Lesch–Nyhan Syndrome: Disorder of Self-mutilating Behavior

Prasad Jathar; Madhura Jathar

doi:10.5005/jp-journals-10005-1350

SEARCH WITHIN CONTENT

Online First

Lesch–Nyhan Syndrome: Disorder of Self-mutilating Behavior

Prasad Jathar, Madhura Jathar

Keywords : HPRT enzyme, Lesch-Nyhan syndrome, Self-mutilating behavior

Citation Information : Jathar P, Jathar M. Lesch–Nyhan Syndrome: Disorder of Self-mutilating Behavior. Int J Clin Pediatr Dent 2016; 9 (2):139-142.

DOI: 10.5005/jp-journals-10005-1350

License: CC BY-NC 4.0

Published Online: 01-02-2019

ARTICLE
REFERENCES

Abstract

Lesch–Nyhan syndrome (LNS), a rare inborn error of metabolism, is characterized by self-injurious behavior, which results in partial or total destruction of oral and perioral tissues and/or fingers. Persistent self-injurious behavior (biting the fingers, hands, lips, and cheeks; banging the head or limbs) is a hallmark of the disease. Prevention of self-mutilation raises significant difficulties. A case of a 10-month-old boy with aggressive behavior and severe lower lip injuries is presented. How to cite this article: Jathar P, Panse AM, Jathar M, Gawali PN. Lesch–Nyhan Syndrome: Disorder of Self-mutilating Behavior. Int J Clin Pediatr Dent 2016;9(2):139-142.

PDF Share

Cusumano FJ, Penna KJ, Panossian G. Prevention of selfmutilation in patients with Lesch–Nyhan syndrome: review of literature. J Dent Child 2001 May-Jun;68(3):175-178.
Lesch M, Nyhan WL. A familial disorder of uric acid metabolism and central nervous system function. Am J Med 1964 Apr;36:561-570.
Jinnah HA, Visser JE, Harris JC, Verdu A, Larovere L, Ceballos- Picot I, Gonzalez-Alegre P, Neychev V, Torres RJ, Dulac O, et al. Delineation of the motor disorder of Lesch-Nyhan disease. Brain 2006 May;129(Pt 5):1201-1217.
Preston R. An error in the code: what can a rare disorder tell us about human behavior? New Yorker 2007 Aug;13: 30-36.
Neychev VK. Jinnah HA. Sudden death in Lesch-Nyhan disease. Dev Med Child Neurol 2006 Nov;48(11):923-926.
Torres RJ, Puig JG. Lesch-Nyhan syndrome. Drugs of the Future 2010;35:421-427.
Nyhan WL. Lesch-Nyhan disease. J Hist Neurosci 2005 Mar;14(1):1-10.
Bakay B, Tucker-Pian C, Seegmiller JE. Detection of Lesch- Nyhan syndrome carriers: analysis of hair roots for HPRT by agarose gel electrophoresis and autoradiography. Clin Genet 1980 Jun;17(6):369-374.
Visser JE, Smith DW, Moy SS, Breese GR, Friedmann T, Rothstein JD, Jinnah HA. Oxidate stress and dopamine deficiency in a genetic mouse model of Lesch-Nyhan disease. Brain Res Dev Brain Res 2002 Feb28;133(2):127-139.
Lloyd KG, Hornykiewicz O, Davidson L, Shannak K, Farley I, Goldstein M, Shibuya M, Kelley WN, Fox IH. Biochemical evidence of dysfunction of brain neurotransmitters in the Lesch-Nyhan syndrome. N Engl J Med 1981 Nov5;305(19): 1106-1111.
Robey KL, Reck JF, Glacomini KD, Barabas G, Eddey GE. Modes and patterns of self-mutilation in persons with Lesch-Nyhan disease. Dev Med Child Neurol 2003 Mar;45(3): 167-171.
Dicks JL. Lesch–Nyhan syndrome: a treatment-planning dilemma. Paediatr Dent 1982 Jun;4(2):127-130.
Macpherson DW, Wolford LM, Kortebein MJ. Orthognathic surgery for the treatment of chronic self-mutilization of the lips. Int J Oral Maxillofac Surg 1992 Jun;21(3):133-136.
Chen L-R, Liu J-F. Successful treatment of self-inflicted oral mutilation using an acrylic splint retained by a head gear. Pediatr Dent 1996 Sep-Oct;18(5):408-410.
Dabrowski E, Smathers SA, Ralstrom CS. Botulinum toxin as a novel treatment for self-mutilation in Lesch–Nyhan syndrome. Dev Med Child Neurol 2005 Sep;47(9):636-639.
Rasul CH, Mostafa KG, Nasrin E. Role of antiepileptics in Lesch- Nyhan syndrome. Glob Adv Res J Med Med Sci 2012;2:1-4.

PDF Share