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Physiologic assessment of phosphoglycerate mutase deficiency

Incremental exercise tests

Department of Neurology of the Ohio State University College of Medicine and the Department of Exercise Physiology, Ohio State University, Columbus, OH; and the H. Houston Merritt Clinical Research Center for Muscular Dystrophy and Related Diseases, Columbia University College of Physicians and Surgeons, New York.

A third case of phosphoglycerate mutase (PGAM) deficiency, a metabolic myopathy involving terminal glycolysis, was identified in a 24-year-old black man with episodic, exercise-induced myoglobinuria since age 13. To better understand the physiologic consequences of PGAM deficiency, incremental exercise testing was performed. Results were compared with those of two patients having myophosphorylase deficiency and five normals. In contrast to the patients with phosphorylase deficiency, the PGAM-deficient patient achieved near-normal levels of maximal exercise and produced a normal peak lactate after exercise. The mechanisms underlying the asymptomatic performance of such strenuous exercise in this case are uncertain, but the data suggest that unidentified factors are operative in precipitating attacks of myoglobinuria in patients with some metabolic myopathies. Despite similar clinical histories, patients with different glycolytic enzyme deficiencies can have striking differences in exercise tolerance.

Address correspondence and reprint requests to Dr. Kissel, 1655 Upham Drive, Columbus, OH 43210.

Dr. Kissel is the recipient of a postdoctoral research fellowship from the Muscular Dystrophy Association

Accepted for publication October 1, 1984

This article has been cited by other articles:

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