Doose syndrome

by Zhang Dan on March 6, 2011

Doose syndrome, which is also named as myoclonic-astatic epilepsy(MAE), is a rare epilepsy syndrome of children.

94% patients onset before age of 5, with a peak of 3-4 year-old. Usually, they are normal children before onset.

Doose syndrome has multiple types of seizures.  Although myclonus and astatic attack are the main characteristics of Doose syndrome, 60% patients start with general tonic-clonic seizures with or without fever. Myoclonic jerks, astonic or myoclonic-astatic seizure could be seen in 100% patients. Besides, absence seizure and nonconvulsive status seizure could also exist.

Now, I wil describe the main characteristic, myoclonic-astatic seizure, for you. This usually started with brief myoclonic jerks with wide range of intensity, from facial muscle twitching to symmetrical arms and shoulders jerking. Astatic attacks immediately followed myoclonus, ranging from head drop to fall down to the ground.

There are special Electroencephalogram(EEG)  findings in Doose patients. Considering the complexity of EEG, I will skip this part to avoid giving you confusion.

Doose syndrome needs to be differentiated from Dravet syndrome, West syndrome, febrile convulsion, Lennox-Gastaut syndrome and so on. But differentiation would be quite difficult in some children. So, if your doctor changed the diagnosis as the diseas went by, it would not be strange at all.

As to antiepileptic drugs(AEDs), Valproic acid is the first choice. But other drugs can also be used according to types of seizures and response to initial treatment. Many researches show that ketogenic diet is even more effective than any kinds of AEDs.

The prognosis is generally good. 80-90% patients would have no or just mild cognitive impairment.

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