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Basal metabolic rate in myotonic dystrophy

Evidence against hypometabolism

Departments of Neurology and Medicine, University of Rochester, Rochester, NY.

We studied the basal metabolic rate in 13 males with myotonic dystrophy and 14 normal male subjects. Basal 0₂ consumption (Vo₂) and CO₂ production (Vco₂) were measured by direct gas analysis with a ventilated hood system. Lean body mass was estimated by total body potassium (⁴⁰K method) and muscle mass by urine creatinine excretion. The basal metabolic rate of patients with myotonic dystrophy was significantly reduced when related to surface area (—9%), but elevated when related to lean body mass (+17%). Previous reports of hypometabolism in myotonic dystrophy did not take into account the amount of muscle wasting in this disorder, and we conclude that in myotonic dystrophy the basal metabolic rate is actually elevated when corrected for lean body mass.

Address correspondence and reprint requests to Dr. Jozefowicz, Department of Neurology, University of Rochester School of Medicine and Dentistry, 601 Elmwood Avenue, Box 673, Rochester, NY 14642.

Presented in part at the thirty-eighth annual meeting of the American Academy of Neurology, New Orleans, LA, April 1986.

Supported in part by grants from the USPHS Division of Research Resources (RR 00044), the National Institutes of Health (AM 32562 and NS22099), and the Muscular Dystrophy Association. Dr. Jozefowicz waa supported by a Clinical Fellowship from the Muscular Dystrophy Association.

Received June 17, 1986. Accepted for publication in final form September 5, 1986.

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