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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{ 10 comments… read them below or add one }

Kathleen Deviaene June 27, 2011 at 3:10 am

Can you please share your email with me, I’d like to ask you a question for a 12 year old boy with Lennox-Doose . thanks. Kathleen,


ricardo matucci July 20, 2011 at 1:22 am

Hello, my name is Ricardo and I from Sao Paulo, Brazil. My daughter has benn diagnosed with Doose Syndrome about 10 months ago (she’s now 3 and half years old). We’ve been tryng different medicantions (valproic acid, lamotrigine, clobazam, keppra), but so far we havent’n reached total control of the seizures. It’s true, though, we are in our best period (my daughter has “only” 1 or 2 mioclonic attacks per day – she’s had more than 10 per day). We currently using DPK + lamotrigine + clobazam… me and my wife fell we have reached a good improvement, but it’s so hard to win this beast. Is there any other medication we should consider, or ketogenic diet is the last step? Tks!


Dawn October 18, 2011 at 2:27 am


Find the yahoo group for dose syndrome. Excellent group. Ketogenic diet is our miracle!


K Frank October 18, 2011 at 1:20 pm

Ricardo, I am originally from Peru and one of my son’s was diagnosed with Doose at age 3. He is now 6 and a half and seizure free. We were on the Modified Atkins Diet (similar to Keto but easier) for over 2 and a half years, almost all of that time with no medicines (we weaned the medicines after 2 or 3 months of no seizure activity). In my opinion Keto or Modified Atkins should be one of the first treatments for Doose, not a last resource. Join the yahoo groups atkins4seizures and doosesyndrome if you haven’t yet, there are tons of good information there. Good luck!


Jessie Day November 1, 2011 at 2:45 am

My son was diagnosed with MAE aka Doose Syndrome last year. We were in the hospital countless times running EEG’s and experimenting with various treatments. At his worst he was having 25-30 drop attacks and several tonic-clonic episodes as well as a sprinkle of absence seizures. The disease is extremely difficult to diagnose and treat, especially since every child is different.
We live in Utah and had him at Primary Children’s Hospital the vast majority of the times we had him in. Towards the end, they were basically stumped and had tried every medication through to steroids. Finally, we had him transported down to The Children’s Hospital in Denver, CO. They have 14 epileptologists and an awesome support network. Ultimately, he ended up on Felbatol,Keppra, Clonopin, Clobazam, Zonegran, and Ativan as well as Diastat for rescue. He is well controlled. No problems at all until his last seize at the beginning of Oct 2011.
I don’t know if that is helpful, but perhaps it is nice to know you aren’t alone in the battle.


Angela March 22, 2012 at 12:42 pm

My daughter has been diagnosed with Infantile Spasms. We have tried Keppra, Clonazapam, Tomapax, ACTH injections, and the Ketogenic Diet. We are in the process of discovering whether or not her diagnosis is correct. They are now thinking Dravet’s Syndrome although she is still not diagnosed with this. She started having seizures at ten months of age. In the beginning, it was tough to get a diagnosis because her seizures were sporadic and she did not have them very often. By very often, I mean no more than once a week. We had a hard time catching it on the EEG and her MRI looked normal. We finally caught it under 24 hour observation after three days. Since then, she has had a peak of 96 seizures in multiple clusters ranging from one to 50. When we initiated the Ketogenic diet there was little improvement, but as we increased the ratio of the diet, we have gained seizure control of Infantile Spasms, we have just started to see a milder different form of seizure. My daughter is now 27 months old and has just started to make developmental progress. Because of so many seizures a day in the beginning, she was either eating, seizing, or sleeping all day, every day. There was not a time that she was playing or awake long enough to learn to do anything. The seizures just made her so exhausted. Although she is not completely seizure free, the Ketogenic diet (in all of the pain in the rear measuring and pouring, and fighting) has been a miracle for us. She is now holding her head up well, sitting unassisted for a minute and a half, getting up on her knees and rocking preparing for crawling, and saying “mama.” It may not sound like much for a two-year-old, but she has come so far! She was comatose and now I have my baby back, learning and progressing, and smiling again. She was normal with slowed physical development until her seizures started. Her physical development was a sign of something wrong in the beginning, so there was no surprise when she started having seizures. We were already forewarned that she was not currently having seizures but would most likely begin to have them at some time due to the nature of her physical limitations before she was ten months old. She is almost back to where she was over a year ago and we could not feel more blessed!


Chad H August 28, 2012 at 9:24 am

William had his 2 year old check-up in June and he was perfect. Then a month later, July 14, he had his first tonic-clonic seizure. We didn’t even know it was really a seizure. We thought maybe he had been choking on something. About 10 days later he had another and we realized our prayer of this being a onetime event was not going to happen. We quickly started using Children’s Hospital in Denver. They tried different drugs that appeared to help for a few weeks. Then the seizure’s increase in frequency again, increase the dosage, temporarily reduce seizures, then the seizures increase again. On bad days he was up to 30+ drop seizures. Development had stopped. Our faith kept us going. We prayed even more. Their next step was a 48 hour long EEG and they diagnosed him with Doose Syndrome. They suggested another drug, but my wife had had already found the yahoo Doose support group and heard so much about the Ketogenic diet. I’m so proud of her, she said no more drugs until we try the diet. Our prayers were answered. Within 48 hours of him being on the diet his seizures stopped. He was reduced from two down to one medication. He’s basically seizure free. He sometimes still has seizures when he has a fever. Try the Ketogenic Diet, TRY THE DIET, TRY THE DIET! The diet is hard for the whole house, but our children are worth the effort and challenge of the diet. Be faithful to your god and your prayer. If you are anywhere near Colorado, use the amazing doctors at Children’s Hospital in Denver.


Sherry Harding July 12, 2013 at 4:27 am

Hi! My name is Sherry & my son Gavin started having MAE/Doose syndrome @ 16 months. It started with an up gaze focal seizure with progression to drop seizures. We took Gavin to CHOP hospital & they ended up doing a 24hr EEG. Gavin had about 200 to 500 seizures a day! Well Gavin was on a cocktail of seizure meds 4 of the top typical ones they did nothing for Gavin’s seizures! He was zombie like & miserable! Then they suggested the ketogenic diet, I thought that’s not gonna work because Gavin loves vegetables & disliked meat @ that time. I decided I needed a 2nd opinion, I took Gavin to DuPont neurology to see Dr. Bodak in Delaware. After an hour & a half if sitting with my husband & I discussing Gavin’s history she said, how about we try some vitamin B6? I was thinking to myself, is this doctor off her rocker? After all these big seizure meds & she wants me to try a freakin vitamin? Well, 2 weeks later my Gavin’s seizures went from 200-500 a day to none! We then weaned Gavin off all the other seizure meds with the exception of Depakote. He’s had seizure free EEGs for a year now! I wanted to share Gavin’s story because I want the neurologists out there to at least think of trying something less traumatizing then mixing up cocktails of heavy meds! I hope Gavin’s story helps some family out there because it was so scary going through this!


Jennifer September 6, 2013 at 4:03 pm

I just want to share a story of hope… My daughter went through this when she was about 3/4 and was weaned from Depekote at about 6/7 (1st grade). She has not had a seizure since and is now 17!
Praise to God! I know the terror, worry and emotions of Doose Syndrome….Keep praying! God is good and my understanding is that there is a high rate that the children will outgrow it!


Jennifer September 6, 2013 at 4:05 pm

I also have a question….I have heard both answers…yes and no….Is Doose Syndrome hereditary?


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