Proton MR spectroscopy of Sjogren-Larsson's syndrome

We performed single-voxel proton MR spectroscopy (H-1-MRS) in two children with Sjogren-Larsson's syndrome (SLS). Both patients showed two abnormal sp ectral peaks at 1.3 ppm and 0.9 ppm that were obtained with short echo time s. These two abnormal spectral peaks were seen in high-intensity areas on T 2-weighted images and also in basal ganglia of normal intensities. H-1-MRS may be useful for establishing the diagnosis and investigating the natural history of SLS, and for evaluating the efficacy of therapeutic approaches t o SLS.