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.
Sheehy
E C
EC
Department of Orthodontics and Paediatric Dentistry, Guy's Hospital, London, UK. e.sheehy@umds.ac.uk
Longhurst
P
P
Pool
D
D
Dandekar
M
M
D002648:Child; D003730:Dental Care for Persons with Disabilities; D005260:Female; D006801:Humans; D008046:Lip / Q000293:injuries; D008297:Male; D009061:Mouth Mucosa / Q000293:injuries*; D009064:Mouth Protectors; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000150:complications*; D012652:Self Mutilation / Q000209:etiology* / Q000517:prevention & control; D018677:Tooth Injuries / Q000209:etiology* / Q000628:therapy