Phosphorus magnetic resonance spectroscopy (31P MRS) in neuromuscular disorders
- 1 July 1991
- journal article
- review article
- Published by Wiley in Annals of Neurology
- Vol. 30 (1), 90-97
- https://doi.org/10.1002/ana.410300116
Abstract
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.Keywords
This publication has 52 references indexed in Scilit:
- Detection of abnormal calf muscle metabolism in patients with heart failure using phosphorus-31 nuclear magnetic resonanceThe American Journal of Cardiology, 1988
- Alterations of skeletal muscle metabolism in humans studied by phosphorus 31 magnetic resonance spectroscopy in congestive heart failureThe American Journal of Cardiology, 1988
- Metabolic changes in human muscle denervation: Topical31P NMR spectroscopy studiesMagnetic Resonance in Medicine, 1988
- Detection of muscle injury in humans with 31‐P magnetic resonance spectroscopyMuscle & Nerve, 1988
- McArdle's disease heterozygotes. Metabolic adaptation assessed using 31P-nuclear magnetic resonance.JCI Insight, 1986
- 31P-NMR Studies on an animal model of human defective muscle glycolysisMuscle & Nerve, 1986
- In vivo P-NMR spectroscopy: Muscle energy exchange in paramyotonia patientsMuscle & Nerve, 1985
- 31P-NMR studies of normal and dystrophic chicken muscleMuscle & Nerve, 1984
- EXAMINATION OF A MYOPATHY BY PHOSPHORUS NUCLEAR MAGNETIC RESONANCEThe Lancet, 1981
- Examination of a Case of Suspected McArdle's Syndrome by31P Nuclear Magnetic ResonanceNew England Journal of Medicine, 1981