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NEUROLOGY 1987;37:663
© 1987 American Academy of Neurology

Phosphorus NMR spectroscopy study of muscular enzyme deficiencies involving glycogenolysis and glycolysis

D. Duboc, MD, P. Jehenson, MD, S. Tran Dinh, PhD, C. Marsac, MD, A. Syrota, MD and M. Fardeau, MD

Service Hospitalier Frédéric Joliot (Drs. Jehenson, Tran Dinh, and Syrota), Departement de Biologie du C.E.A. Orsay, and the Croupe de Recherches Biologie et Pathologie Neurornusculaires, INSERM U. 153 (Drs. Duboc and Fardeau), Paris; and from INSERM U. 75 (Dr. Marsac), and the Faculté de Médecine Cochin Port Royal, Université Paris V, France.

We used phosphorus NMR spectroscopy to study 16 patients with muscular enzyme deficiencies affecting glycogenolysis and glycolysis. Study of phosphomonoester (Pm) kinetics and intracellular pH during exercise and recovery provided criteria for the distinction of these metabolic myopathies by NMR spectroscopy. The Pm peak was undetectable in patients lacking debrancher enzyme or phosphorylase. By contrast, in phosphofructokinase (PFK) or phosphoglycerate kinase (PGK) deficiency, the Pm peak was larger than that of inorganic phosphate in exercise, whereas it was always smaller in normal subjects. During recovery, the disappearance of Pm was slower in PGK than in PFK deficiency.

Address correspondence and reprint requests to Dr. Duboc, INSERM U. 153, 17 rue du Fer-à-Moulin, 75005 Paris, France.

Received March 5, 1986. Accepted for publication in final form August 6, 1986.




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