Botulinum toxin: treatment of self-mutilation in patients with Lesch-Nyhan syndrome.

Abstract

Lesch-Nyhan syndrome (LNS) is a rare X-linked recessive disorder involving purine metabolism caused by the congenital absence of hypoxanthine guanine phosphoribosyl transferase. A characteristic feature of LNS is the appearance of intractable self-injurious behavior, usually in the form of severe lip and finger biting. The mechanism behind this severe self-mutilating behavior is unknown, and is one of the main challenges in the management of this condition. We here report the case of a 30-year-old man with a confirmed diagnosis of LNS who was successfully treated for self-mutilation of his lips with repeated botulinum toxin A (BTX-A) injections in the facial perioral muscles. Our findings suggest that treatment with BTX-A helped reduce self-abusive behavior in this patient. Our case illustrates that BTX-A injections can be a useful therapeutic approach in patients with self-abusive behavior.

• Authors: Cristiam Gutierrez, Alejandro Pellene, Federico Micheli
• Journal: Clinical neuropharmacology (Clin Neuropharmacol) 2008 May-Jun Vol. 31 Issue 3 Pg. 180-3 ISSN: 1537-162X [Electronic] United States
• PMID: 18520986 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anti-Dyskinesia Agents
• Botulinum Toxins

Topics

• Adult
• Anti-Dyskinesia Agents (therapeutic use)
• Botulinum Toxins (therapeutic use)
• Face
• Humans
• Injections, Intramuscular
• Lesch-Nyhan Syndrome (complications)
• Lip (injuries)
• Male
• Self Mutilation (drug therapy, etiology)
• Treatment Outcome
• Wound Healing