A Patient with Doose Syndrome Who Received Low Glycemic Index Treatment

Abstract

Epilepsy with myoclonic-atonic seizures (EMAS), also known as Doose syndrome, is characterized by the presence of myoclonic-atonic seizures (MAS) in an otherwise normal child who may have a history of febrile and/or afebrile seizures [1]. Doose syndrome was previously classified as cryptogenic or idiopathic epilepsy with primary generalized minor seizures with Lennox-Gastaut syndrome (LGS) in the 1970s and 1980s [1,2]. However, research eventually distinguished Doose syndrome and LGS as different diseases. The recommended therapies for Doose syndrome include anti-seizure medication (ASM) and diet therapy, such as ketogenic diet (KD) and modified atkins diet (MAD). ASMs such as valproic acid, levetiracetam, clobazam, lamotrigine, ethosuximide, and topiramate are known to be effective. However, it has been reported that pharmacoresistance occurs in about 90% of cases. When diet therapy is applied to patients with Doose syndrome, it is known that seizure reduction of more than 50% is achieved in about 80% of patients [3]. Although there is no consensus on the most suitable treatment because Doose syndrome is mostly drug-resistant, diet therapy is by far the most effective. However, the application of KD and MAD in pediatric patients is somewhat difficult due to dietary discomfort and gastrointestinal troubles [3-5]. Recently, growing evidence has shown that low glycemic index treatment (LGIT) is as good as the classic KD or MAD in terms of efficacy for drug-resistant epilepsy [4,6]. Herein, we report the case of a patient with Doose syndrome who showed improvement in clinical seizures and electroencephalogram (EEG) after LGIT. This study was approved by the Institutional Review Board of the Gangnam Severance Hospital, Yonsei University College of Medicine. Informed consent for this retrospective study was waived by the board (3-2022-0135). An 8-year-old boy with no related medical history and with normal development had his first seizure when he was 5. At that time, he complained of sudden fall-like events and frequent myoclonus. The main seizures were MAS, with absence seizures occurring frequently. In addition, clonic seizures of both extremities with impaired awareness often lasted for 3 to 5 minutes. There was no past history of febrile seizures and no family history of seizures. Five months after his first seizure, the same seizures happened again, so he was treated with valproate acid from the regional tertiary hospital. As the patient complained of memory loss and seizure recurred once every 2 weeks, he visited our hospital outpatient clinic. His magnetic resonance imaging (MRI) was normal and his awake EEG demonstrated frequent multifocal sharp wave discharges. His memory loss was considered