title: Development of new forms of self-injurious behavior following total dental extraction in Lesch-Nyhan disease.
abstract: We report two Lesch-Nyhan Disease (LND) patients who developed new forms of self-injurious behavior following total dental extraction. Patients 1 and 2 were submitted to total teeth extraction at the age of 13 and 8 years, respectively, due to continuous self-biting, not prevented by mouth guards. Severity of dystonia was markedly reduced and quality of life improved. After 12 and 17 months, respectively, patient 1 started rubbing one foot against other and scratching toenails with his hands, and patient 2 stuck his legs and feet against hard objects. These forms of self-injury behavior could be easily prevented with protective materials, according to the mothers.
authors.affiliation: a Department of Internal Medicine , Metabolic-Vascular Unit, La Paz University Hospital, IdiPaz , Madrid , Spain.
authors.forename: Livia
authors.initials: L
authors.lastname: Gisbert de la Cuadra
authors.affiliation: b Department of Biochemistry , La Paz University Hospital, IdiPaz and Center for Biomedical Network Research on Rare Diseases (CIBERER), ISCIII , Madrid , Spain.
authors.forename: Rosa J
authors.initials: RJ
authors.lastname: Torres
authors.affiliation: a Department of Internal Medicine , Metabolic-Vascular Unit, La Paz University Hospital, IdiPaz , Madrid , Spain.
authors.forename: Luis M
authors.initials: LM
authors.lastname: Beltrán
authors.affiliation: a Department of Internal Medicine , Metabolic-Vascular Unit, La Paz University Hospital, IdiPaz , Madrid , Spain.
authors.forename: Arantxa
authors.initials: A
authors.lastname: Sánchez
authors.affiliation: a Department of Internal Medicine , Metabolic-Vascular Unit, La Paz University Hospital, IdiPaz , Madrid , Spain.
authors.forename: Juan G
authors.initials: JG
authors.lastname: Puig
mesh_terms: D000293:Adolescent; D002648:Child; D006801:Humans; D007926:Lesch-Nyhan Syndrome / Q000175:diagnosis* / Q000523:psychology / Q000601:surgery; D008297:Male; D011788:Quality of Life; D016728:Self-Injurious Behavior / Q000175:diagnosis* / Q000517:prevention & control; D014081:Tooth Extraction
keywords: HPRT; Lesch Nyhan; SIB