Self-inflicted orodental injury in a child with Leigh disease

Leigh disease is an inherited progressive mitochondrial neurodegenerative disease that affects the neurological, respiratory and cardiovascular systems and is associated with retardation of the intellectual and physical development. This report describes the case of a 4-year-old boy with Leigh disease who presented with self-inflicted traumatic injury to the teeth, alveolar bone, lips and tongue during repeated episodes of intense orofacial spasms. Conservative management of the injury included repositioning the fractured alveolar bone, splinting the traumatized teeth and planning for a mouthguard. However, after a second incident of severe self-induced injury to the teeth and alveolar bone, extraction of the anterior teeth became inevitable to protect the child from further self-mutilation and to allow healing of the injured tissues.