Dravet 症候群患者の歯科治療における静脈内鎮静法および全身麻酔管理経験

Dravet syndrome (DS) is characterized by severe myoclonic epilepsy, and its onset is most common during infancy. DS is associated with a mutation of the gene coding SCN1A in most patients ; however, its cause remains unclear. The epileptic seizures that are triggered by various causes are drug-resistant and intractable. Status epilepticus can cause severe cerebral function defects and be life-threatening. We treated a 23-year-old woman with DS who required intravenous sedation and general anesthesia for a dental treatment. She had been taking clonazepam, sodium valproate and topiramate for her epilepsy, but her seizures continued to be triggered semimonthly by seeing objects with a lattice-like pattern and/or in connection with menstruation. She had several dental caries with acute symptoms requiring immediate treatment. Since she had mental retardation and was uncooperative with dental treatment, temporary treatment under intravenous sedation followed by actual treatment under general anesthesia were planned. The timings of both treatments were scheduled so as to avoid her menstrual period, and objects with a lattice-like pattern were removed from the treatment room prior to her entrance. We used propofol for sedation and the induction of general anesthesia and isoflurane for the maintenance of the general anesthesia. A continuous electroencephalogram was monitored using the bispectral index(BIS). No perioperative complications, including seizures, occurred during either treatment. In the present case, we focused on preventing seizures during the perioperative period. Propofol and isoflurane were useful for intravenous sedation and general anesthesia without triggering epileptic seizures.