[Case report of dentatorubral pallidoluysian atrophy in a patient on a ketogenic diet].

ABSTRACT

We report a 16-year-old woman with dentatorubral pallidoluysian atrophy (DRPLA) on a ketogenic diet. She was scheduled for dental treatment under general anesthesia. She was diagnosed as having DRPLA at the age of 10. Medication included clonazepam, chlordiazepoxide, ethosuximide, sodium valproate and piracetam. She had been placed on a ketogenic diet at the age of 15, and seizures decreased. Preoperative laboratory data were normal except for serum cholesterol level (228 mg x dl(-1)) and blood ketones (1.8 mmol x l(-1)) with the use of Medisense Xtra. General anesthesia was induced using thiamylal, vecuronium, sevoflurane and maintained with sevoflurane and nitrous oxide-oxygen. Fluid infusion employed Solita T1 (glucose content of 2.6% total 150 ml). Operation and general anesthesia presented no problems. We continued to infuse Solita T1 (total 350ml) for about 4 hours postoperatively. Ketogenic diet and additional medicine were started after 5 hours postoperatively. We measured perioperative and postoperative BS and blood ketones with the use of Medisense Xtra. She recovered from anesthesia without any significant complication.