Localized Proton MR Spectroscopic Detection of Nonketotic Hyperglycinemia in an Infant

  • Choong-Gon Choi (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Ho Kyu Lee (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jong-Hyun Yoon (Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine)
  • 투고 : 2001.05.18
  • 심사 : 2001.10.05
  • 발행 : 2001.12.31

초록

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.

키워드

참고문헌

  1. Probst A, Cortes R, Palacios JM. The distribution of glycine receptors in the human brain: a light microscopic autoradiographic study using [3H]strychinine. Neuroscience 1986;17:11-35
  2. Kemp JA, Leeson PD. The glycine binding site of the NMDA receptor: five years on. Trends Pharmacol Sci 1993;14:20-25
  3. Heindel W, Kugel H, Roth B. Noninvasive detection of increased glycine content by proton MR spectroscopy in the brains of two infants with non-ketotic hyperglycinemia. Am J Neuroradiol 1993;14:629-635
  4. Suzuki Y, Ueda H, Toribe Y. Proton MR spectroscopy of nonketotic hyperglycinemia. No To Hattatsu 2001;33:422-425
  5. Gabis L, Parton P, Roche P, Lenn N, Tudorica A, Huang W. In vivo 1H magnetic resonance spectroscopic measurement of brain glycine levels in nonketotic hyperglycinemia. J Neuroimaging 2001;11:209-211
  6. Michaelis T, Merboldt KD, Haenicke W, Gyngell ML, Bruhn H, Frahm J. On the identification of cerebral metabolites in localized 1H NMR spectra of human brain in vivo. NMR Biomed 1991;4:90-98
  7. Press GA, Barshop BA, Haas RH, Nyhan WL, Glass RF, Hesselink JR. Abnormalities of the brain in nonketotic hyperglycinemia: MR manifestations. Am J Neuroradiol 1989;10:315-321
  8. Frahm J, Hanefeld F. Localized proton magnetic resonance spectroscopy of brain disorders in childhood. In Bachelard HS. Magnetic resonance spectroscopy and imaging in neurochemistry. New York: Plenum Press, 1997:329-402
  9. Schiffmann R, Kaye EM, Willis JK 3rd. Africk D, Ampola M. Transient neonatal hyperglycinemia. Ann Neurol 1989;25:201-203
  10. Jackson AH, Applegarth DA, Toone JR, Kure S, Levy HL. Atypical nonketotic hyperglycinemia with normal cerebrospinal fluid to plasma glycine ratio. J Child Neurol 1999;14:464-467