Myoclonic astatic epilepsy (Doose syndrome)

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 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, and 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

9 March 2007