Myoclonic-Astatic Epilepsy (MAE) was first described and identified in the late 1960s by Herman Doose as an epilepsy syndrome, hence its original label, Doose Syndrome. MAE is an epilepsy syndrome of early childhood that is often resistant to medication and for this reason it is typically difficult to treat. It is usually characterized by difficult-to-control generalized seizures, and is idiopathic (no known cause) in nature. The generalized seizure types seen in this syndrome vary, but many of the afflicted children can experience large numbers of seizures daily, part of what makes this condition so difficult to manage. Onset generally occurs between ages one and five, usually in children with an uneventful history. In some cases, there is a positive family history of seizures, and family studies over the years have supported a genetic basis.
As with most medical disorders, the spectrum of severity seen in MAE ranges from mild to those more severely affected. Children mildly affected by MAE may have their seizures quickly and easily controlled with first-line medications, alone or in combinations. Those children on the more severe end of the MAE spectrum may have difficulty finding an effective medication or treatment. As more is learned about MAE and new treatment options emerge, the outcomes continue to improve for our children. ¨