Sjogren Larsson syndrome is an autosomal recessive disorder, characterized by congenital ichtiyosis, mental retardation and spastic diplegia tetraplegia. These patients have impaired fatty alcohol oxidation, lie presented a female patient, 11 months of age, with congenital ichtyosis, spastic diplegia, cerebral cortical atrophy and subdural effusion. [Journal ofTurgut Ozal Medical Center l(3):207-209,1994]
Key Words: Cortical atrophy, ichtyosis, Sjogren Larsson syndrome, spastic diplegia
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