Neonatal type of nonketotic hyperglycinemia

Neonatal type of nonketotic hyperglycinemia Two infants with the neonatal type of nonketotic hyperglycinemia that had manifested as early neonatal consciousness disturbance are presented. Transient hyperammonemia had been detected in both initially. High levels of glycine in plasma and cerebrospinal fluid disturb the nervous system, causing variable manifestations of this disease. Both cases were complicated by intracranial hemorrhage, which has never before been reported. After treatment with sodium benzoate and dextromethorphan, some neurologic improvement was observed, although the glycine levels did not lower. Recent clinical trials are reviewed, and because of the unfavorable outcomes, the special need for prenatal diagnosis is highlighted. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Pediatric Neurology Elsevier

Neonatal type of nonketotic hyperglycinemia

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Publisher
Elsevier
Copyright
Copyright © 1999 Elsevier Science Inc.
ISSN
0887-8994
DOI
10.1016/S0887-8994(98)00157-X
Publisher site
See Article on Publisher Site

Abstract

Two infants with the neonatal type of nonketotic hyperglycinemia that had manifested as early neonatal consciousness disturbance are presented. Transient hyperammonemia had been detected in both initially. High levels of glycine in plasma and cerebrospinal fluid disturb the nervous system, causing variable manifestations of this disease. Both cases were complicated by intracranial hemorrhage, which has never before been reported. After treatment with sodium benzoate and dextromethorphan, some neurologic improvement was observed, although the glycine levels did not lower. Recent clinical trials are reviewed, and because of the unfavorable outcomes, the special need for prenatal diagnosis is highlighted.

Journal

Pediatric NeurologyElsevier

Published: Apr 1, 1999

References

  • Transient neonatal hyperglycinemia
    Schiffmann, R.; Kaye, E.M.; Willis, J.K.; Africk, D.; Ampola, M.
  • The glycine cleavage system
    Kikuchi, G.
  • Nonketotic hyperglycinemia
    Ohya, Y.; Ochi, N.; Mizutani, N.; Hayakawa, C.; Watanabe, K.
  • Prognostic clues and outcome of early treatment of nonketotic hyperglycinemia
    Boneh, A.; Degani, Y.; Harari, M.
  • Long-term use of high-dose benzoate and dextromethorphan for the treatment of nonketotic hyperglycinemia
    Hamosh, A.; Maher, J.F.; Bellus, G.A.; Rasmussen, S.A.; Johnston, M.V.

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