Treatment of self-injurious behaviors in Lesch-Nyhan syndrome with S-adenosylmethionine.

Abstract

An 11-year-old boy with Lesch-Nyhan syndrome (LNS) had persistently injured himself by biting his lips and buccal mucosa since infancy. Risperidone was only partially effective in suppressing this behavior. Oral administration of S-adenosylmethionine (SAMe), involving increasing the dose from 400 mg to 1 g, resulted in the amelioration of self-injurious behavior and anxiety as well as marked improvement in his self-esteem, performance at school, and friendships. No adverse effects were noted. SAMe may have a favorable effect on symptoms of LNS by activating monoaminergic pathways and/or increasing the adenosine pool in the salvage pathway of guanosine monophosphate synthesis. Defects in these pathways have been essentially implicated in the neurological pathophysiology of LNS.

• Authors: Masami Togawa, Yoshiaki Saito, Yoshihiro Maegaki, Kousaku Ohno
• Journal: No to hattatsu = Brain and development (No To Hattatsu) Vol. 49 Issue 1 Pg. 25-7 (Jan 2017) ISSN: 0029-0831 [Print] Japan
• PMID: 30011150 (Publication Type: Case Reports, Journal Article)

Chemical References

• S-Adenosylmethionine
• Hypoxanthine Phosphoribosyltransferase

Topics

• Child
• Humans
• Hypoxanthine Phosphoribosyltransferase (genetics)
• Lesch-Nyhan Syndrome (complications, therapy)
• Male
• Quality of Life
• S-Adenosylmethionine (therapeutic use)
• Self-Injurious Behavior (drug therapy, etiology)