Summary of dental treatment in Lesch–Nyhan syndrome
| Study | Age (yr)/sex | Treatment | Treatment effect |
|---|---|---|---|
| Dabrowski et al.5 (2005) | 10/M | BTX-A injection in both masseter muscles, a total of 40 units, every 12 weeks | • ↑ Speech articulation • No impact on eating or swallowing |
| Cauwels and Martens4 (2005) | 5/M | Combination of a mouthguard in the upper jaw and a lip-bumper in the lower jaw | • Positive behavioral modification |
| Gutierrez et al.12 (2008) | 30/M | BTX-A injection in both zygomatic muscles 12.5 IU each, six sites in the lower part of the lip orbicularis muscle of 2.5 IU each, and three injections in the levator labii inferioris of 5 IU each, every 3 months | • ↓ Lip biting and self-mutilating behavior • No side effects |
| Arhakis et al.2 (2010) | 14/M | Maxillary acrylic appliance with a posterior occlusal bite plate | • During the 3-year follow up, self-injury behavior disappeared |
| Gilbert et al.3 (2021) | 13/M | BoNT injection in both masseter muscles, to a total of 50 units, every 4-6 months | • ↑ Speech articulation • ↓ Self-mutilating behavior • ↑ Feeding |
(M: male, BTX: botulinum toxin, BoNT: bonabotulinum toxin)