LOCALIZED PROTON MAGNETIC-RESONANCE SPECTROSCOPY IN PATIENTS WITH ADULT ADRENOLEUKODYSTROPHY - INCREASE OF CHOLINE COMPOUNDS IN NORMAL-APPEARING WHITE-MATTER

Objectives: To describe the changes in the results of magnetic resonan ce imaging and spectroscopy occuring in the normal-appearing white mat ter of patients with adult adrenoleukodystrophy and to present evidenc e of a particular change that may serve as a marker for the follow-up of the disease. Design: Neurologic, magnetic resonance imaging, and lo calized proton spectroscopic examinations were performed in 11 patient s with adult adrenoleukodystrophy and compared with 11 sex- and age-ma tched controls. Patients: Eleven patients with adult adrenoleukodystro phy participated in a trial of dietary therapy with glyceryl trioleate and glyceryl trierucate (Lorenzo's oil) in the Federation de Neurolog ie 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 no rmal in 2 patients and showed areas of mild symmetrical hypersignals o n T-2-weighted images and fluid attenuated inversion recovery sequence s, localized in the posterior white matter in 9 patients. The results of spectroscopy indicated that the peak of the area of choline-contain ing compounds was increased at long echo times in patients with adult adrenoleukodystrophy, which may reflect very long-chain fatty acid acc umulation in this disease. The peak of the area of myo-inositol-contai ning compounds was increased at short echo times in patients with adul t adrenoleukodystrophy, which may indicate a rise in this metabolite c oncentration. The N-acetylaspartate-creatine amplitude ratio was signi ficantly decreased in patients with motor deficit. The significance of this finding remains to be established. Conclusions: The results of l ocalized 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 cha racteristics of the disease. Although changes in the results of spectr oscopy 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 r esonance spectroscopy may prove a valuable technique, in addition to m agnetic resonance imaging, for noninvasive investigation of patients w ith adult adrenoleukodystrophy undergoing future clinical trials.