Proton MR spectroscopy in infants with cerebral energy deficiency due to hypoxia and metabolic disorders.
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.
Kugel
H
H
Department of Diagnostic Radiology, Children's Hospital, University of Cologne, Germany.
Heindel
W
W
Roth
B
B
Ernst
S
S
Lackner
K
K
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
D008744:Methylamines; D011522:Protons; D001224:Aspartic Acid; D019344:Lactic Acid; C000179:N-acetylaspartate; C023336:trimethylamine