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Sjogren-Larsson syndrome: A rare neurocutaneous disorder
Velusamy Subramanian1, Praveen Hariharan1, J Balaji1
1Department of Paediatric Neurology, Institute of Social Paediatrics, Stanley Medical College and Hospital, Chennai, Tamil Nadu, India.
Abstract:
Sjogren-Larsson syndrome is an autosomal recessive disorder characterized by defective activity of fatty aldehyde dehydrogenase. It presents as a triad of congenital ichthyosis, spastic diplegia, and mental retardation. The pathology behind this syndrome is the failure of degradation of fatty aldehydes. This case is presented for its rarity.
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