US EPA

2775227 

Journal Article 

Dextromethorphan and high-dose benzoate therapy for nonketotic hyperglycinemia in an infant 

Hamosh, A; McDonald, JW; Valle, D; Francomano, CA; Johnston, MV; Et al 

1992 

Yes 

Journal of Pediatrics
ISSN: 0022-3476
EISSN: 1097-6833 

IPA/93/1015142 

Pediatr 

REF 13 

131-135 

English 

1385627 

WOS:A1992JC51900027 

IPA COPYRIGHT: ASHP The use of high dose sodium benzoate and dextromethorphan polistirex (Delsym), a blocker of the N-methyl-D-aspartate channel, in the treatment of nonketotic hyperglycinemia was studied in a male neonate who received 500 mg/kg/day of sodium benzoate increased to 750 mg/kg/day, and later 7.5 mg/kg/day of dextromethorphan polistirex in 3 divided doses. There was prompt normalization of the neurologic and electroencephalographic findings after therapy with benzoate. The glycine concentrations in both plasma and CSF were substantially reduced. When dextromethorphan was added the electroencephalogram remained normal until the infant was 8 months of age, when diffuse slowing became apparent. Serial brain magnetic resonance imaging showed delayed myelination. At 12 months of age, physical examination and growth were normal except for hypotonia. It was concluded that although this outcome is not ideal, it is better than that typical for nonketotic hyperglycinemia.