2023 Volume 51 Issue 2 Pages 27-29
Alexander disease is an extremely rare and intractable neurodegenerative disease caused by mutations in the GFAP gene. Symptoms include convulsions, macrocephaly, psychomotor retardation, limb muscle weakness, spastic paralysis, limb and trunk ataxia, dysarthria, dysphonia, dysphagia, and other medullary and spinal cord symptoms, as well as cerebellar symptoms. We report our experience providing general anesthesia management during dental treatment in a child with Alexander disease. The patient was a 5-year and 1-month-old girl with a height of 89.6 cm and a weight of 12 kg. Dental treatment under general anesthesia was scheduled for the treatment of multiple dental caries. Her symptoms included epilepsy, autism, and dysphagia. Her epilepsy symptoms were stable and were being managed with the ongoing administration of regular medications. Regarding her autism, we were able to avoid non-cooperation at the time of induction by providing a prior explanation. Anesthesia was slowly induced with oxygen and sevoflurane. After securing a venous route, rocuronium bromide was administered ; nasotracheal intubation was then performed. The anesthesia was maintained using oxygen, nitrous oxide, and desflurane. Approximately 30 minutes after the start of anesthesia, spontaneous breathing occurred, and the anesthesia was maintained with assisted ventilation until the end of the operation. In consideration of postoperative respiratory depression, no opioids were administered intraoperatively. The operation time was 1 hour and 50 minutes, and the anesthesia time was 2 hours and 50 minutes. No complications were observed during or after the general anesthesia, and the patient was discharged from the hospital on the second postoperative day. This case suggests that the selection of anesthetic agents capable of preventing postoperative respiratory depression and neuromuscular blockade monitoring are both important for providing safe anesthesia in patients with Alexander disease.
