A method for the quantitation of cerebral metabolites on a clinical MR scan
ner by natural abundance C-13 MRS in vivo is described. Proton-decoupled sp
ectra were acquired with a power deposition within FDA guidelines using a n
ovel coil design, myo-Inositol, quantified by a separate proton MRS, and re
adily detectable in C-13 MRS, was used as an internal reference. Normal con
centrations, measured in four control subjects, age 7 months to 12 years, w
ere glutamate 9.9 +/- 0.7, glutamine 5.6 +/- 1.0, and NAA 8.8 +/- 2.8 mmol/
kg. In a patient diagnosed with Canavan disease, examined four times, gluta
mate was reduced to 46% of normal, 4.6 +/- 0.5 mmol/kg, NAA was increased b
y 50% to 13.2 +/- 1.6 mmol/kg in C-13 MRS, consistent with the 41% increase
to 12.3 +/- 1.1 from control 8.7 +/- 1.1 mmol/kg assayed by H-1 MRS. Limit
ed concentration of glutamate may impact on glutamatergic neurons and excit
atory neurotransmission in Canavan disease. Quantitation of cerebral glutam
ate in human brain may have clinical value in human neuropathologies in whi
ch glutamate is believed to play a central role. (C) 1999 Academic Press.