STUDIES ON CARBOHYDRATE METABOLISM IN AMYOTROPHIC LATERAL SCLEROSIS AND HEREDITARY PROXIMAL SPINAL MUSCULAR ATROPHY

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Abstract
The study includes 10 patients with amyotrophic lateral sclerosis, 5 patients with hereditary proximal spinal muscular atrophy, and 11 controls, in whom arterial and venous glycemia was studied in a certain muscle territory (region of the forearms and of the hands) before and after the administration of glucose, as well as the venous values of lactic acid, pyruvic acid, inorganic phosphate, serum potassium and serum aldolase. The following findings were made: Low glucose tolerance in 9 of the 15 patients studied. Normal fasting figures and normal dynamics for the arteriovenous difference in glucose, inorganic phosphate, serum potassium, pyruvic acid, lactic acid and serum aldolase. These data show that muscular carbohydrate metabolism is not altered in neurogenous atrophic diseases. In myogenous atrophic diseases (progressive muscular dystrophies) with the same biochemical investigations, a defective glucose assimilation and consumption could be revealed.