Childhood adrenoleukodystrophy: assessment with proton MR spectroscopy.
OBJECTIVE
Image-guided, single-voxel, localized proton magnetic resonance (MR) spectroscopy was performed to assess white matter in childhood adrenoleukodystrophy (ALD).


METHODS
Eleven X-linked ALD subjects, seven with neurologic symptoms or white matter lesions at MR imaging and four asymptomatic patients, were compared with nine aged-matched, healthy volunteers.


RESULTS
Compared with those from normal white matter, MR spectra from white matter lesions (n = 6) showed 65% reduction in the ratio of N-acetyl aspartate (NAA) to total creatine (tCr) (P < .01); 55% increase in the ratio of choline-containing compounds (Cho) to tCr (P < .02); substantial levels of tCr; 94% increase in the ratio of glutamate, glutamine, or inositol to tCr (P < .02); and lactate accumulation in four patients. Patients without brain lesions (n = 4) exhibited a 51% increase in Cho-to-tCr ratio (P < .01) and 11% nonsignificant increase in NAA-to-tCr ratio.


CONCLUSIONS
Proton MR spectroscopy may prove a valuable technique for noninvasive diagnostic and prognostic assessment of ALD.
Tzika
A A
AA
Department of Radiology, Children's Hospital Medical Center, Cincinnati, Ohio.
Ball
W S
WS
Vigneron
D B
DB
Dunn
R S
RS
Nelson
S J
SJ
Kirks
D R
DR
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism; D001224:Aspartic Acid / Q000031:analogs & derivatives / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D002794:Choline / Q000032:analysis; D003401:Creatine / Q000032:analysis; D008040:Genetic Linkage; D005947:Glucose / Q000032:analysis; D005971:Glutamates / Q000032:analysis; D005973:Glutamine / Q000032:analysis; D005998:Glycine / Q000032:analysis; D006801:Humans; D006859:Hydrogen; D007294:Inositol / Q000032:analysis; D008055:Lipids / Q000032:analysis; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D011522:Protons; D014960:X Chromosome; D005680:gamma-Aminobutyric Acid / Q000032:analysis
D005971:Glutamates; D008055:Lipids; D011522:Protons; D005973:Glutamine; D001224:Aspartic Acid; D007294:Inositol; D005680:gamma-Aminobutyric Acid; D006859:Hydrogen; C000179:N-acetylaspartate; D005947:Glucose; D003401:Creatine; D002794:Choline; D005998:Glycine