A Case of Dentatorubral-pallidoluysian Atrophy for Which Intravenous Sedation Was Effective

Abstract

Dentatorubral-pallidoluysian atrophy (DRPLA) is a form of spinocerebellar degeneration, and patients may require behavioral adjustment for some problems such as myoclonus, psychiatric symptoms associated with intellectual disability, ataxia, and seizure during dental treatment. We report herein a case in which intravenous sedation was effective in the dental treatment of a patient with DRPLA.

The case was a 22-year-old woman. The patient visited our hospital with a complaint of toothache of the right mandibular second molar. At the age of 16, she was diagnosed with juvenile DRPLA, and at the age of 20, she could not talk and needed long-term care such as eating and changing clothes. When infiltration anesthesia was started as usual treatment to perform pulpectomy on the right mandibular second molar, the blood pressure increased to systolic blood pressure of 192mmHg, the heart rate became sinus tachycardia at 150 beats/minute, and myoclonus became remarkable. Therefore, systemic management was performed by applying intravenous sedation using midazolam (6mg+2mg). Since recovery was slow at this time, we changed to propofol (initial bolus administration of 40mg, continuous intravenous infusion of 5mg/kg/hour) from the second time onward, and behavioral control was good and recovery was quick.

Two and a half years after finishing the treatment, she returned to the hospital. A gastrostomy and a tracheostomy tube were indwelled. At the time of the examination, the blood pressure and heart rate suddenly increased and myoclonus became remarkable as in the previous examination. Therefore, we were able to perform safe and appropriate treatment by performing mental relaxation and behavioral adjustment again by intravenous sedation using propofol.