International Journal of Paediatric Dentistry 1999-Dec
Self-inflicted injury in a case of Hallervorden-Spatz disease.
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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.