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Physiologic and metabolic response to progressive and prolonged exercise in amyotrophic lateral sclerosis

Neurology Service (Drs. Sufit and Brooks) and the Metabolic and Lipid Research Laboratory (Drs. Paulson and Shug), William S. Middleton Memorial Veterans' Administration Hospital, and the Departments of Neurology (Drs. Smjak, Paulson, and Sufit, Ma. Beaulieu, Ms. Erickson, and Drs. Shug and Brooks) and Preventive Medicine (Drs. Sanjak, Paulson, and Reddan), ALS Clinical Research Center (Drs. Sanjak and Sufit, Ms. Beaulieu, Ms. Erickson, and Dr. Brooks), University of Wisconsin Medical School, Madison, WI.

Physical work capacity in 35 ALS patients and 6 untrained controls was evaluated during progressive bicycle ergometery. In the ALS patients, maximum oxygen consumption (VO₂max) and work capacity (Wmax) drop in relation to the decrease in ALS functional score. However, the oxygen cost (ml O₂/kpm) of submaximal exercise was significantly increased. Prolonged submaximal exercise tests in six ALS patients and six matched untrained controls indicated that the exercise-induced increase in plasma free fatty acids, beta-hydroxybuty-rate, esterified carnitine, and muscle esterified carnitine was significantly retarded in ALS patients.

Address correspondence and reprint requests to Dr. Brooks, Neurology Service (127), Wm. S. Middleton VA Hospital, 2500 Overlook Terrace, Madison, WI 53705.

Supported in part by research grants from the Muscular Dystrophy Association (B. R. B., R. L. S., A. S.), the Hamilton Roddis Foundation (B. R. B.), and the United States Public Health Service (A. S.).

Received February 7, 1986. Accepted for publication in final form October 23, 1986.

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