Nonketotic hyperglycinemia: clinical and electrophysiologic effects of dextromethorphan, an antagonist of the NMDA receptor.
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.
Schmitt
B
B
Division of Clinical Neurophysiology, University Children's Hospital, Zürich, Switzerland.
Steinmann
B
B
Gitzelmann
R
R
Thun-Hohenstein
L
L
Mascher
H
H
Dumermuth
G
G
D000592:Amino Acid Metabolism, Inborn Errors / Q000188:drug therapy* / Q000378:metabolism / Q000503:physiopathology; D003915:Dextromethorphan / Q000493:pharmacokinetics / Q000627:therapeutic use*; D004569:Electroencephalography / Q000187:drug effects*; D005260:Female; D005998:Glycine / Q000097:blood* / Q000378:metabolism; D006801:Humans; D007223:Infant; D016194:Receptors, N-Methyl-D-Aspartate / Q000037:antagonists & inhibitors*
D016194:Receptors, N-Methyl-D-Aspartate; D003915:Dextromethorphan; D005998:Glycine