Pediatric Doose Syndrome
Doose syndrome is a seizure disorder in young children that causes difficult-to-control generalized seizures.
What is Pediatric Doose Syndrome?
Doose Syndrome, also called Myoclonic-Astatic Epilepsy (MAE), is an epileptic condition in children that has no known cause. The seizures, which often begin between the ages of 1 and 5, can be frequent and involve the abrupt loss of muscle control, causing the child to fall to the ground, often resulting in injury. Boys are twice as likely as girls to have the syndrome. The seizures originate from all parts of the brain, which makes controlling them with medication difficult.
The Epilepsy Center at Children’s Health is the first program in the country to be certified by the Joint Commission, the nation’s preeminent standard-setting accrediting and certifying regulatory body in healthcare. We are also a Level 4 Epilepsy Center, providing the highest level of treatment for children with epilepsy. That means we have experience identifying Doose Syndrome, and in treating the condition.
Our interdisciplinary medical team, which includes genetic counselors, has the experience to not only comprehensively diagnose children with this condition, but we remain the area’s only center to perform the most advanced procedures and therapies for the treatment of epilepsy.
At Children’s Health, our treatment includes education and support for the whole family. In addition, the Epilepsy Center works with referring physicians and area Emergency Departments to:
- Provide seizure safety education programs
- Expedite appointments in order to get your child evaluated quickly
What are the signs and symptoms of Pediatric Doose Syndrome?
Children with this condition can experience several seizures a day. They usually experience atonic drop seizures (muscles go limp) or staring seizures (staring off into space).
How is Pediatric Doose Syndrome diagnosed?
To diagnose epilepsy, your physician will do a detailed physical exam of your child, ask you about your child’s past medical history and family history, and learn as much as possible from you about how a seizure started and what you observed.
If Doose Syndrome is suspected, testing may be prescribed including an electroencephalogram (EEG) to determine where in the brain the seizures are coming from, and to determine if your child has Doose Syndrome. An EEG is performed by placing electrodes on the scalp and recording the electrical activity of the brain.
How is Pediatric Doose Syndrome treated?
With epilepsy, some children improve with age, while for others, it represents a life-long condition. While it can be difficult to control the seizures associated with Doose Syndrome with medication, there are varying degrees of severity of the condition, and some children may easily respond to the first line anti-epileptic drugs (AEDs) prescribed. Others may need second or even third medications added to achieve complete control, but there will still be some children who fail to respond even to this level of therapy.
Other treatments that have shown promise in controlling the seizures associated with Doose Syndrome, alone or in conjunctions with AEDs, include:
- A ketogenic diet, which includes a regimen of high-fat, low-carbohydrate foods.
- Injected or oral steroids.
- Vagal Nerve Stimulation.
Pediatric Doose Syndrome Doctors and Providers
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Susan Arnold, MD Pediatric NeurologistAssociate Professor at UT Southwestern Medical CenterRead Susan Arnold's Full ProfileSusan Arnold, MDPediatric Neurologist
Board Certification:
American Board of Psy&Neurology/Child Neurology -
Alison Dolce, MD Pediatric NeurologistAssistant Professor at UT Southwestern Medical CenterRead Alison Dolce's Full ProfileAlison Dolce, MDPediatric Neurologist
Board Certification:
American Board of Psy&Neurology/Child Neurology -
Andrea Lowden, MD Pediatric NeurologistAssistant Professor at UT Southwestern Medical CenterRead Andrea Lowden's Full ProfileAndrea Lowden, MDPediatric Neurologist
Board Certification:
American Board of Psy&Neurology/Neurology -
Rana Said, MD Pediatric NeurologistAssistant Professor at UT Southwestern Medical CenterRead Rana Said's Full ProfileRana Said, MDPediatric Neurologist
Board Certification:
American Board of Psy&Neurology/Child Neurology,
American Board of Psychiatry And Neurology(Epilepsy) -
Deepa Sirsi, MD Pediatric NeurologistAssistant Professor at UT Southwestern Medical CenterRead Deepa Sirsi's Full ProfileDeepa Sirsi, MDPediatric Neurologist
Board Certification:
American Board of Psy&Neurology/Clin Neurologyphysiology,
American Board of Psy&Neurology/Child Neurology
Frequently Asked Questions
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Is epilepsy always part of Doose Syndrome?
Children who are diagnosed with Doose Syndrome have a high risk for epilepsy.
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What is epilepsy?
Epilepsy is a condition that affects how the nerve cells in the brain communicate with each other. When the normal electrical activity in the brain interferes with this communication process, seizures can occur.
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What are the symptoms of a seizure?
The epilepsy symptoms most people recognize are shaking (called convulsions) combined with the body getting rigid, clenching the jaw, and biting the tongue. These are called tonic-clonic seizures and many people still know them as grand mal seizures. But some seizures are barely noticeable. Children may stare into space, stop talking abruptly and then start talking again, or start repetitious movements like chewing their lips or moving a hand. These types of seizures are called absence seizures, which used to be known as petit mal seizures. Sometimes a child may experience smelling something that isn’t there, numbness or a tingling sensations before the seizure. These are called auras.
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What should I do while my child is having a seizure?
If the seizure is small twitching or staring, they should come out of it easily. For convulsions, make sure your child is in a chair that they can’t fall out of or lying on the floor with a pillow under their head. Turn their head to the side, but NEVER try to put anything into their mouth. Do not shake or hit them or shout at them. If the seizure lasts for more than five minutes or seems violent, call 9-1-1.
After a seizure, your child may be confused, foggy or tired. If they want to lie down, let them.
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What kind of tests are there for epilepsy?
Doctors first need to be sure that seizure aren’t due to something else before they diagnose epilepsy. Simple blood work and/or kidney tests might find that there is an infection causing seizures, and that might be cured easily. Otherwise there are a variety of brain-related tests, including an electroencephalogram (EEG), CT scan and MRI that can show the electrical activity in the brain that indicates epilepsy.
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How is epilepsy treated?
There is no one specific treatment for any child with epilepsy. Each treatment plan is developed based on the child’s diagnosis and symptoms. For many children, medication can control seizures. Special diets can also help. If seizures happen so often that they reduce the child’s quality of life, surgery may be necessary. Surgery can include removing the section of brain where seizures originate or implanting a small device that regulates electronic brain activity.
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What are the side effects of epilepsy treatment?
There can be side effects from treatment as well as from the disorder. Treatments can cause dizziness, fatigue and lead to balance problems. Seizure drugs can also cause bone density loss. Living with epilepsy can be stressful. A child may feel isolated, alone or depressed. Support groups and counseling can help.
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Where can I find a support group?
Our Epilepsy Center will provide you with resources to help both you and your child. The Resources link on this webpage is also a good source for more information about epilepsy and support groups.