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.