SJOGREN LARSSON SYNDROME- a case report

Abstract : SJOGREN LARSSON SYNDROME(SLS) is a rare autosomal recessive disorder characterized by a triad of mental retardation, congenital ichthyosis and diplegia or tetraplegia. This disorder needs to be differentiated from other neurocutaneous disorders like Neutral lipid storage disease, Refsums disease and HID syndrome (Hysterix like Ichthyosis and Deafness). We report here a classical case of SLS with features of congenital ichthyosis, moderate mental retardation and spastic paraparesis.