This type of epilepsy is uncommon and happens in one to two out of 100 children with epilepsy. It usually starts around ages one-and-a-half to five years. It is more common in boys than girls. In about a third of cases some other family member also has epilepsy though not always of the same sort.
Symptoms
The seizures can be very different, consisting of jerks, sudden falls to the ground, or sometimes a jerk followed by a fall. Absences can happen when consciousness is lost briefly, a
nd in some cases there may be major seizures with stiffness and jerking all over (generalised tonic-clonic seizure).
Approximately one-third of children with myoclonic astatic epilepsy will have a very long seizure at some time during their childhood or adolescence.
Diagnosis
A full account of what happens during the seizures is essential for the doctors making the diagnosis.
The electroencephalogram (EEG) test, which records the electrical activity in the brain, 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.
Treatment
Treatment of myoclonic astatic epilepsy is often very difficult. The medications which have been most successful are sodium valproate (Epilim), lamotrigine (Lamictal) and occasionally clonazepam, clobazam or nitrazepam.
Prognosis (Outlook)
The outlook for children with myoclonic astatic epilepsy is rather variable.
In half of cases there is a good response to medication but the children will continue to have learning difficulties.
In a very few cases the seizures come under control and the child’s learning progress is quite good.
In the remainder, seizures continue despite medication and children develop significant learning difficulties.
Prognosis is more likely to be unfavourable in children who have generalised tonic-clonic seizures at the start of their seizures.
Support organisations
Contact a Family, 209-211 City Road, London, EC1V 1JN, telephone 0808 808 3555, www.cafamily.org.uk
Because this page is written by an epilepsy healthcare professional and not by Epilepsy Action, it falls outside the requirements of the Information Standard.
Our thanks
Epilepsy Action is indebted to Dr Richard Appleton, a Consultant Paediatric Neurologist who specialises in children’s epilepsy, and to Dr Rachel Kneen, Consultant Paediatric Neurologist and Dr Stewart Macleod, Specialist Registrar in paediatric neurology, at Alder Hey at Alder Hey Children’s Hospital, Liverpool, who have kindly prepared the information on this page.
This information is exempt under the terms of The Information Standard.
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Updated July 2009To be reviewed December 2013
Comments: read the 2 comments or add yours
Comments
My 13 yr old son has MAE and has multiple grand mal seizures every week (8-10), in addition to absence, complex partial, tonic and myoclonic seizures. He has a VNS, has tried diet and has been on many, many types of meds and combinations of meds. He has had a Corpus Callosotomy and Resection surgery yet continues to have seizures. With each procedure, things have been better or different, but nothing has stopped them. He is now developmentally about a 4 year old and has short term memory loss. Daily life can be very trying for him as well as our family, but we do everything we can to make things better. I would love to find out about any research studies or information that might help my sweet son.
Hi Cindy
It sound like your son has a very loving and supporting family. It can be so difficult for everyone living with epilepsy that isn’t controlled.
Doose Syndrome is a rare condition and there isn’t much information on the syndrome.
A good source for research papers is the Pubmed website. PubMed comprises more than 22 million citations for biomedical literature from MEDLINE, life science journals, and online books.
To find out if there is any research taking place, you could look into the following suggestions.
Make your son’s epilepsy specialist aware you are interested in any research past or present.
Epilepsy Action has a research department. We support a wide range of activities from funding research studies, to offering opportunities for patients and public to get involved in the research process. You will find out more about this on our research webpage.
You may wish to view Epilepsy Research UK website. They support and promote basic and clinical scientific research into the causes, treatments and prevention of epilepsy.
If you’d like to make contact with other families who have a child with Doose Syndrome, you could try Contact a Family and our forum – forum4e. Many people find our forum a good source for contacting others who understand what you are experiencing.
I hope this is of help. If we can help any further please feel free to contact our Advice and Information Team, either email, helpline@epilepsy.org.uk or the Epilepsy Helpline freephone 0808 800 5050.
Yours sincerely
Diane