Short answer · Medically reviewed summary · Last updated: 2026-04-07

TL;DR: Doose syndrome, also known as Myoclonic Atonic Epilepsy (MAE), is a rare form of childhood epilepsy characterized by multiple seizure types, particularly myoclonic-atonic seizures, and often associated with developmental delays or cognitive challenges. It typically begins in early childhood and is considered a generalized epilepsy syndrome that can be challenging to treat with standard anti-seizure medications. What is Doose syndrome? Doose syndrome is a distinct, rare epilepsy syndrome that primarily affects young children.

What is Doose Syndrome

Name: What is Doose Syndrome
Creator: DiseaseMaps Editorial Team
Published: 2015-09-13UTC12:48:14.0000000

What is Doose Syndrome? Plain-language, medically reviewed definition plus the lived reality told by patients.

TL;DR: Doose syndrome, also known as Myoclonic Atonic Epilepsy (MAE), is a rare form of childhood epilepsy characterized by multiple seizure types, particularly myoclonic-atonic seizures, and often associated with developmental delays or cognitive challenges. It typically begins in early childhood and is considered a generalized epilepsy syndrome that can be challenging to treat with standard anti-seizure medications.

What is Doose syndrome?

Doose syndrome is a distinct, rare epilepsy syndrome that primarily affects young children. The hallmark of Doose syndrome is the occurrence of myoclonic-atonic seizures, which involve a sudden muscle jerk (myoclonus) followed immediately by a loss of muscle tone (atonia), often causing the child to fall. Because these seizures frequently result in sudden drops, they are sometimes referred to as "drop attacks." In the DiseaseMaps community, 65 individuals have shared their experiences, highlighting the diverse ways this condition impacts daily life and development.

Which body systems are affected by Doose syndrome?

While Doose syndrome is a neurological condition, its effects extend beyond the brain. The primary impact is on the central nervous system, leading to recurrent seizure activity. However, the recurring nature of these seizures and the potential for status epilepticus (prolonged seizure activity) can affect a child’s physical safety, coordination, and cognitive development. Many children with Doose syndrome experience fluctuations in their cognitive abilities, language development, and executive function, which may be impacted both by the seizures themselves and the medications used to manage them.

How common is Doose syndrome and who is affected?

Doose syndrome is quite rare, accounting for approximately 1% to 2% of all childhood epilepsies. It typically has an onset between the ages of 7 months and 7 years, with a peak incidence occurring between 2 and 4 years of age. While it can affect any child, studies suggest a slight male predominance. There is no geographic or ethnic predilection for the condition, and it occurs globally.

What causes Doose syndrome and how is it classified?

The exact underlying mechanism of Doose syndrome remains a subject of active research. It is generally classified as a genetic generalized epilepsy, meaning it is not caused by a single structural lesion in the brain. Instead, it is thought to be a polygenic condition, where multiple genes interact with environmental factors to lower the seizure threshold. Key clinical features that differentiate Doose syndrome from other childhood epilepsies include:

Seizure Variety: Patients often experience a mix of myoclonic, atonic, absence, and generalized tonic-clonic seizures.

EEG Patterns: The electroencephalogram (EEG) often shows generalized spike-wave or polyspike-wave discharges, which are characteristic of this syndrome.

Cognitive Impact: Unlike some benign childhood epilepsies, Doose syndrome is frequently associated with varying degrees of learning disabilities or developmental delays.

Treatment Response: Doose syndrome is often considered "treatment-resistant," meaning it does not always respond well to traditional anti-seizure drugs, often requiring alternative approaches like the ketogenic diet.

Next steps

Consult a Pediatric Epileptologist: Seek care from a specialist who has specific experience managing complex, genetic epilepsy syndromes.

Keep a Seizure Diary: Track the frequency, duration, and triggers of seizures to help your medical team adjust treatment plans effectively.

Join a Support Community: Connect with other families through platforms like DiseaseMaps.org to share resources and coping strategies.

Explore Dietary Therapies: Discuss the potential role of the ketogenic or modified Atkins diet with your neurologist, as these are often highly effective for Doose syndrome.

Medical disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment; always consult with a qualified healthcare provider regarding your specific medical condition.