This information is for people affected by or concerned about epilepsy with myoclonic-atonic seizures, including parents and carers. On this page we talk about how epilepsy with myoclonic-atonic seizures is diagnosed, what causes it, symptoms, treatment options and long-term outlook.
Use this page as a general guide and speak to a health professional for more information and support.
On this page
What is epilepsy with myoclonic-atonic seizures?
Epilepsy with myoclonic-atonic seizures is a rare form of epilepsy. It was previously known as Doose syndrome and myoclonic-astatic epilepsy.
Epilepsy with myoclonic-atonic seizures affects around two in every 100 children with epilepsy. It usually starts between the ages of two and six years, but may begin as young as six months or as late as eight years. It’s slightly more common in boys than girls.
About a quarter of children who develop epilepsy with myoclonic-atonic seizures have previously had febrile seizures (seizures triggered by a high temperature).
What causes epilepsy with myoclonic-atonic seizures?
Though it’s not clear exactly what causes epilepsy with myoclonic-atonic seizures, it’s thought that genetics may play an important role.
Mutations (changes) in a number of different genes are likely to lead to children developing the condition. Not all children with this type of epilepsy have an identified gene mutation, but mutations have been identified in some cases.
What are the signs and symptoms of epilepsy with myoclonic-atonic seizures?
Children with epilepsy with myoclonic-atonic seizures have many types of seizures. The main type is myoclonic-atonic seizures, which is what gives the syndrome its name. Find out more about myoclonic and atonic seizures.
Children with epilepsy with myoclonic-atonic seizures will experience drop seizures. These usually involve an abrupt fall (atonic) or a brief body jerk followed by an abrupt fall (myoclonic-atonic).
Other types of seizures include:
- Generalised tonic-clonic seizures – seen in most children with this type of epilepsy and may be triggered by a fever
- Absence seizures and myoclonic seizures
- Tonic seizures, which can be seen later as the condition progresses
Find out more about tonic, tonic-clonic and absence seizures.
Epilepsy with myoclonic-atonic seizures often begins very suddenly and the child may go through ‘active phases’ when they experience lots of different seizures. During the active phase, you may notice delays in your child’s development, including their behaviour, memory, concentration and co-ordination. These all normally improve once seizures are controlled.
Many children with epilepsy with myoclonic-atonic seizures may have prolonged seizures at some point. These are seizures that can last for hours or even days. This is called non-convulsive status epilepticus. You should discuss an emergency care plan with your child’s doctor in case this happens. Find out more about non-convulsive status epilepticus and emergency medication.
How is epilepsy with myoclonic-atonic seizures diagnosed?
If you think a child has epilepsy, including epilepsy with myoclonic-atonic seizures, contact their doctor. You may be referred to a paediatrician (a doctor who specialises in child health).
In some cases, your child’s paediatrician may refer you to tertiary care – a specialist neurology centre based in a regional hospital. This is usually because of a child’s age and because it may be difficult to control their seizures.
Find out more about preparing for consultations and appointments and keeping accurate seizure records.
Tests for diagnosing epilepsy with myoclonic-atonic seizures
Your child may be offered an EEG (electroencephalogram) test – a painless test that uses small sensors attached to the scalp to pick up the electrical signals produced by the brain. Although EEG testing cannot confirm or rule out a diagnosis of epilepsy, it can help doctors to identify patterns in brain activity.
Your child may also be offered an MRI (magnetic resonance imaging) scan – a scan that uses magnetic fields and radio waves to produce images from inside the body. MRI results are usually normal in children with epilepsy with myoclonic-atonic seizures.
Blood tests may also be needed to confirm epilepsy with myoclonic-atonic seizures.
Treatment for epilepsy with myoclonic-atonic seizures
It is important for people with epilepsy with myoclonic-atonic seizures to be treated as quickly as possible with the most effective medicines. Successful treatment depends on accurate diagnosis.
Remember that everyone is different and what works for one person might not work well for someone else. People with this type of epilepsy may have to try a few different medicines until something works well.
Talk to a doctor about treatment options and what might be suitable. They should explain the risks and benefits of each treatment, including any possible side effects.
First treatment options
For epilepsy with myoclonic-atonic seizures, the first treatment options are:
- Levetiracetam (also known as Keppra) – a medicine that comes as a tablet, liquid or granules, that you swallow or mix with water.
- Sodium valproate (also known as Epilim) – a medicine that comes as capsules, tablets or a liquid that you swallow. It also comes as granules that you mix with food or drink. It can also be given by injection, but this is usually only done in hospital or in emergencies.
If one of these medicines does not work, then the other option can be tried.
However, sodium valproate may not suitable for everyone. In women and girls sodium valproate may affect fertility (the ability to get pregnant naturally).
For women who can have children, and young girls who are likely to need treatment when they’re old enough to have children, a doctor should consider the following things before prescribing sodium valproate:
- The benefits and risks of sodium valproate, including any risks to an unborn child.
- The likelihood of pregnancy and if appropriate, a pregnancy prevention programme should be put in place.
Read more about the process for prescribing sodium valproate and how it can affect fertility.
Sodium valproate
In January 2024, new rules were introduced for prescribing sodium valproate.
Two specialists are now needed to approve:
- New or ongoing valproate treatments for girls and women.
- New valproate treatments for boys and men
The specialists must agree that your epilepsy does not respond to other treatments and the benefits of treatment outweigh the risks.
For girls and women, valproate can seriously harm an unborn baby when taken during pregnancy.
For boys and men, valproate may cause infertility. Some people’s fertility may return when their treatment is stopped or reduced. There is also some evidence that valproate can cause harm in the testicles of animals, but it’s unclear what this means for humans.
Never stop taking valproate before talking to your specialist first. Not taking medication as prescribed can increase the risk of a seizure.
Your epilepsy specialist will let you know when your treatment is due to be reviewed.
If you are planning a pregnancy it is important to speak to your healthcare professional as soon as possible about your treatment options.
If you have any questions or concerns about valproate, please speak to your healthcare professional.
Find out more about sodium valproate in the MHRA’s patient guide.
Find out more about Anti-seizure medications, contraception and pregnancy.
Second treatment options
If the first treatment options do not work, a ketogenic diet can be tried either on its own or as an add-on treatment, under the supervision of a ketogenic diet team. This is a special type of diet that is high in fat and protein, but low in carbohydrates. It can work well at reducing the intensity and frequency of seizures.
Third treatment options
If the second treatment option does not work, the doctor may suggest trying one of the following anti-seizure medications either on their own or as an add-on treatment:
- Clobazam (also known as Frisium and Onfi) – a medicine that comes as tablets or liquid, that you swallow.
- Ethosuximide (also known as Zarontin) – a medicine that comes as a capsule or solution, that you swallow.
- Topiramate (also known as Topamax) – a medicine that comes as a tablet or capsule, that you take by mouth.
- Zonisamide (also known as Zonegran) – a medicine that comes as a capsule, that you swallow.
Stopping treatment with anti-seizure medications
For some children, it may be possible to stop anti-seizure medication altogether if they have not had any seizures for at least three years. Remember, never stop taking medicine without talking to a health professional first. If it’s possible to stop treatment, the specialists involved in your child’s care will gradually and safely reduce the dose.
What’s the long-term outlook for people with epilepsy with myoclonic-atonic seizures?
The long-term outlook for children with epilepsy with myoclonic-atonic seizures varies from person-to-person.
It’s common for anti-seizure medications not to work at first. But, in two-thirds of children with this type of epilepsy, the seizures usually stop within three years. For some children, it can sometimes take a little longer.
You may notice that children with this type of epilepsy have some behavioural or developmental problems. This may be particularly obvious if a child is having lots of seizures or taking more than one anti-seizure medicine at the same time. However, these behavioural and development problems usually go away once seizures are under control and are being managed with the correct medicine. Find out more about how epilepsy affects learning and behaviour.
Living with epilepsy with myoclonic-atonic seizures and getting support
Having a child with any form of epilepsy can be challenging and upsetting. Talk to the child’s doctor for help on how to support them well. And talk to your own doctor if you need support for yourself. If you’re caring for someone with epilepsy with myoclonic-atonic seizures, it’s important that you look after yourself and get any support you need.
You may find it helpful joining an in-person or online support group, where you can listen to or share experiences with other people who understand what you’re going through.
Other useful organisations
Doose Syndrome Epilepsy Alliance
An American organisation that provides information for people affected by epilepsy with myoclonic-atonic seizures.
