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    P.13.6 Nuclear Magnetic Resonance imaging and spectroscopy provide quantitative indices of disease severity in forearms of boys with Duchenne Muscle Dystrophy
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    Abstract: Ischemia‐induced changes in 31 P NMR relaxation were examined in 16 piglets. NMR spectra were acquired under control conditions and during complete cerebral ischemia induced via cardiac arrest. Changes in T 1 were assessed directly in six animals during control conditions and after 30–45 min of complete ischemia when changes in brain P 1 levels had reached a plateau. The T 1 for P 1 did not change, i.e., 2.3 ± 0.5 s during control conditions versus 2.4 ± 1.0 s during ischemia. To evaluate phosphocreatine and ATP, two types of spectra, with a long (25‐s) or short (1‐s) interpulse delay time, were collected during the first 10 min of ischemia (n = 10). Both types of spectra showed the same time course of changes in phosphocreatine and ATP levels, implying that the T 1 relaxation times do not change during ischemia. There were no changes in the linewidths of phosphocreatine, ATP, or P 1 during ischemia, implying that the T * 2 values remain constant. Our results suggest that the 31 P T 1 and T * 2 for phosphocreatine, P i , and ATP do not change during ischemia, and therefore changes in 31 P NMR peak intensity accurately reflect changes in metabolite concentrations.
    Phosphorus-31 NMR spectroscopy
    ABSTRACT 31 Phosphorus‐Nuclear Magnetic Resonance ( 31 P‐NMR) was used to evaluate degree of freshness of loach muscle depending on metabolic changes of high energy phosphate compounds. The phosphocreatine ([PCr])/inorganic phosphate ([Pi]) ratio was found to be a sensitive index of early metabolic hypofunction. Ratios of [PCr]/β‐phosphate of ATP ([β‐ATP]), and [Pi]/[β‐ATP] were demonstrated to be appropriate indexes to estimate such metabolic changes in fresh fish. The intramuscular levels of creatine phosphate, ATP and pH were maintained at higher levels in blood‐drained loach than in untreated fish. The freshness of fish which were bled and washed at lower temperatures were better preserved.
    Creatine
    The brain and skeletal muscle of eight adult patients with migraine with prolonged auras or migraine strokes leaving a permanent hemianopic defect were studied by phosphorus magnetic resonance spectroscopy. Biochemical assays performed on muscle biopsy and platelets had revealed abnormal mitochondrial enzyme activities. Brain magnetic resonance spectroscopy showed an abnormally low phosphocreatine to inorganic phosphate ratio in all patients, apparently due to decreased phosphocreatine and increased inorganic phosphate contents. Muscle phosphorus magnetic resonance spectroscopy showed low recovery from exercise in seven patients. Three patients had an increased phosphocreatine/inorganic phosphate ratio at rest, and the exercise transfer characteristics were abnormal in four patients for relatively low levels of exercise. The mitochondrial metabolic defects present in platelets and muscle of complicated migraine patients are therefore also expressed in the brain.
    To evaluate possible progressive metabolic changes in Duchenne muscular dystrophy, we used 31P nuclear magnetic resonance spectroscopy to measure high-energy phosphate compounds and phosphorylated diesters (PDE) in resting gastrocnemius muscle of 14 Duchenne patients and 10 normal boys. The patients had higher inorganic phosphate (Pi), intracellular pH, and PDE; and lower phosphocreatine (PCr) and PCr/Pi ratio; ATP was not significantly different. The patients showed significant age-related decreases in PCr and PCr/Pi, and increases in Pi and PDE, but ATP did not change. In normal boys, ATP increased with age, but PCr and Pi did not. These studies imply progressive metabolic deterioration in Duchenne dystrophy.
    Intracellular pH
    Gastrocnemius muscle
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