From the Guidelines
Myoclonus epilepsy in childhood should be managed with antiepileptic medications such as valproic acid, levetiracetam, or clonazepam, with the goal of achieving optimal seizure control and improving quality of life, as supported by recent guidelines 1. The treatment of myoclonus epilepsy in childhood typically involves the use of antiepileptic medications, with the specific choice depending on the exact syndrome, patient age, and other factors.
- Valproic acid (Depakote) is often used at a dose of 10-60 mg/kg/day divided into 2-3 doses,
- levetiracetam (Keppra) at 20-60 mg/kg/day divided twice daily,
- or clonazepam (Klonopin) at 0.01-0.2 mg/kg/day divided into 2-3 doses. Management should also include regular follow-up with a pediatric neurologist every 3-6 months, monitoring of drug levels when applicable, and vigilance for side effects, as emphasized in the recent guidelines for the appropriate use of PET and SPECT for patients with epilepsy 1. Additionally, parents should keep a seizure diary, avoid seizure triggers like sleep deprivation and flickering lights, and learn appropriate seizure first aid. Childhood myoclonic epilepsies encompass several syndromes, including juvenile myoclonic epilepsy and progressive myoclonic epilepsies, with varying prognoses, and early diagnosis and consistent medication adherence are crucial for optimal seizure control and neurodevelopmental outcomes, highlighting the importance of a tailored approach to management 1.
From the Research
Myoclonus Epilepsy in Childhood
- Myoclonus epilepsy in childhood can manifest in various forms, including idiopathic and symptomatic epilepsies 2, 3.
- The treatment of myoclonic seizures in children is mainly based on prospective and retrospective studies, with limited evidence from randomized clinical trials 2, 3.
Treatment Options
- Valproate is commonly used as the first choice, alone or in combination with benzodiazepines or levetiracetam, for the treatment of myoclonic seizures in children 2, 3, 4.
- Other treatment options, such as topiramate and zonisamide, may be used, but there is insufficient evidence to support their use as monotherapy 2, 3.
- Lamotrigine can be effective in treating juvenile myoclonic epilepsy and eyelid myoclonia with absences, but its use requires careful consideration due to potential adverse effects 4.
- Avoidance of medications that may aggravate myoclonic seizures, such as carbamazepine and phenytoin, is crucial 2, 3.
Specific Syndromes
- Valproate is the treatment of first choice for several specific syndromes, including benign myoclonic epilepsy in infants, myoclonic astatic epilepsy, and juvenile myoclonic epilepsy 4.
- The addition of ethosuximide to valproate can be helpful in treating myoclonic absences and eyelid myoclonia with absences 4.
- Zonisamide may be effective in reducing myoclonia in progressive myoclonus epilepsies, but relapse may occur after initial improvement 4.
Emerging Therapies
- Recent advances in the management of Dravet syndrome, a severe form of myoclonic epilepsy, include the use of pharmaceutical-grade cannabidiol oil, stiripentol, and fenfluramine 5.
- Several novel agents are in development for the treatment of Dravet syndrome, including TAK935, lorcaserin, and selective sodium channel modulators 5.