Doose Syndrome

Doose Syndrome, also known as Epilepsy with Myoclonic Atonic Seizures (EMAS), is an epilepsy syndrome of childhood that is often resistant to medication.

Previously also known as Myoclonic Astatic Epilepsy (MAE), the syndrome is usually characterised by generalised seizures, which may vary in type and frequency. These are most often myoclonic and/or myoclonic-atonic, (drop seizures) astatic or generalised tonic-clonic seizures. Absence seizures can also occur, as can non-convulsive status epilepticus (a period of prolonged seizure activity.)

Many children experience large numbers of seizures daily, which contributes to difficulty in treatment.

Prevalence

This type of epilepsy is not common, and occurs in 1-2 children out of 100 children with epilepsy and more commonly in boys than girls. Onset generally occurs between ages one and five, usually in children with an uneventful history. In some cases, there is a positive family history of seizures, and family studies over the years have supported a genetic basis.

The ketogenic diet, which is a specialised high fat- low carbohydrate diet may be an effective treatment for children with this syndrome.