The patient was a 21-year-old female. She had frequently had status seizures when she had a fever or while taking a bath since she was 6 months old. At 1 year and 8 months old, she developed epilepsy. She was treated with multiple antiepileptic drugs, but her condition was intractable. At the age of 3, the patient suffered from acute encephalopathy, which was complicated by severe psychomotor developmental retardation. Tonic seizures continued to occur on a daily basis even after school age, but they did not worsen even during periods of fever, and the patient was not hospitalized until the age of 8. At the age of 19, the diagnosis was revised and Dravet syndrome was diagnosed. Lamotrigine, which had been taken at the time of diagnosis, was discontinued and stiripentol was administered, but the seizure frequency worsened. Because of the pathology of Dravet syndrome in adults may differ from that in children, care must be taken when selecting antiepileptic drugs.
Keywords: Dravet syndrome; SCN1A gene; lamotrigine; stiripentol.