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Myoclonic astatic epilepsy (Doose syndrome)

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.

Epilepsy Action has more information about seizure types, learning disabilities, and the EEG.

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.

Other names for myoclonic astatic epilepsy

• Doose syndrome

Myoclonic astatic epilepsy

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

In about a third of cases another family member will also have epilepsy, although 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. 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 a third of children with myoclonic astatic epilepsy will have a prolonged seizure at some time during their childhood or adolescence.

Diagnosis

The EEG, 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.

Epilepsy Action has more information about diagnosing epilepsy.

Treatment

Treatment of myoclonic astatic epilepsy is often very difficult. The epilepsy medicines that have been most successful are sodium valproate (Epilim), lamotrigine (Lamictal) and occasionally clonazepam (Rivotril), clobazam or nitrazepam. This type of epilepsy can respond well to the ketogenic diet.

Epilepsy Action has more information about treating epilepsy.

Outlook

The outlook for children with myoclonic astatic epilepsy is quite variable. In half of cases there is a good response to medicines, but the children will have learning disabilities. In a few cases the seizures come under control and the child’s learning progress is good. In the remainder of cases, seizures continue despite treatment. These children develop significant learning disabilities. The prognosis is generally worse in children who begin with seizures with stiffening and jerking of the limbs (generalised tonic-clonic seizures).

Support 

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

You may be interested in taking part in some research into Doose syndrome. They are recruiting until January 2016.

Pay it forward

This resource is freely available as part of Epilepsy Action’s commitment to improving life for all those affected by epilepsy.

On average it costs £414 to produce an advice and information page – if you have valued using this resource, please text FUTURE to 70500 to donate £3 towards the cost of our future work. Terms and conditions. Thank you

Code: 
S019.02

Epilepsy Action would like to thank

  • Dr Richard Appleton, and Dr Rachel Kneen, consultant paediatric neurologists at Alder Hey Children’s Hospital, Liverpool, UK and
  •  Dr Stewart Macleod, consultant paediatric neurologist at Royal Hospital for Sick Children, Glasgow, UK.
    They have kindly prepared the information on this page. It is based on their own research, experience and expertise.

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

  • Updated August 2013
    To be reviewed August 2016

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