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.
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.
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.
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 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.
Information about treatments for children can be found on the Medicines for Children website.
There may be research studies open that are relevant to this epilepsy syndrome. Your child’s doctor will be able to discuss this with you.
If your child has this syndrome they may have prolonged or repeated seizures. Your child’s doctor will discuss a ‘rescue’ or emergency care plan with you to treat any prolonged or repeated seizures.
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).
You may be interested in taking part in some research into Doose syndrome. They are recruiting until January 2016.
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.