• Korean J Radiol · Oct 2001

    Case Reports

    Localized proton MR spectroscopic detection of nonketotic hyperglycinemia in an infant.

    • C G Choi, H K Lee, and J H Yoon.
    • Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. cgchoi@amc.seoul.kr
    • Korean J Radiol. 2001 Oct 1; 2 (4): 239-42.

    AbstractNonketotic hyperglycinemia (NKH) is a rare metabolic brain disease caused by deficient activity of the glycine cleavage 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 spectroscopic 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.

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