Localized proton magnetic resonance spectroscopy in patients with adult adrenoleukodystrophy. Increase of choline compounds in normal appearing white matter.
OBJECTIVE
To describe the changes in the results of magnetic resonance imaging and spectroscopy occurring in the normal-appearing white matter of patients with adult adrenoleukodystrophy and to present evidence of a particular change that may serve as a marker for the follow-up of the disease.


METHODS
Neurologic, magnetic resonance imaging, and localized proton spectroscopic examinations were performed in 11 patients with adult adrenoleukodystrophy and compared with 11 sex- and age-matched controls.


METHODS
Eleven patients with adult adrenoleukodystrophy participated in a trial of dietary therapy with glyceryl trioleate and glyceryl trierucate (Lorenzo's oil) in the Fédération de Neurologie and the Institut National de la Santé et de la Recherche Médicale, Unité 134, at the Hôpital de la Salpêtrière in Paris, France.


RESULTS
The results of magnetic resonance imaging of the white matter were normal in 2 patients and showed areas of mild symmetrical hypersignals on T2-weighted images and fluid attenuated inversion recovery sequences, localized in the posterior white matter in 9 patients. The results of spectroscopy indicated that the peak of the area of choline-containing compounds was increased at long echo times in patients with adult adrenoleukodystrophy, which may reflect very long-chain fatty acid accumulation in this disease. The peak of the area of myo-inositol-containing compounds was increased at short echo times in patients with adult adrenoleukodystrophy, which may indicate a rise in this metabolite concentration. The N-acetylaspartate-creatine amplitude ratio was significantly decreased in patients with motor deficit. The significance of this finding remains to be established.


CONCLUSIONS
The results of localized proton magnetic resonance spectroscopy show abnormalities in the cerebral white matter of patients with adult adrenoleukodystrophy, which may contribute to the understanding of the pathophysiologic characteristics of the disease. Although changes in the results of spectroscopy found in this disease are not specific, the increase of choline-containing compounds may reflect the accumulation of very long-chain fatty acids in the central nervous system. Localized proton magnetic resonance spectroscopy may prove a valuable technique, in addition to magnetic resonance imaging, for noninvasive investigation of patients with adult adrenoleukodystrophy undergoing future clinical trials.
Tourbah
A
A
Service de Neuroradiologie, Centre Hospitalier National d'Ophtalmologie des XV-XX, Paris, France.
Stievenart
J L
JL
Iba-Zizen
M T
MT
Lubetzki
C
C
Baumann
N
N
Eymard
B
B
Moser
H W
HW
Lyon-Caen
O
O
Cabanis
E A
EA
D000326:Adrenoleukodystrophy / Q000175:diagnosis* / Q000178:diet therapy / Q000378:metabolism*; D000328:Adult; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D002794:Choline / Q000378:metabolism*; D004042:Dietary Fats, Unsaturated / Q000627:therapeutic use; D004338:Drug Combinations; D004883:Erucic Acids / Q000627:therapeutic use; D005260:Female; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D014304:Triolein / Q000627:therapeutic use
D004042:Dietary Fats, Unsaturated; D004338:Drug Combinations; D004883:Erucic Acids; C079420:Lorenzo's oil; D014304:Triolein; D002794:Choline