Abstract
Beta-propeller protein-associated neurodegeneration, formerly called static encephalopathy of childhood with neurodegeneration in adulthood, is a disease caused by mutations in the autophagy gene WDR45. The clinical course over time is very specific : non-progressive epilepsy, intellectual disability, and motor developmental retardation are seen in childhood. From adolescence to adulthood, rapid neurological regression occurs, and dystonia and parkinsonism develop, along with progressive dysphagia. Eventually, the patient becomes bedridden. We performed dental treatment under intravenous sedation in a school-aged child with beta-propeller protein-associated neurodegeneration. Because of the patient’s dysphagia, sedation was carefully administered to a level that prevented any loss of the swallowing reflex. Water injection was kept to a minimum, and the injected water was rapidly suctioned away. Cervical auscultation was used continuously to monitor the patient’s respiratory status and to ascertain the presence of fluid retention in the pharynx. Midazolam and propofol were selected as the primary sedatives because of their anti-epileptic effects. Although a small bolus of intravenous propofol was also administered, a continuous infusion was not used.
