Abstract
Sjögren-Larsson syndrome (SLS) is a rare, autosomal recessive congenital disorder that affects mainly skin and central nervous system. It is characterized by spastic diplegia or tetraplegia, mental retardation and congenital ichthyosis. We report a 2-year-old patient with SLS in whom both mildly restricted diffusion of water in immediate periventricular white matter and mildly increased diffusion of water in the rest of periventricular white matter were observed on diffusion-weighted images. Brain magnetic resonance spectroscopy demonstrated typical high, sharp lipid peak at 1.3 ppm that was stable at long TEs.
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