Abstract
Lesch-Nyhan syndrome (LNS), is a rare inborn error of metabolism. It is characterized by neurological and behavioral abnormalities and the overproduction of uric acid in the body. It occurs almost exclusively in males. Signs and symptoms may include inflammatory arthritis (gout), kidney stones, bladder stones, and moderate cognitive disability. It is characterized by self-injurious behaviour, which results in partial or total destruction of oral and perioral tissues and/or fingers and hands. Prevention of self-mutilation raises significant difficulties. Treatment modalities include drug therapy, oral appliances, and extraction of teeth This case report is of a 13-year-old boy with aggressive behaviour and severe lower lip and tongue injuries. Keywords - HPRT Gene, Lesch Nyhan Syndrome, Self Mutilative Behaviour , X Linked Genetic Disorder
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