We report MRI and MRS of the brain in a patient with Sjögren-Larsson syndrome (SLS) in whom fatty alcohol oxidoreductase (FAO) deficiency has been verified. MRI showed periventricular lesions, high intensity on T2-weighted and low intensity on T1-weighted images at trigones of the lateral ventricles. 1H-MRS of these lesions revealed high lipid and low N-acetyl aspartate peaks. We presume such lipids in periventricular lesions with high T2 signal may be pathognomonic of SLS.

This paper analyzes the information content of medical images, with 3-D MRI images as an example, in terms of information entropy. The results of the analysis justify the use of Pixel Difference Coding for preserving all information contained in the original pictures, lossless coding in other words. The experimental results also indicate that the compression ratio CR=2:1 can be achieved under the lossless constraints. A pratical implementation of Pixel Difference Coding which allows interactive retrieval of local ROI (Region of Interest), while maintaining the near low bound information entropy, is discussed.

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We report MRI and MRS of the brain in a patient with Sjögren-Larsson syndrome (SLS) in whom fatty alcohol oxidoreductase (FAO) deficiency has been verified. MRI showed periventricular lesions, high intensity on T2-weighted and low intensity on T1-weighted images at trigones of the lateral ventricles. 1H-MRS of these lesions revealed high lipid and low N-acetyl aspartate peaks. We presume such lipids in periventricular lesions with high T2 signal may be pathognomonic of SLS.