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Ketamine and strychnine treatment of an infant with nonketotic hyperglycinaemia

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Abstract

Abstract

Non-ketotic hyperglycinaemia (NKH) is a severe seizure disorder associated with high glycine levels. Glycine is a major inhibitory neurotransmitter in the CNS, but has also modulating effects at one of the glutamate receptors, the N-methyl-d-aspartate-(NMDA) receptor. Based on this knowledge we treated a female newborn suffering from severe NKH with the NMDA receptor blocker ketamine in association with strychnine and magnesium supplementation. This treatment led to cessation of seizures, reappearance of swallowing and sucking and improved the neurological status. Some pharmacokinetic data of strychnine and ketamine in the infant are given.

Conclusion

Ketamine in combination with strychnine may be beneficial in non-ketotic hyperglycinaemia.

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Abbreviations

GABA :

gamma-amino-butyric acid

HPLC :

high performance liquid chromatography

NKH :

non-ketotic hyperglycinaemia

NMDA :

N-methyl-d-aspartate

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Authors and Affiliations

  1. Klinik und Poliklinik für Kinderheilkunde, Universität Köln, Joseph-Stelzmann-Strasse 9, D-50924, Köln, Germany

    H. Tegtmeyer-Metzdorf, B. Roth & M. Günther

  2. Institut für Pharmacologie, Universität Köln, Germany

    M. Theisohn

  3. Institut für Neurophysiologie, Universität Köln, Germany

    U. Heinemann

  4. Abteilung für Anacsthesiologie, Marienkrankenhaus, August-Antz-Strasse 22, D-54293, Trier, Germany

    H. A. Adams

  5. Institut für Gerichtsmedizin, Universität Köln, Germany

    G. Sticht

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  1. H. Tegtmeyer-Metzdorf
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  2. B. Roth
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  3. M. Günther
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  4. M. Theisohn
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  5. U. Heinemann
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  6. H. A. Adams
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  7. G. Sticht
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Tegtmeyer-Metzdorf, H., Roth, B., Günther, M. et al. Ketamine and strychnine treatment of an infant with nonketotic hyperglycinaemia. Eur J Pediatr 154, 649–653 (1995). https://doi.org/10.1007/BF02079070

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  • DOI: https://doi.org/10.1007/BF02079070

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