Summary
The concentrations of glucose, pyruvate and lactate were determined in the blood and lumbar CSF of 63 patients with neuromuscular disorders and 40 controls. The glucose/lactate and lactate/pyruvate ratios were also calculated, in addition to the cytoplasmic NADH/NAD+ (nicotinamide-adenine dinucleotide-H/nicotinamide-adenine dinucleotide) ratio in the brain. Remarkable deviations from control values were found not only in blood but also in the CSF of patients with different neuromuscular diseases, particularly in Duchenne muscular dystrophy and peroneal muscular atrophy, indicative of disturbance of the carbohydrate metabolism in the central nervous system.
Zusammenfassung
Die Konzentration von Glukose, Pyruvat und Laktat wurde im venösen Blut und lumbalen Liquor bei 63 Patienten mit neuromuskulären Erkrankungen und bei 40 Kontrollpatienten gemessen. Die Glukose/Laktat- und Laktat/Pyruvat-Quotienten, im weiteren der cerebrale cytoplasmatische NADH/NAD+-Quotient, wurden errechnet. Nicht nur im Blut, sondern auch im Liquor der neuromuskulären Patienten, u.a. bei Kranken mit Duchenne-Dystrophie und mit neuronaler peronealer Atrophie, konnten ausgeprägte Abweichungen von den Kontrollen gefunden werden. Diese weisen auf die Störung des Kohlenhydrat-Stoffwechsels im ZNS dieser Kranken hin.
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References
Astin KJ, Wilde GE, Davies-Jones AB (1975) Glucose metabolism and insulin response in the plasma and CSF in motor neurone disease. J Neurol Sci 25:205–210
Caughey JE, Brown J (1950) Dystrophia myotonica: an endocrine study. Quart J Med 19:303–309
Collis WJ, Engel WK (1968) Glucose metabolism in five neuromuscular disorders. Neurology 18:915–925
Cooper AC, Miller JR (1962) Progressive muscular dystrophy: a review. Rev canad Biol 21:337–351
Davson H (1976) The blood-brain barrier. J Physiol 255:1–28
Dreyfus JC, Schapira G, Schapira F (1954) Biochemical study of muscle in progressive muscular dystrophy. J Clin INvest 33:794–797
Dreyfus JC, Schapira G, Schapira F, Demos J (1956) Activités enzymatiques du muscle humain. Recherches sur la biochimie comparée de l'homme normal et myopathique et du rat. Clin chim Acta 1:434–448
Granholm L, Kaasik AE, Nilsson L, Siesjö BK (1968) The lactate/pyruvate ratios of cerebrospinal fluid of rats and cats related to the lactate/pyruvate, the ATP/ADP and the phosphocreatine/creatine ratios of brain tissue. Acta Physiol Scand 74:398–409
Granholm L (1969) Studies of the lactate/pyruvate system of brain and cerebrospinal fluid. Studentlitteratur, Lund
Hindfelt B (1978) On clinical diagnosis of metabolic encephalopathy. Acta Neurol Scand Suppl 61:191–204
Kolmogorow AN, Smirnow NV (1963) (cit Fisz M) Probability theory and mathematical statistics. Wiley, New York
Laudahn G, Heyck H (1965) Muskelenzym-Befunde bei progressiver Muskeldystrophie. In: Beckmann R (Hrsg) Myopathien. Thieme, Stuttgart, S 165–175
Lee FE, Hughes DTD (1964) Systemic affects in dystrophia myotonica. Brain 87:521–536
Moir ATB, Ashcroft GW, Crawford TBB, Eccleston D, Guldberg HC (1970) Cerebral metabolites in cerebrospinal fluid as a biochemical approach to the brain. Brain 93:357–368
Molnár L (1972) A központi idegrendszer energia-forgalmának zavarai ideg- és elmebetegségekben. Ideggyógy Szemle 25:483–493
Molnár L (1975) Prognostic value of carbohydrate metabolites in CSF.—“The luxury glucose supply” of the brain. In: Langfitt ThW et al (eds) Cerebral circulation and metabolism. Springer, Heidelberg, pp 518–519
Molnár L, Vámosi B (1980) Cytoplasmic redox changes in the brain of stroke patients.—“Luxury oxygenation” of the brain? In: Circulation cérébrale. Fournié, Toulouse, pp 129–131
Nadler CS, Steeger WA, Troncelleti M, Durant TM (1950) Dystrophia myotonica, with special reference to endocrine function (Klinefelter's syndrome). J Clin Endocr 10:630–638
Pennington RJT (1969) Biochemical aspects of muscle disease. In: Walton JN (ed) Disorders of voluntary muscle. Churchill, London, pp 385–410
Plum FJ, Posner JB (1967) Blood and cerebrospinal fluid lactate during hyperventilation. Am J Physiol 212:864–870
Roos A (1965) Intracellular pH and intracellular buffering power of the cat brain. Am J Physiol 209:1233–1246
Schapiro HM (1975) CSF ... Neural urine or more? Anesthesiology 42:647–650
Steinke J, Tyler HR (1964) The association of amyotrophic lateral sclerosis (motor neurone disease) and carbohydrate intolerance. A clinical study. Metabolism 13:1376–1381
Vignos PJ, Lefkowitz M (1959) A biochemical study of certain skeletal muscle constituents in human progressive muscular dystrophy. J Clin Invest 38:813–821
Walton JN, Latner AL (1954) Ribosuria in muscular dystrophy. Arch Neurol Psychiat (Chicago) 72:362–367
Welch BL (1937) The significance of the difference between two means when the population variances are unequal. Biometrika 29:350–362
Williamson DH, Lund P, Krebs HA (1967) The redox state of free nicotinamide-adenine dinucleotide in the cytoplasm and mitochondria of rat liver. Biochem J 103:514–527
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Mechler, F., Diószeghy, P., Csenkér, E. et al. Carbohydrate metabolites in the blood and CSF of patients with neuromuscular disorders. J Neurol 226, 111–118 (1981). https://doi.org/10.1007/BF00313438
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DOI: https://doi.org/10.1007/BF00313438