Management of Involuntary Self-Mutilation in a Child with Lesch-Nyhan Syndrome

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Jinda Lertsirivorakul
Khunton Wichajarn
Somsak Tiamkao
Panawat Chawyotha

Abstract

Lesch–Nyhan syndrome (LNS) is a rare X-linked recessive disorder of purine metabolism caused by deficiency of the enzyme hypoxanthine guanine phosphoribosyltransferase (HPRT).  It is characterized by hyperuricemia, neurologic dysfunction, and self-mutilation.  Medical and dental management in a 10-year-old boy diagnosed with LNS are described.  Involuntary self-mutilation could be controlled using a soft mouth guard and repeated injections of botulinum toxin A (BTX-A) into the bilateral masseter and temporalis muscles.  The findings suggest that patients with LNS require treatment from a multidisciplinary team.  In addition, a soft mouth guard, repeated BTX-A injections, and pharmacological therapy could be a promising management of involuntary self-mutilation in LNS patients.

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1.
Lertsirivorakul J, Wichajarn K, Tiamkao S, Chawyotha P. Management of Involuntary Self-Mutilation in a Child with Lesch-Nyhan Syndrome. Khon Kaen Dent J [Internet]. 2020 Dec. 16 [cited 2024 Mar. 29];23(3):91-8. Available from: https://he01.tci-thaijo.org/index.php/KDJ/article/view/240818
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Clinical Science
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