Preventions of oral self-mutilation in patients with Lesch-Nyhan syndrome

Abstract

ABSTRACTLesch-Nyhan syndrome (LNS) is a rare genetic disorder linked to the X chromosome that implies an inborn error of metabolism, due to deficiency in the purine metabolic pathway, affected individuals present neurological symptoms, such as cognitive and motor impairment, besides behavioral changes such as self and hetero-aggression. This characteristic aggressive behavior leads to insults and self-mutilation, thus requiring methods of self-protection, some invasive and permanent, such as tooth extraction. Noninvasive methods, such as the use of oral protectors, pharmacological therapies and botulinum toxin A applications, are also employed to decrease discomfort and the implications of such behavior. We describe briefly the methods of prevention against oral self-mutilation in patients with Lesch-Nyhan syndrome found in the literature.