Nonketotic hyperglycinemia: clinical and electrophysiologic effects of dextromethorphan, an antagonist of the NMDA receptor

B Schmitt; B Steinmann; R Gitzelmann; L Thun-Hohenstein; H Mascher; G Dumermuth

doi:10.1212/wnl.43.2.421

Nonketotic hyperglycinemia: clinical and electrophysiologic effects of dextromethorphan, an antagonist of the NMDA receptor

Neurology. 1993 Feb;43(2):421-4. doi: 10.1212/wnl.43.2.421.

Authors

B Schmitt¹, B Steinmann, R Gitzelmann, L Thun-Hohenstein, H Mascher, G Dumermuth

Affiliation

¹ Division of Clinical Neurophysiology, University Children's Hospital, Zürich, Switzerland.

PMID: 8437713
DOI: 10.1212/wnl.43.2.421

Abstract

A 10-week-old girl with nonketotic hyperglycinemia was treated with increasing amounts of dextromethorphan, an NMDA receptor antagonist. She improved neurologically; at 35 mg/kg/d, seizures ceased and EEG normalized. Dextromethorphan withdrawal resulted in a dramatic clinical deterioration coinciding with epileptic and high-voltage slow activity in the EEG. After reintroduction of dextromethorphan (35 mg/kg/d), recovery occurred within 24 hours.

Publication types

Case Reports

MeSH terms

Amino Acid Metabolism, Inborn Errors / drug therapy*
Amino Acid Metabolism, Inborn Errors / metabolism
Amino Acid Metabolism, Inborn Errors / physiopathology
Dextromethorphan / pharmacokinetics
Dextromethorphan / therapeutic use*
Electroencephalography / drug effects*
Female
Glycine / blood*
Glycine / metabolism
Humans
Infant
Receptors, N-Methyl-D-Aspartate / antagonists & inhibitors*

Substances

Receptors, N-Methyl-D-Aspartate
Dextromethorphan
Glycine