Re: Re: possible Doose??Kathy

[Guest guest]

Doose is the name often used, as the syndrome was identified by a Dr

Herman Doose, but the actual diagnosis is MAE - or Myoclonic Astatic

Epilepsy of Early Childhood.

V similar to Lennox Gastaut Syndrome in many aspects, kinda like a

'cousin' of it I guess you could say. There is a fairly definite EEG

pattern, and this is what helps differentiate the syndrome from LGS - which

usually has a 1.5 - 2.5 hz spike and wave.

There is often a family history of epilepsy somewhere along the line (for

instance my first cousin had epilepsy, this apparently counts as a close

enough genetic link), and many children had febrile seizures as infants

before going on to develop Doose. ( wasn't one though - never saw a

seizure in my life before his first at age 3 and a half.)

LGS can be symptomatic to an underlying disorder, whereas Doose is always

cryptogenic in origin (no known or identifiable cause) and all Doose

children have no existing cognitive deficits at the time of the first

seizure. Develoment and prognosis is variable, some children go on to

function perfectly ok, some have a poorer cognitive outcome, usually those

whose seizures remained uncontrolled for a long period.

Tonics are usually v common, or even one of the predominant seizure types

in LGS, whereas in Doose they are not as common. Myoclonics are one of the

main Doose seizure types, along with astatic (drop) seizures, either in the

form of atonic, or as myoclonic astatic drops, also known as 'stare jerk and

fall'. Absences and tonic clonics also feature, and complex partials, but

these focal seizures are quite rare.

Minor motor status is a feature of Doose - ie - like non convulsive

status, but with subtle twitching of extremities and sometimes round the

mouth and eyes, often interspersed with full-on convulsive myoclonic jerks.

So it is one of the more 'catastrophic' epilepsies, but if one's kiddie

was gonna develop a nasty one, I guess you could say Doose is one of the

better ones to get, as it can have a fairly benign outcome, percentages

quoted vary between papers, but on average about 50% of children 'grow out'

of the disorder, (another difference to LGS) mean time after about 3 yrs.

Age of onset is the same as LGS, usually age 2-5, some a bit earlier, some a

bit later. Siblings are at risk of developing epilepsy as well, again, diff

figures quoted in diff papers, some say a 10% possibility some say as high

as 40-50%. If a sibling (male dominance approx 2:1) does develop seizures,

it isn't always as Doose though, it could be say JME or absence epilepsy

etc.

There are some AEDs documented as definitely aggravating or worsening

Doose seizures - namely tegretol, phenytoin, phenobarb and vigabatrin.

Latest studies actually quote the ketogenic diet as the first line

treatment, with the most successful outcome for seizure control, ACTH as a

close second, and traditonal AEDs coming in as third.

Think that's about it, feel free to holler if anyone wants any more info,

----- Original Message -----

> Hi Kathy,

> I'm kind of ignorant about Doose right now, too. From what I can

> gather, it is a syndrome involving some myoclonic epilepsies.

> Children can varying degrees of severity. It is supposed to be rare,

> and difficult to treat. Some of the others may be able to help us

> out on understanding it better.