Childhood adrenoleukodystrophy: assessment with proton MR spectroscopy.

Abstract

Image-guided, single-voxel, localized proton magnetic resonance (MR) spectroscopy was performed to assess white matter in childhood adrenoleukodystrophy (ALD). 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. 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. Proton MR spectroscopy may prove a valuable technique for noninvasive diagnostic and prognostic assessment of ALD.