A Case of Doose Syndrome Successfully Treated with Ethosuximide in a 3-Year-Old Boy Diagnosed after Carbamazepine Administration Induced Myoclonic Seizures

Abstract

A 3-year-old boy with a history of four febrile convulsions since the age of 1 year was admitted to the hospital after experiencing a cluster of tonic seizures. Treatment was started with valproic acid (VPA), which proved to be ineffective, so carbamazepine (CBZ) was administered instead. This was judged to be effective because the tonic seizures disappeared temporarily. The patient was discharged, but he then had a similar cluster of tonic seizures and was consequently readmitted. Zonisamide (ZNS) was administered, which resolved the tonic seizures. CBZ was discontinued because we thought it would exacerbate the myoclonic seizures he was experiencing. The myoclonic seizures disappeared, and the addition of ethosuximide (ESM) completely resolved his tonic seizures. In this patient, the onset of tonic seizures was characterized by clusters, but because myoclonic seizures were not prominent initially, we were unable to diagnose Doose syndrome early. However, ESM proved remarkably effective, and the patient has remained seizure free.