PEDIATRICS Vol. 71 No. 4 April 1983, pp. 581-584
This Article
Right arrow Full Text (PDF)
Right arrow Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when eLetters are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Matalon, R.
Right arrow Articles by Michals, K.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Matalon, R.
Right arrow Articles by Michals, K.

Nonketotic Hyperglycinemia: Treatment with Diazepam—A Competitor for Glycine Receptors

Reuben Matalon MD, PhD1, Sakkubai Naidu MD1, John R. Hughes MD, PhD1, and Kimberlee Michals RD, PhD1

1 From the Department of Pediatrics and Neurology, Abraham Lincoln School of Medicine, University of Illinois, Chicago; and Loyola University, Maywood, Illinois

Two female infants with nonketotic hyperglycinemia (NKH) were treated with diazepam for the control of seizures. The first infant had seizures, lethargy, and respiratory distress in the first 24 hours of life. The diagnosis of NKH was made at 3 weeks of age and she was then placed on a regimen of strychnine and a lowprotein diet. Strychnine therapy was discontinued after three months of treatment because there was no improvement in the seizure control or in the patient's condition. At 5 months of age the patient was referred to our clinic for further work-up. The second infant had seizures, hypotonia, and respiratory distress shortly after birth. She was treated with phenobarbital and diphenylhydantion, which had no effect on her seizures. The baby was referred to our clinic at 8 months of age and diagnostic studies revealed NKH. All previous medications were stopped and both infants were placed on diazepam, a competitor for glycine receptors in the CNS. Choline and folic acid were added for one-carbon unit transfer and sodium benzoate to bind excessive glycine. Both infants responded to this treatment with cessation of seizures; they became more responsive and alert, and their EEGs showed remarkable improvement despite the persistence of elevated glycine levels in plasma, CSF, and urine. Diazepam as a competitor for the receptors of glycine may prove helpful in controlling the intractable seizures associated with NKH.

Key Words: nonketotic hyperglycinemia • glycine receptors • diazepam

Submitted on July 6, 1981
Accepted on November 4, 1981




This article has been cited by other articles:


Home page
 J Child NeurolHome page
R. E. Frye, E. Donner, A. Golja, and C. M. Rooney
Folinic Acid--Responsive Seizures Presenting as Breakthrough Seizures in a 3-Month-Old Boy
J Child Neurol, August 1, 2003; 18(8): 562 - 569.
[Abstract] [PDF]

Home page
 J. Neurophysiol.Home page
L. L. Thio, A. Shanmugam, K. Isenberg, and K. Yamada
Benzodiazepines Block {alpha}2-Containing Inhibitory Glycine Receptors in Embryonic Mouse Hippocampal Neurons
J Neurophysiol, July 1, 2003; 90(1): 89 - 99.
[Abstract] [Full Text] [PDF]

Home page
 J Child NeurolHome page
M. R. Pranzatelli
Topical Review: Antidyskinetic Drug Therapy for Pediatric Movement Disorders
J Child Neurol, September 1, 1996; 11(5): 355 - 369.
[Abstract] [PDF]