title: Proton MR spectroscopy in infants with cerebral energy deficiency due to hypoxia and metabolic disorders.
abstract: OBJECTIVE
  To investigate the similarities and differences of cellular energy deficiencies due to asphyxia and to impaired oxidative phosphorylation caused by enzyme deficiencies using localized 1H-MR spectroscopy of the brain.
  
  
  METHODS
  In 35 neonates and infants with a postconceptional age of 36.4-153 weeks, 1H-MR spectra were obtained from the basal ganglia. Of the 35 children, 14 served as a control group, 12 suffered from hypoxic-ischemic disease (HIE), and 9 had congenital enzymatic disorders.
  
  
  RESULTS
  Eleven of the 12 children with HIE showed low N-acetyl aspartate (NAA) to trimethylamine (TMA) ratios, indicating neuronal damage. Lactate (Lac) to TMA ratios correlated with the HIE grade. Children with congenital disorders of energy metabolism had NAA/TMA ratios ranging from severely decreased to normal, but 7 of the 9 had high Lac signals, even in brains with normal NAA/TMA.
  
  
  CONCLUSIONS
  High Lac/TMA even with normal NAA/TMA in children with enzyme deficiencies, in contrast to Lac/TMA that correlates with clinical grade and low NAA/TMA in asphyxic children, hints at different mechanisms of cell damage in the two disease groups.
authors.affiliation: Department of Diagnostic Radiology, Children's Hospital, University of Cologne, Germany.
authors.forename: H
authors.initials: H
authors.lastname: Kugel
authors.forename: W
authors.initials: W
authors.lastname: Heindel
authors.forename: B
authors.initials: B
authors.lastname: Roth
authors.forename: S
authors.initials: S
authors.lastname: Ernst
authors.forename: K
authors.initials: K
authors.lastname: Lackner
mesh_terms: D001224:Aspartic Acid / Q000031:analogs & derivatives / Q000172:deficiency; D001479:Basal Ganglia / Q000378:metabolism; D001921:Brain / Q000378:metabolism*; D001928:Brain Diseases, Metabolic / Q000175:diagnosis / Q000209:etiology / Q000378:metabolism*; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D000860:Hypoxia / Q000150:complications / Q000175:diagnosis / Q000378:metabolism*; D007223:Infant; D019344:Lactic Acid / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D008661:Metabolism, Inborn Errors / Q000150:complications / Q000175:diagnosis / Q000378:metabolism*; D008744:Methylamines / Q000378:metabolism; D010085:Oxidative Phosphorylation; D011522:Protons; D012720:Severity of Illness Index
chemical_list: D008744:Methylamines; D011522:Protons; D001224:Aspartic Acid; D019344:Lactic Acid; C000179:N-acetylaspartate; C023336:trimethylamine