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Myoclonic astatic epilepsy (Doose syndrome)

This type of epilepsy is uncommon. It affects only one to 2 out of 100 children with epilepsy. It usually starts around the ages of 18 months to 5 years and is more common in boys than girls.

The cause of myoclonic-astatic epilepsy is not known. It is probably caused by a genetic abnormality but this has not been proven. In about a third of cases another family member will also have epilepsy, although not always of the same sort.

The term ‘atonic’ can sometimes be used instead of ‘astatic’. Both of these words mean ‘loss of tone’ which causes the person to fall to the ground.

Other names for myoclonic-astatic epilepsy

• Doose syndrome


The seizures can be very different, consisting of jerks (myoclonic seizures), sudden falls to the ground (atonic or astatic seizures), or sometimes a jerk followed by a fall (myoclonic-astatic seizure). Absence seizures can happen (when consciousness is lost briefly). In some cases there may be seizures with stiffness and jerking of the whole body (generalised tonic-clonic seizure). Approximately one third of children with myoclonic-astatic epilepsy will have prolonged seizures at some time during childhood or adolescence.


The EEG (electroencephalogram), which records the electrical activity in the brain, is usually abnormal. It shows evidence of epileptic discharges from both sides of the brain at the same time, and sometimes from all over the brain. The EEG during sleep may show an increase in epileptic discharges. The type of abnormal activity is called ‘spike and wave’ or ‘polyspike and wave’. Children with myoclonic-astatic epilepsy are not usually photosensitive.

Brain scans, including magnetic resonance imaging (MRI) scans, and blood tests are usually normal.


Treatment of myoclonic-astatic epilepsy is often very difficult. The epilepsy medicines that have been most successful are sodium valproate (Epilim), topiramate (Topamax), lamotrigine (Lamictal) and occasionally clobazam (Frisium), ethosuximide (Zarontin) or nitrazepam. Levetiracetam (Keppra) may also be helpful, particularly for the myoclonic seizures. Children will usually need to be treated with a combination of two epilepsy medicines.

Importantly, this type of epilepsy can respond well to the ketogenic diet. Because of this the ketogenic diet should be considered when the seizures have not stopped after treatment with two epilepsy medicines.

There is a surgical treatment which may very occasionally be used in children with myoclonic-astatic epilepsy – and in only one situation. This is when children continue to have many seizures each and every day when they fall or drop to the ground – and that have not stopped with epilepsy medicines and the ketogenic diet. The surgical treatment is called a ‘corpus callosotomy’.

Information about treatments for children can be found on the Medicines for Children website.

There may be research studies that are going on in this epilepsy syndrome. Your child’s hospital doctor will be able to discuss this with you.

If your child has this syndrome there is a high chance that they may have prolonged or repeated seizures, including status epilepticus. Your child’s hospital doctor (paediatrician or paediatric neurologist) will discuss a ‘rescue’ or emergency care plan with you to treat any prolonged or repeated seizures. It is important that all children with myoclonic-astatic epilepsy have an up-to-date rescue or emergency care plan.


The outlook for children with myoclonic-astatic epilepsy is variable. In half of cases there is a good response to epilepsy medicines, but the children will have some learning difficulties. In a few cases the seizures come under control and the child’s learning will be satisfactory or possibly only a little slow or delayed.  In the remainder of cases, seizures continue despite treatment. These children usually develop moderate or sometimes severe learning difficulties. The prognosis is generally worse in children where the epilepsy begins with generalised tonic-clonic seizures, or if seizure control does not respond to the first anti-epileptic medicine that is prescribed.


Freephone helpline (UK only): 0808 808 3555
Website: cafamily.org.uk
Email: info@cafamily.org.uk

What is a syndrome?

A syndrome is a group of signs and symptoms that, added together, suggest a particular medical condition. In epilepsy, examples of these signs and symptoms would be things like the age at which seizures begin, the type of seizures, whether the child is male or female and whether they have physical or learning disabilities, or both. The results of an electroencephalogram (EEG) are also used to help identify epilepsy syndromes.

If you would like to know more about an epilepsy syndrome, please speak to your doctor. If you would like to know more about epilepsy in general, please contact Epilepsy Action.


Epilepsy Action would like to thank Dr Richard Appleton, consultant paediatric neurologist at Alder Hey Children’s Hospital, Liverpool, UK for preparing this information.

This information is exempt under the terms of The Information Standard.

  • Updated November 2016
    To be reviewed November 2019

Comments: read the 8 comments or add yours


my baby two years is having some period of laughter with shaking of head folkowed by crying. could it be an epilepsy.

Submitted by jane nyaga on

Hi Jane
Thank you for your question.

As trained epilepsy advisers, we can give general information on epilepsy and related issues. But we’re not able to diagnose.

It would be best for you to talk to your family doctor. Keep a diary and maybe try video the episodes that are causing you concern.

If necessary, your family doctor will refer your baby for further tests.

If we can be of any more help, please feel free to contact us again, either by email helpline@epilepsy.org.uk  or phone the Epilepsy Action Helpline freephone 0808 800 5050. Our helpline is open Monday to Friday, 8.30am until 5.30pm.

Diane Wallace
Epilepsy Action Advice and Information Team

Submitted by Diane, Epilepsy... on

I have a child who is 8 years old is was diagnosed with mycolonic static epylepcy when he was 2years and 10 month since then we try a combination of the seizure medication and mid to genic diet . And still not controlled 100 % and we right now he is having a seizure once a week and his neurogy saying that we run out of choice we can try VNS which is planting electonic medal in this chest. And that even not shore. Can anybody knows about this . Please let me know if there is any option left for him to stop this seizure. Thanks..

Submitted by Nadia Ahmed on

Hi Nadia

It must be hard watching all this happen for your son. We don’t have any more information than is on our website. But you may want to be in touch with Contact. This is an organisation that supports families with a disabled child. In particular they may be able to put you in touch with other families with a child with the same condition.

I hope this is helpful for you.



Epilepsy Action Helpline Team

Submitted by rich on

Hi our now 3 yr old boy was diagnosed with a working diagnosis of epilepsy a year ago today. Since then and prior to that we have been in and out of hospital for stays. He was formally diagonsed with MAE last May. He is currently in hospital now with a bad respiatory cold and got a bad seizure 3 nights ago. What I am querying is there a supplement or anything a child with MAE can take to help prevent flu or colds as when gets them it triggers also bad epilepsy and then suffers on the double. 12 months later and no real progress now on 4 medicines a night well 5 if include melatonin for getting to sleep and then 3 medicines by day. I am afraid he will get poisoined! Is the attempted cure worse than the condition with some bad side effects as u go from one medicine to the next.

Submitted by Seàn Considine on


You may have seen from our information. written by a paediatric epilepsy specialist, that normally only 2 medicines at a time are recommended. And that for some children the ketogenic diet can really help: https://www.epilepsy.org.uk/info/treatment/ketogenic-diet.

We know of no special supplements but  certainly it would be worth checking this with your son’s specialist. And it you did decide to investigate the ketogenic diet, the dietician would certainly be able to give you their thoughts on this.

You might also want to look at our information on getting the right treatment and care. This is because I’m not sure you are currently being seen by a specialist centre: https://www.epilepsy.org.uk/info/treatment/getting-right-treatment-care-for-epilepsy

I do hope there is some here that will make things a little easier for him.



Epilepsy Action Helpline Team

Submitted by Cherry-Epilepsy... on

Hi my 4yr old daughter was diagnosed with MAE last year, she had been on Levetiracetam before diagnosis and then got put on sodium valporate both meds helped but didnt stop her seizures, she is also on ethosuximide and the ketogenic diet. The ketogenic diet has helped immensely that we have started to ween my daughter of Levetiracetam to see how effective the diet is...my question though is that my daughter is showing horrendous behavioural problems at home and in nursery since starting the diet and has outbursts every day saying she's hungry befire lashing out at everybody and anybody . I truely believe it has something to do with the sodium valporate and frustration from the diet. I.m hoping to get her off sodium valporate soon and swap with another med if required. Do u have any idea of what other meds i can ask her consultant to try her on.

Thank you and sorry for long winded question.

Submitted by Julie on


The information about ways to treat MAE is under the Treatment heading on this MAE page. Certainly sodium valproate is known to possibly affect behaviour especially in young children so you may well notice an improvement if she reduces that.

But you might find it useful to contact the Doose Syndrome Epilepsy Alliance. This is an American group but they still might be worth being in touch with. You could also try our online community for people with epilepsy and carers of people with epilepsy. It’s called forum4e.

I hope that helps.



Epilepsy Action Helpline Team

Submitted by rich on