Botulinum toxin: treatment of self-mutilation in patients with Lesch-Nyhan syndrome.
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.
Parkinson's Disease and Movement Disorders Unit, Hospital de Clínicas José de San Martín, Universidad de Buenos Aires, Buenos Aires, Argentina.
Cristiam
C
Gutierrez
Alejandro
A
Pellene
Federico
F
Micheli
D000328:Adult; D018726:Anti-Dyskinesia Agents / Q000627:therapeutic use*; D001905:Botulinum Toxins / Q000627:therapeutic use*; D005145:Face; D006801:Humans; D007273:Injections, Intramuscular; D007926:Lesch-Nyhan Syndrome / Q000150:complications*; D008046:Lip / Q000293:injuries; D008297:Male; D012652:Self Mutilation / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome; D014945:Wound Healing
D018726:Anti-Dyskinesia Agents; D001905:Botulinum Toxins