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Previous Volume 292:1269-1273 June 12, 1975 Number 24 Next

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Abstract

Glycine concentrations were measured in plasma and cerebrospinal fluid of five patients in different types of hyperglycinemia to determine why severe neurologic deterioration is confined to the so-called nonketotic form of hyperglycinemia. Glycine content and glycine-cleavage enzyme activity were also determined in brain obtained in autopsy from three of these patients. Spinal-fluid glycine concentrations were 15 to 30 times above normal in patients with nonketotic hyperglycinemia, but were normal in those with hyperglycinemias of undetermined type who had comparable elevations of plasma glycine. Glycine content was two to four times above normal in several brain regions, and brain glycine cleavage enzyme activity was absent in two patients dying of nonketotic hyperglycinemia. By contrast, glycine content was normal and glycine cleavage activity present in the brain of an infant who died of hyperglycinemia of unknown cause. These results suggest that elevated glycine levels may be harmless in blood, but lethal in brain.

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• Jackson, A. H., Applegarth, D. A., Toone, J. R., Kure, S., Levy, H. L. (1999). Atypical Nonketotic Hyperglycinemia With Normal Cerebrospinal Fluid to Plasma Glycine Ratio. J Child Neurol 14: 464-467 [Abstract]  
• Sankaran, K., Casey, R.E., Zaleski, W.A., Mendelson, I.M. (1982). Glycine Encephalopathy in a Neonate: Treatment with Intravenous Strychnine and Sodium Benzoate. CLIN PEDIATR 21: 636-637  
• Frazier, D. M., Summer, G. K., Chamberlin, H. R. (1978). Hyperglycinuria and Hyperglycinemia in Two Siblings With Mild Developmental Delays. Arch Pediatr Adolesc Med 132: 777-781 [Abstract]  
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