Proton MR spectroscopy of childhood adrenoleukodystrophy.
OBJECTIVE
To determine the potential of proton MR spectroscopy to monitor patients with childhood-onset cerebral adrenoleukodystrophy (COCALD).


METHODS
Single-voxel MR spectroscopy was performed in 16 children with COCALD (24 examinations) who had had no treatment and in 7 children (13 examinations) who had had bone marrow transplantation.


RESULTS
In the untreated children with clinically active COCALD, the metabolite ratios N-acetyl-aspartate (NAA)/creatine (Cr) and NAA/choline (Ch) were decreased while Ch/Cr was increased. This trend agrees well with those reported by other researchers, although different experimental sequences and parameters were used in our study. Comparison of these ratios with those from a control group yielded significant differences in the occipital region. In the children who were clinically stable after bone marrow transplantation, the mean levels of the three ratios were between those of the control subjects and the patients with untreated COCALD: the differences in these ratios approached significance. In patients who had been monitored periodically, MR spectroscopy metabolite ratios correlated well with the dementia rating score, reflecting clinical status.


CONCLUSIONS
There is good correlation between MR spectroscopy metabolite ratios and a patient's clinical status. MR spectroscopy appears to be a useful, noninvasive tool to monitor patients with adrenoleukodystrophy, and it increases the overall sensitivity of MR techniques in clinical applications.
Rajanayagam
V
V
Department of Radiology, University of Minnesota, Minneapolis 55455, USA.
Grad
J
J
Krivit
W
W
Loes
D J
DJ
Lockman
L
L
Shapiro
E
E
Balthazor
M
M
Aeppli
D
D
Stillman
A E
AE
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D001224:Aspartic Acid / Q000031:analogs & derivatives* / Q000378:metabolism; D001928:Brain Diseases, Metabolic / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D002794:Choline / Q000378:metabolism*; D003401:Creatine / Q000378:metabolism*; D005260:Female; D005625:Frontal Lobe / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D009778:Occipital Lobe / Q000473:pathology; D018901:Peroxisomal Disorders / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D012016:Reference Values
D001224:Aspartic Acid; C000179:N-acetylaspartate; D003401:Creatine; D002794:Choline