Citation

Gutiérrez C, Pellene A, Micheli F. Clin. Neuropharmacol. 2008; 31(3): 180-183.

Affiliation

Parkinson's Disease and Movement Disorders Unit, Hospital de Clínicas José de San Martín, Universidad de Buenos Aires, Buenos Aires, Argentina.

Copyright

(Copyright © 2008, Lippincott Williams and Wilkins)

DOI

10.1097/WNF.0b013e31814a62cc

PMID

18520986

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.

Language: en