Safety and Efficacy of Botulinum Toxin in the Treatment of Self-Biting Behavior in Lesch-Nyhan Disease
| Autor: | Rosa J. Torres, María del Mar Garcia-Romero, Juan García-Puig, Samuel I. Pascual-Pascual |
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| Rok vydání: | 2021 |
| Předmět: |
Male
Botulinum Toxins Adolescent Lesch-Nyhan Syndrome Self-biting Developmental Neuroscience Outcome Assessment Health Care Medicine Humans In patient Adverse effect Child Muscle Skeletal business.industry Retrospective cohort study Botulinum toxin Masticatory force Biting Neurology Neuromuscular Agents Lesch-Nyhan Disease Anesthesia Pediatrics Perinatology and Child Health Masticatory Muscles Arm Self Mutilation Female Neurology (clinical) business medicine.drug |
| Zdroj: | Pediatric neurology. 127 |
| ISSN: | 1873-5150 |
| Popis: | Introduction Lesch-Nyhan disease (LND) is a disease of purine metabolism linked to chromosome X due to the absence or near-absence of enzyme HGPRT. LND patients have a compulsive autoaggressive behaviour that consists on self-mutilation by biting. Objective The objective of this study was to explore the safety and efficacy of botulinum toxin (BoNT) injected into the masticatory muscles and biceps brachii to reduce self-mutilation in patients with LND. Materials and methods This was a retrospective study of 6 LND patients treated with BoNT to prevent automutilatory behaviour. Results The ages at which patients started on treatment with BoNT were 4, 4.5, 6.6, 7.9, 13.9 and 32.3 years old. Patients received a mean number of injections of 20, ranging from 3 to 29, over a period that ranged from 1.5 to 7.1 years. The maximum total dose of Botox ® was 21.3 units/kg mean and the maximum total dose of Dysport ® was 37.5 units/kg mean. A total of 119 injections were performed. Of these 113 (95%) were partially or completely effective. Only 3 out of 119 injections (2.5%) produced adverse effects. Conclusions Botulinum toxin is useful and safe for the treatment of self-biting behaviour in patients with Lesch Nyhan disease. |
| Databáze: | OpenAIRE |
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