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NEUROLOGY 1987;37:1720
© 1987 American Academy of Neurology

Muscle energy metabolism in McArdle's syndrome by in vivo phosphorus magnetic resonance spectroscopy

Zohar Argov, MD, William J. Bank, MD, John Maris, BS and Britton Chance, MD

Departments of Biochemistry/Biophysics (Dr. Argov, Mr. Maris, and Dr. Chance), University of Pennsylvania, and the Department of Neurology (Drs. Argov and Bank), Hospital of the University of Pennsylvania, Philadelphia, PA.

Five patients with McArdle's syndrome were examined by phosphorus magnetic resonance spectroscopy (31P-NMR). Adenosine triphosphate (ATP) levels at rest were reduced by 22%, but did not fall further during exercise or contracture. The slope of work rate versus inorganic phosphate/phosphocreatine (Pi/PCr) was 42 ± 8 joules/min/Pi/PCr in three patients without muscle wasting, compared with 13 and 16 in patients with atrophy (normal, 30 to 50 joules/min/Pi/PCr). Recovery from exercise showed similar rates in patients (postischemic exercise 1.03 ± 0.17, post-aerobic 1.63 ± 0.17 PCr/Pi units per minute) and controls (1.0 ± 0.2 and 1.8 ± 0.2, respectively) independent of intracellular pH. Infusion of glucose improved exercise kinetics by 163 to 190%, but an oral load of protein had no effect. We conclude that (1) muscle mitochondria operate normally in vivo in this glycogenolytic disorder, suggesting a sufficient alternate fuel supply. (2) Blood-borne glucose may serve as one alternate fuel for the "second wind" phenomenon. (3) ATP control mechanisms are altered only at rest. (4) Recovery from exercise is relatively pH-independent.

Address correspondence and reprint requests to Dr. Bank, Department of Neurology, Hospital of the University of Pennsylvania, Philadelphia, PA 19104.

Supported by grants from NIH (NS08075 and RR-02305) and the Muscular Dystrophy Association. Dr. Argov is a recipient of a Muscular Dystrophy Association postdoctoral fellowship. The Clinical Research Center of the Hospital of the University of Pennsylvania waa used for patients' care during the study.

Presented in part at the 110th meeting of the American Neurological Association, Chicago, IL, 1985, and the thirty-eighth annual meeting of the American Academy of Neurology, New Orleans, LA, April 1986.

Received November 25, 1986. Accepted for publication in final form February 3, 1987.






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