction; D009126:Muscle Relaxation; D009132:Muscles / Q000378:metabolism*; D009136:Muscular Dystrophies / Q000378:metabolism; D009468:Neuromuscular Diseases / Q000188:drug therapy / Q000378:metabolism*; D010085:Oxidative Phosphorylation; D010710:Phosphates / Q000032:analysis*; D010725:Phosphocreatine / Q000032:analysis*; D010732:Phosphofructokinase-1 / Q000172:deficiency; D010759:Phosphorus Isotopes; D006005:Phosphorylases / Q000172:deficiency
D010710:Phosphates; D010759:Phosphorus Isotopes; D010725:Phosphocreatine; D006003:Glycogen; D006005:Phosphorylases; D010732:Phosphofructokinase-1; D005947:Glucose