Epilepsy with Myoclonic-Atonic Seizures (Doose Syndrome)
Epilepsy with Myoclonic-Atonic Seizures (Doose Syndrome)
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Authors: Theresa Estiphan, MD
Michigan Medicine, University of Michigan  

Charuta Joshi, MBBS, MD
University of Texas Southwestern – Dallas Children’s Hospital 

Reviewed: March 2022 


Epilepsy with myoclonic-atonic seizures (EMAS) is a rare form of childhood epilepsy. Seizures are caused by abnormal electrical firings in the brain. In epilepsy, seizures occur repeatedly. 

Childhood epilepsy begins between 1 month and 18 years of age. EMAS causes 1% to 2% of all cases of childhood epilepsy. In EMAS, myoclonic-atonic seizures are the most common type of seizure. Myoclonic-atonic seizures begin with a brief jerk, which is the myoclonic component. This is followed by a loss of muscle tone, the atonic component.  

Myoclonic-atonic seizures can cause either sudden falls or more subtle head movements downward onto the chest. The head movements are called head drops.

EMAS is also known as myoclonic-astatic epilepsy (MAE) or Doose syndrome. 


Disorder Overview


EMAS is one of many forms of childhood epilepsy. It is unique because it mostly causes myoclonic-atonic seizures. Children with EMAS usually have normal development before any seizures begin. However, a small number of children with EMAS have mild delays prior to having seizures. 

The first seizure in EMAS usually occurs between 2 and 6 years of age.  

In 1 in 4 children, the first seizure occurs during a fever. Boys have EMAS more often than girls.  

Children with EMAS mostly have generalized seizures, or seizures that affect the whole brain. Focal seizures affect only one part of the brain, and they are rarely reported in EMAS. EMAS can include multiple types of seizures, such as:

Sometimes, in EMAS, seizures can change a child’s level of consciousness. This can happen without obvious signs of a seizure. For instance, there may be no jerking. When this occurs and goes on for a long period of time, it is called nonconvulsive status epilepticus.

Myoclonic seizures

These cause a brief jerking movement.

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Atonic seizures

These cause a drop due to a sudden loss of muscle tone.

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Myoclonic-atonic seizures

These cause a brief jerking movement and a drop due to a sudden loss of muscle tone.

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Tonic seizures

These cause a stiffening of the whole body and can result in fall.

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Tonic-clonic seizures

These are also known as grand-mal seizures. They cause the entire body to stiffen briefly. This is followed by a rhythmic shaking.

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Absence seizures

These are also known as petit-mal seizures. They can cause staring, unresponsiveness, or slow responses.

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Sometimes, in EMAS, seizures can change a child’s level of consciousness. This can happen without obvious signs of a seizure. For instance, there may be no jerking. When this occurs and goes on for a long period of time, it is called nonconvulsive status epilepticus.

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Main Symptoms 

The primary symptom of EMAS is having myoclonic-atonic seizures. 

Developmental setbacks, however, are also a symptom. They are seen as EMAS evolves. When seizures begin, a child’s development is usually normal or only mildly delayed. Developmental setbacks begin around the same time as the seizures. Many researchers believe that the seizures contribute to the developmental setbacks, even though there could be a different cause underlying both.  

 Parents of children with EMAS may report: 

  • Regression (a loss of milestones) 
  • Developmental plateau (a failure to gain additional skills) 

Stormy Phase 

Most children experience an active phase, when seizures can get very frequent and there are many seizure types. This seizure activity, along with the setbacks, is known as the “stormy phase.”

Other Symptoms 

Other symptoms that can appear alongside setbacks include: 

  • Poor balance 
  • Speech and language problems 
  • Poor memory 
  • Poor attention 
  • Behavioral challenges 
  • Features of autism spectrum disorder

Diagnosing EMAS 

EMAS is diagnosed when families or doctors notice myoclonic-atonic seizures. Doctors can learn a little about the seizures from a clinical exam and health history. They may ask about seizure types and when seizures began. They will often use lab investigations to learn more.

 Other factors that point to an EMAS diagnosis include: 

  • Development that had been normal or close to normal prior to seizures 
  • Seizures that began between 2 and 6 years of age 
  • Normal brain imaging results 
  • No other known reasons for these seizures (they can also be caused by a disorder called GLUT1 syndrome) 


The cause of EMAS is not currently known. Genetics seem to play a role. However, no single genetic abnormality seems to cause it.  

Several genetic differences have been linked with EMAS. For instance, the SLC6A1 gene and the SYNGAP1 gene are thought to be important in EMAS. Future work may identify more genes of interest.  

In 1 in 3 EMAS cases, there is a family history of seizures, including fever-related seizures.


There is no single test that can diagnose EMAS. Rather, doctors must use clinical data along with lab studies and tests.

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Doctors may order the following laboratory tests: 

  • Electroencephalogram (EEG). An EEG measures electrical activity in the brain. It can show if a child is having myoclonic-atonic seizures. However, it can only show this if the seizures are recorded during the EEG. 
  • Magnetic resonance imaging (MRI). MRI results are usually normal in children with EMAS. MRI can help to rule out other forms of epilepsy. 
  • Genetic testing. Genetic testing can try to identify a gene associated with EMAS. It can also make sure that serious genetic disorders that look like EMAS are not missed.



Several antiseizure medications can be used to try to control seizures in EMAS. Doctors choose them based on the types of seizures a child has. They are often used in combination. Ask your doctor to discuss medication options that are right for your child.

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The ketogenic diet is an effective treatment option for EMAS. It is a diet high in fat and low in carbohydrates (sugars). It is especially effective during the stormy phase.It can help prevent some of the seizures and developmental setbacks. 

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The outcomes in EMAS can vary. The goal of treatment is to improve children’s outcomes. Early, aggressive treatment can improve outcomes. Some children can even catch up from setbacks once the seizure activity is stopped or reduced.



Doose Syndrome Epilepsy Alliance

Doose Syndrome Epilepsy Alliance (DSEA) exists to improve lives for those living with Doose syndrome and their families. DSEA provides a chance to connect with other families through their Doose Foundation private Facebook group. The DSEA website provides important Resources for Next Steps, Finding Support, Newly Diagnosed, and much more.

Epilepsy Leadership Council  

The Epilepsy Leadership Council is made up of individuals representing organizations serving individuals with epilepsy and their families, as well as professionals, and governmental organizations. The mission is to develop and coordinate among its members shared projects that will have a positive impact on the lives of individuals with epilepsy, focusing on those areas where working together produces greater efficiency and impact than working independently.  

For a list of more than 40 professional societies, patient advocacy organizations, and governmental agencies, please click here. 

Childhood Stroke 1

Pediatric Epilepsy Surgery Alliance
The Pediatric Epilepsy Surgery Alliance (formerly known as The Brain Recovery Project) enhances the lives of children who need neurosurgery to treat medication-resistant epilepsy. They empower families with research, support services, and impactful programs before, during, and after surgery. PESA’s programs include research-based, reliable information to help parents and caregivers understand when a child’s seizures are drug-resistant; the risks and dangers of seizures; the pros and cons of the various neurosurgeries to treat epilepsy; the medical, cognitive, and behavioral challenges a child may have throughout life; school, financial aid, and life care issues. PESA’s resources include a comprehensive website with downloadable guides, pre-recorded webinars, and virtual workshops; an informative YouTube channel with comprehensive information about epilepsy surgery and its effects; a private Facebook group (Education After Pediatric Epilepsy Surgery) with over 300 members; Power Hour (bi-monthly open forums and live virtual workshops on various topics); and free school training to help your child’s education team understand the impact of their epilepsy surgery in school. Their Peer Support Program will connect you with a parent who has been there. The Pediatric Epilepsy Surgery Alliance also hosts biennial family conferences and regional events that allow families to learn from experts, connect with other families, and form lifelong friendships. They also provide a travel scholarship of up to $1,000 to families in need to fund travel to a level 4 epilepsy center for a surgical evaluation.

In addition, PESA has resources for medical professionals to assist in helping clinicians help the parents of their patients find the resources they need after surgery. Educators and therapists will also find helpful resources and information, including videos, guides, and relevant research. Patients who have undergone surgery are encouraged to register with the Global Pediatric Epilepsy Surgery Registry to help set future research priorities.

Child Neurology Foundation (CNF) solicits resources from the community to be included on this webpage through an application process. CNF reserves the right to remove entities at any time if information is deemed inappropriate or inconsistent with the mission, vision, and values of CNF. 


ClincalTrials.gov for Epilepsy with Myoclonic-Atonic Seizures (Doose Syndrome) are clinical trials that are recruiting or will be recruiting. Updates are made daily, so you are encouraged to check back frequently.  

ClinicalTrials.gov is a database of privately and publicly funded clinical studies conducted around the world. This is a resource provided by the U.S. National Library of Medicine (NLM), which is an institute within the National Institutes of Health (NIH). Listing a study does not mean it has been evaluated by the U.S. Federal Government. Please read the NLM disclaimer for details.  

Before participating in a study, you are encouraged to talk to your health care provider and learn about the risks and potential benefits. 

Family Stories 

Learn all about individuals living with Doose syndrome on the Doose Syndrome Epilepsy Alliance Home page. Scroll down to the bottom of the page to “meet” these exceptional children and/or Submit Your Story. 

The information in the CNF Child Neurology Disorder Directory is not intended to provide diagnosis, treatment, or medical advice and should not be considered a substitute for advice from a healthcare professional. Content provided is for informational purposes only.  CNF is not responsible for actions taken based on the information included on this webpage. Please consult with a physician or other healthcare professional regarding any medical or health related diagnosis or treatment options. 


Epilepsy with myoclonic-atonic seizures [Internet]. International League Against Epilepsy; [cited 2022 Feb 20]. Available from: https://www.epilepsydiagnosis.org/syndrome/epilepsy-myoclonic-atonic-overview.html 

Nickels K, Kossoff EH, Eschbach K, Joshi C. Epilepsy with myoclonic-atonic seizures (Doose syndrome): Clarification of diagnosis and treatment options through a large retrospective multicenter cohort. Epilepsia. 2021 Jan;62(1):120-127. https://doi.org/10.1111/epi.16752. Epub 2020 Nov 14. PMID: 33190223. 

Joshi C, Nickels K, Demarest S, Eltze C, Cross JH, Wirrell E. Results of an international Delphi consensus in epilepsy with myoclonic atonic seizures/ Doose syndrome. Seizure. 2021 Feb; 85:12-18. https://doi.org/10.1016/j.seizure.2020.11.017. Epub 2020 Dec 28. PMID: 33383403. 

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