Localized proton magnetic resonance spectroscopy in patients with adult adrenoleukodystrophy. Increase of choline compounds in normal appearing white matter
A. Tourbah, J. L. Stievenart, M. T. Iba-Zizen, C. Lubetzki, N. Baumann, B. Eymard, H. W. Moser, O. Lyon-Caen and E. A. Cabanis
Service de Neuroradiologie, Centre Hospitalier National d'Ophtalmologie des XV-XX, Paris, France.
OBJECTIVES: 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. DESIGN: 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.
PATIENTS: Eleven patients with adult adrenoleukodystrophy participated in a
trial of dietary therapy with glyceryl trioleate and glyceryl trierucate
(Lorenzo's oil) in the Federation de Neurologie and the Institut National
de la Sante et de la Recherche Medicale, Unite 134, at the Hopital de la
Salpetriere 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.
