1‐13C glucose magnetic resonance spectroscopy of pediatric and adult brain disorders

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Abstract
With protocols designed for use in a clinical environment we investigated the feasibility and diagnostic potential of 13C MRS after 1‐13C labeled glucose infusion. 13C MRS brain examinations were performed in 27 subjects (17 children and pediatric patients, six adult patients, and four adult controls), using a standard 1.5 T clinical MR scanner. 1‐13C glucose, 99% enriched (20% w/v) was administered intravenously (690 or 210 mg/kg body weight) or orally (730 mg/kg). Cerebral 13C‐enrichment patterns and time courses were compared. 1‐13C glucose appeared in brain spectra within 2.5–15 min, with ensuing enrichment of its metabolites. No complications were encountered. When data obtained in patients were compared with controls, striking abnormalities in hepatic encephalopathy and in premature brain were observed, consistent with reduced cerebral glucose metabolism. Abnormalities in the 13C enrichment pattern were also observed in pediatric patients with leukodystrophies and mitochondrial disorders. In this preliminary survey, we conclude that 13C MRS in combination with glucose infusion is safe and efficient and provides new insights into the pathophysiology of brain disorders. Copyright © 2001 John Wiley & Sons, Ltd. Abbreviations used: Ala alanine ALD adrenoleukodystrophy Asp aspartate CACH childhood ataxia with diffuse central nervous system hypomyelination Glc glucose Gln glutamine Glu glutamate, HE, hepatic encephalopathy HCO3− bicarbonate i.v. intravenous Lac lactate mI myo‐inositol NAA N‐acetylaspartate TCA tricarboxylic acid.
Funding Information
  • Rudi Schulte Research Institute
  • HMRI
  • J.W. & Ida M. Jameson Foundation of California