Ketamine and strychnine treatment of an infant with nonketotic hyperglycinaemia.
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.


CONCLUSIONS
Ketamine in combination with strychnine may be beneficial in non-ketotic hyperglycinaemia.
Tegtmeyer-Metzdorf
H
H
Klinik und Poliklinik für Kinderheilkunde, Universität Köln, Germany.
Roth
B
B
Günther
M
M
Theisohn
M
M
Heinemann
U
U
Adams
H A
HA
Sticht
G
G
D000592:Amino Acid Metabolism, Inborn Errors / Q000097:blood / Q000188:drug therapy* / Q000235:genetics; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D004359:Drug Therapy, Combination; D018691:Excitatory Amino Acid Antagonists / Q000493:pharmacokinetics / Q000627:therapeutic use*; D005260:Female; D005808:Genes, Recessive / Q000235:genetics; D005998:Glycine / Q000097:blood*; D018684:Glycine Agents / Q000493:pharmacokinetics / Q000627:therapeutic use*; D006801:Humans; D007231:Infant, Newborn; D007649:Ketamine / Q000493:pharmacokinetics / Q000627:therapeutic use*; D009460:Neurologic Examination / Q000187:drug effects; D016194:Receptors, N-Methyl-D-Aspartate / Q000037:antagonists & inhibitors*; D013036:Spasms, Infantile / Q000097:blood / Q000188:drug therapy* / Q000235:genetics; D013331:Strychnine / Q000493:pharmacokinetics / Q000627:therapeutic use*
D018691:Excitatory Amino Acid Antagonists; D018684:Glycine Agents; D016194:Receptors, N-Methyl-D-Aspartate; D007649:Ketamine; D013331:Strychnine; D005998:Glycine