-Sjögren-Larsson syndrome (SLS) is a rare autosomal recessive neurocutaneous disorder caused by deficiency of the microsomal enzyme fatty aldehyde dehydrogenase. Patients present the classical triad of congenital ichthyosis, mental re t a rdation and spastic di-or tetraplegia. Magnetic resonance imaging (MRI) of the brain usually shows hypomyelination involving the periventricular white matter. Cere b r a l p roton MR spectroscopy ( 1 H-MRS) reveals a characteristic abnormal lipid peak. We re p o rt three cases of SLS from different families with the typical clinical triad. The MRI and 1 H-MRS findings are discussed.
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