Standard of Care and Medications for EMA
At a Glance
Epilepsy with myoclonic absences often needs a broad-spectrum medicine, such as valproate, because children may have staring, jerking, and tonic-clonic seizures. If two suitable medicines fail, supervised dietary therapy, VNS, and referral to a pediatric epilepsy center may be considered.
Treating Epilepsy with Myoclonic Absences (EMA) is a unique challenge because the condition involves multiple types of “misfiring” in the brain. Unlike more common childhood epilepsies that might only cause staring spells, EMA often requires a “broad-spectrum” approach—medications that can treat both the absence (staring) and the myoclonus (jerking) at the same time [1][2].
Critical Warning: Never stop or change your child’s antiseizure medication abruptly or without consulting the prescribing doctor. Sudden withdrawal can trigger severe, prolonged, and life-threatening seizures.
First-Line Treatment Options
Because children with EMA often have more than one type of seizure—such as myoclonic absences and generalized tonic-clonic (“grand mal”) seizures—doctors typically choose medications that can cover many seizure types at once.
- Valproate (Valproic Acid): This is frequently the first medication tried for EMA. In small observational studies, many children who achieved seizure freedom were taking valproate [2]. It works across a wide range of generalized seizure types [1].
- Important Safety Note: Valproate carries major risks of fetal malformation and neurodevelopmental issues if taken during pregnancy. Strict reproductive counseling applies to any person who could become pregnant [3]. For the child taking it, major risks include hepatotoxicity (liver damage), pancreatitis, thrombocytopenia (low platelets), high ammonia, and metabolic/weight changes. Close blood monitoring is required.
- Lamotrigine: This is often used, sometimes as an “add-on” alongside valproate, as the two are frequently used together [4][5].
- Important Safety Note: Valproate significantly increases the levels of lamotrigine in the blood. Therefore, lamotrigine must be started at an extremely low dose and increased very slowly to avoid a severe, life-threatening skin rash. If your child develops a fever, mouth sores, blistering, or any spreading rash, seek urgent medical advice. Note also that lamotrigine can occasionally worsen myoclonic seizures in some patients.
- Ethosuximide: While this is the “gold standard” for typical Childhood Absence Epilepsy (CAE), it is often not adequate for EMA when used alone [6]. This is because ethosuximide treats staring spells well but does not protect against generalized tonic-clonic or myoclonic seizures [6][7].
A Vital Warning: Medicines That May Worsen Seizures
In EMA, choosing the wrong medicine can be as significant as choosing the right one. Certain medications used to treat focal epilepsy can actually aggravate generalized myoclonic and absence seizures [8][9]. If you observe worsening seizures after a new medication is started, contact your specialist for urgent review, but do not stop the medication on your own.
Medications that may provoke or worsen myoclonic/generalized seizures include:
- Carbamazepine
- Oxcarbazepine
- Phenytoin
- Vigabatrin
- Tiagabine
- Gabapentin and Pregabalin [9]
Understanding Drug Resistance
Unfortunately, EMA is known for being difficult to treat. About half of children with EMA may have drug-resistant epilepsy, which is defined as failing to achieve seizure freedom after trying two appropriately chosen medications at the right doses [2][10].
If your child’s seizures continue despite two medication trials, your care team may discuss other specialized options:
- Dietary Therapy: The ketogenic diet or the modified Atkins diet are medically supervised, high-fat diets that can significantly reduce seizures in some children with drug-resistant epilepsy [11][12].
- Vagus Nerve Stimulation (VNS): A small device implanted under the skin that sends regular, mild pulses of electrical energy to the brain via the vagus nerve [13].
- Specialist Referral: At this stage, a referral to a comprehensive pediatric epilepsy center (often called a Level 4 center in the US) is critical [14]. These centers have specialized teams—including dietitians, surgeons, and geneticists—who focus entirely on children with complex epilepsy.
While the path to the right treatment can be a process of trial and error, identifying the broad-spectrum medications that work best while avoiding those that worsen myoclonus is the foundation of a good management plan.
Common questions in this guide
Why is a broad-spectrum medicine often used for EMA?
Can ethosuximide treat all of the seizures in EMA?
How does valproate affect lamotrigine treatment?
Which medicines can make EMA seizures worse?
When is EMA considered drug-resistant?
Could a ketogenic diet or vagus nerve stimulation help if medicines do not work?
Questions For You
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Questions to Ask Your Doctor
Curated prompts to bring to your next appointment.
- 1.Since my child has both 'staring' and 'jerking' seizures, why is a broad-spectrum medicine like valproate preferred over one like ethosuximide?
- 2.What is the specific plan to monitor for side effects, such as changes in liver function, weight, or the pancreas, if we start valproate?
- 3.If we use lamotrigine, how slowly will we increase the dose to minimize the risk of a serious rash, especially if taken with valproate?
- 4.Are there any medicines my child is currently taking that might be aggravating the myoclonus?
- 5.If the first two medications do not stop the seizures, at what point should we be referred to a comprehensive pediatric epilepsy center?
- 6.Is my child a candidate for a medical diet, like the ketogenic diet, if medications are not fully effective?
References
References (14)
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Hu Q, Luo Y, Hong S, et al.
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Alternatives to valproate in girls and women of childbearing potential with Idiopathic Generalized Epilepsies: state of the art and guidance for the clinician proposed by the Epilepsy and Gender Commission of the Italian League Against Epilepsy (LICE).
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PMID: 33418162 - 4
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Expert review of neurotherapeutics 2020; (20(2)):167-173 doi:10.1080/14737175.2020.1707668.
PMID: 31855066 - 5
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Expert opinion on drug metabolism & toxicology 2021; (17(9)):1075-1090 doi:10.1080/17425255.2021.1959912.
PMID: 34310255 - 6
Ethosuximide, sodium valproate or lamotrigine for absence seizures in children and adolescents.
Brigo F, Igwe SC
The Cochrane database of systematic reviews 2017; (2()):CD003032 doi:10.1002/14651858.CD003032.pub3.
PMID: 28195639 - 7
Low-dose phenobarbital for epilepsy with myoclonic absences: A case report.
Ito S, Nagumo K, Nishikawa A, et al.
Brain & development 2021; (43(5)):666-668 doi:10.1016/j.braindev.2020.12.018.
PMID: 33461850 - 8
Management of epilepsy with eyelid myoclonia: Results of an international expert consensus panel.
Smith KM, Wirrell EC, Andrade DM, et al.
Epilepsia 2023; (64(9)):2342-2350 doi:10.1111/epi.17682.
PMID: 37326215 - 9
Unmasking the entity of 'drug-resistant' perioral myoclonia with absences: the twitches, darts and domes!
Rudrabhatla PK, Er S, Radhakrishnan A, Menon RN
Epileptic disorders : international epilepsy journal with videotape 2021; (23(2)):313-324 doi:10.1684/epd.2021.1265.
PMID: 33851919 - 10
Clinical Management of Drug Resistant Epilepsy: A Review on Current Strategies.
Guery D, Rheims S
Neuropsychiatric disease and treatment 2021; (17()):2229-2242 doi:10.2147/NDT.S256699.
PMID: 34285484 - 11
Dietary Therapies: Emerging Paradigms in Therapy of Drug Resistant Epilepsy in Children : Based on 6th Dr. I. C. Verma Excellence in Research Award Oration.
Gulati S
Indian journal of pediatrics 2018; (85(11)):1000-1005 doi:10.1007/s12098-018-2779-9.
PMID: 30242606 - 12
The changing face of dietary therapy for epilepsy.
Pasca L, De Giorgis V, Macasaet JA, et al.
European journal of pediatrics 2016; (175(10)):1267-76 doi:10.1007/s00431-016-2765-z.
PMID: 27586246 - 13
Vagus Nerve Stimulation and Seizure Outcomes in Pediatric Refractory Epilepsy: Systematic Review and Meta-analysis.
Jain P, Arya R
Neurology 2021; (96(22)):1041-1051 doi:10.1212/WNL.0000000000012030.
PMID: 33849993 - 14
Timing of referral to evaluate for epilepsy surgery: Expert Consensus Recommendations from the Surgical Therapies Commission of the International League Against Epilepsy.
Jehi L, Jette N, Kwon CS, et al.
Epilepsia 2022; (63(10)):2491-2506 doi:10.1111/epi.17350.
PMID: 35842919
This page about EMA medicines and treatment options is for informational purposes only and does not constitute medical advice. Do not change or stop your child's medicine without guidance from the prescribing clinician.