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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.
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