Self-inflicted injury in a case of Hallervorden-Spatz disease

Hallervorden-Spatz disease (HSD) is a rare neurodegenerative disorder characterized by abnormally high deposits of iron in the brain. This report describes a child with HSD who presented with self-inflicted ulceration of the lip and tongue, which was initiated during periods of intense oro-facial spasms. Other findings included dental caries and trauma to the primary incisors. Comprehensive dental care was carried out under general anaesthesia. The self-mutilation of the oro-facial mucosa was eliminated by placement of upper and lower soft resin bite guards.