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Doose Syndrome (MAE)

Myoclonic-Astatic Epilepsy (MAE), or Doose Syndrome, is an epilepsy syndrome of early childhood that is often resistant to medication. For this reason, it can be difficult to treat. MAE is idiopathic generalized epilepsy, meaning that there is no known cause for the seizures (idiopathic) and the seizures originate from all over the brain (generalized) as opposed to coming from one focal area. Onset of MAE occurs commonly in the first five years of life, with the mean age being three.  Statistics show that it usually affects children who have previously developed normally, and boys are twice as likely as girls to develop MAE. In some cases, other family members (immediate or extended) may also have seizures.

As researchers learn more about MAE, new treatment options become available and the outcomes continue to improve for our children. Parents new to MAE can be encouraged knowing that many families and their doctors have found successful ways to treat this challenging disorder.

• Myoclonic-Astatic Epilepsy usually occurs in children with an uneventful history; there is likely to be no pre-existing neurological disorder.
• It clearly affects more boys than girls at a ratio of about 3:1.
• In 24% of the cases, the epilepsy starts during the first year of life (at 2, 4 or 5 months of age).
• In 94% of the cases, the epilepsy starts within the first five years of life.
• In 100% of cases, the child develops myoclonic and/or myoclonic-astatic (or drop) seizures. The -astatic (loss of muscle tone) feature of the myoclonic-astatic seizure is rare and unique to MAE, and is the most important and distinct feature that helps differentiate it from other syndromes.
• In addition to myoclonic seizures, children may also have a combination of other generalized seizures including tonic-clonic, absence and non-convulsive status epilepticus and, rarely, tonic seizures.
• Hallmark features on an EEG help define MAE and, in almost all cases, rhythmic partially accentuated 4-7 Hz background seizure activity develops early in the course.

* Doose Syndrome

* Myoclonic Astatic Epilepsy

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Dr. Roy Sucholeiki, MD