Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant.

Author: Choong Gon CHOI ¹ ; Ho Kyu LEE ; Jong Hyun YOON
Author Information

1. Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine. cgchoi@amc.seoul.kr
Publication Type:Case Report
Keywords: Brain, MR spectroscopy; Brain, metabolism
MeSH: Case Report; Human; Hyperglycinemia, Nonketotic/*diagnosis; Infant, Newborn; Magnetic Resonance Imaging; *Magnetic Resonance Spectroscopy; Male; Protons
From:Korean Journal of Radiology 2001;2(4):239-242
CountryRepublic of Korea
Language:English
Abstract: Nonketotic hyperglycinemia (NKH) is a rare metabolic brain disease caused by deficient activity of the glycine cleveage system. Localized proton MR spectroscopy (echo-time 166 msec), performed in an infant with the typical clinical and biochemical features of neonatal NKH, showed a markedly increased peak intensity at 3.55 ppm, which was assigned to glycine. Serial proton MR spectrosocpic studies indicated that glycine/choline and glycine/total creatine ratios correlated closely with the patient's clinical course. Proton MR spectroscopy was useful for the non-invasive detection and monitoring of cerebral glycine levels in this infant with NKH.