Disturbances of the combustion processes in muscular disorders

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Abstract
Chemical substances involved in carbohydrate metabolism were assayed in blood samples from 130 patients with muscular atrophy; progressive muscular dystrophy patients were included. The results were compared with 20 controls. The blood pyruvic acid content was significantly increased in progressive muscular dystrophy and elevated in most of the other muscular disorders studied. The levels of blood glucose, lactic acid, a-ketoglutaric acid and oxalacetic acid were unchanged in all groups of muscular disorders. Blood citric acid was lowered in progressive muscular dystrophy in contrast to the other types of disorders. Biochemical determinations of pyruvic acid and citric acid in relation to non-collagen N made in muscle biopsies from 10 patients with progressive muscular dystrophy and from 6 patients with neurogenic muscular wasting showed no significant differences in the muscular content of these compounds. However, there was an increase in both pyruvic and citric acid levels greater than twice the standard deviation in 3 of the 10 patients studied. Increased blood pyruvic acid levels with decreased blood citric acid levels in progressive muscular dystrophy might be due to the blocking of the pyruvic acid transfer into the Krebs Cycle. Oxidative decarboxylation may also be impaired. The remaining routes of carboxylations may be insufficient for oxidation of the excess of pyruvic acid. The pyruvic and citric acid levels in muscle biopsies of progressive muscular dystrophy were normal. Therefore, inhibition of the oxidative processes does not take place in muscle.