What’s Doose Syndrome?

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Doose syndrome is a rare form of epilepsy that affects children between the ages of one and five. Symptoms include muscle jerks, difficulty standing, and seizures that can cause falls. The cause is unknown, but it may be genetic. Treatment with medication is not always successful, and learning disabilities are common. Boys are more likely to be affected than girls.

Doose syndrome is a form of epilepsy that usually first appears in young children. Symptoms include seizures that can cause jerky body movements and potentially dangerous falls. There is no known cause for the condition, also known as astatic myoclonic epilepsy (MAE).
Epilepsy is an umbrella term for medical conditions that result from abnormal electrical patterns in the brain. There are different forms of epilepsy. Doose syndrome is a form that affects children between the ages of one and five. It makes up only a small percentage of the forms of epilepsy. The alternative name for the condition is astatic myoclonic epilepsy, which provides some insight into the condition.

“Myoclonic” refers to a muscle jerk involving most parts of the body. Muscle spasms can be gentle and subtle, or strong enough to cause the child to reach up and lift their hands. “Astatic” means that the affected person cannot stand properly. This inability to stand occurs when the seizures cause the muscles in the legs to lose control and the person falls. In addition to these features of Doose syndrome, affected children may also have seizures that cause them to lose consciousness for a short time.

Doctors still don’t know what causes Doose syndrome. This form of epilepsy was only identified in the 1960s by a Dr. Herman Doose, who gave the condition his name. Children typically develop normally up to the age when symptoms of Doose syndrome appear, and then most affected individuals develop learning disabilities.

There are medications available for patients with Doose syndrome, such as valproate sodium, clonazepam, or lamotrigine, but none of these medications are successful for all patients. The effect of the condition depends on the extent of the epilepsy, as children with mild Doose syndrome are more likely to benefit from treatment, although learning disabilities are still likely. Severe cases, such as those patients who have many seizures a day, may not benefit from treatment.

Boys are more likely to have the disease than girls. A possible cause of the syndrome may be genetic, as seizures can run in families. When a child has this syndrome, about a third of the time, another person in the family also has epilepsy. Doctors diagnose the presence of the syndrome through the distinctive symptoms and also through electrical monitoring of the patient’s brain patterns with an electroencephalogram (EEG) machine.




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