Re: just checking in...Tina/Leeanne-MAE

August 23, 2004

Sorry, not been checking mesages for a few days and I am in catch up mode, so

not sure of this is the first in this thread?

Anyways - Leeanne - LGS and MAE are very similar syndromes, similar age of

onset, similar seizure types, but with subtle differences on the EEG and often

in development etc.

TCs are usually (but not always) the first seizure type, then the rest of the

nasties creep in over time (varies with each child, can be days, can take

weeks/months), which include drop attacks - often by way of myoclonic astatic

drops (which the syndrome is named after) - also known as 'stare jerk and fall'

seizures, atonics, absences and myos.

LGS on the other hand often includes (as well as the above) atypical (rather

than typical) absences, focal, and tonic seizures. These ones can occur in Doose

as well, but rarely, ( started with tonic seizures last yr but his were

due to a pardoxical seizure response to benzodiazepine medications) and their

(unprovoked, ie - not med related) inclusion in the range of seizures usually

points more to LGS.

LGS also has a fairly definitive spike and wave on the EEG, MAE also has EEG

characteristics which help with diagnosis.

The correct diagnosis is important more from the treatment point of view, as

many of the AEDs that are often prescribed for LGS are big no no's for MAE -

they can and will excaerbate Doose seizures, mainly vigabatrin, phenytoin,

phenobarb and tegretol. Benzodiazepines are also making a name for themselves as

invoking (over time) tonic seizures in MAE kiddies, and lamictal has in quite a

few cases aggravated myoclonics (can happen in generalised primarily myoclonic

seizure disorders, which MAE is...)

MAE carries a variable prognosis, with permanent remission (sometimes

regardless of which treatment is used) a possibility in the more favorable

prognosis group. Most MAE children appear to respond very well to the keto diet,

which is why it is now becoming known as the first line treatment for this

disorder, even before traditional AEDs.

I am in contact with many MAE parents round the world, (a lot of whom also

participate in this keto webring) and the developmental progress and 'catch ups'

that these kids are capable of if the seizures can be stamped on, is really

quite remarkable.

It sounds like may have this diagnosis based on your seizure type

description, but v hard to know without more detail - perhaps asking your Drs

outright to review her EEGs, this time bearing Doose in mind...may see you

finally get an answer.

was misdagnosed with LGS at seizure onset, we asked for a 2nd opinion 5

mths later, (from reading screeds about LGS we felt he just didn't 'fit' this

one) and his diagnosis was subsequently changed to MAE. At that point he was

only the 2nd case diagnosed in NZ, I think the number is now 4, but I'm not 100%

sure. In other words - it is quite uncommon, but my money says there are many

more kiddies out there misdiagnosed, unfortunately it is often not until a ped

neuro/epileptoligist has actually had a case, that they become aware of the

subtle differences that can occur, hence a lot of children are not treated

correctly at the outset. The keto diet in my opinion is also the best treatment

for the usually meds intractable LGS, so either way the diagnosis is not that

important in that respect, but the meds issue is a biggie. We could have saved

and ourselves (!) a lot of torment had we known earlier how these kids

tend to respond to AEDs - more often than not, adversely

Let me know if you need further info or advice, not getting here a heck of a

lot lately, but will try and keep up to date with messages

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

From: FitzGeralds

Leeanne

hill just educated me on Doose syndrome, I did not really know of

it before that, so maybe she will pipe in on this syndrome. She told me

it had a usual onset btw 2-4 years old, and the best first treatment is

the keto diet, as many antiepileptic drugs exacerbate the problem. I

think that may be why Ethan did so well after we started the diet 5

weeks after the seizures started.! That's all know. Sorry -Tina

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

From: ardwickpat@...

Sent: Sunday, August 22, 2004 9:33 PM

Hi,tina,

just read your email and im probably going to sound really stupid now

but

what is MAE/doose syndrome and what is LGS.My daughter is 12 and

has just

started the keto diet on the 10th August this year,she has tonic

clonic,myclonic and absence epilepsy.Her eeg reports have told us that

her eegs are

remarkably abnormal??? and nobody can tell us why she is this way,she

also has

learning disabilities and language delay.

hope to hear from you soon,Leeanne,mum to aged 12.

August 24, 2004

Hi ,

Thanks for your reply, is 12 now and 1st started with epilepsy when

she was about 2 years five months.it started off the odd jerky movement here and

there,nothing to major.before starting on this diet she had around 400-750

large jerks aday.some other things that she has are these kind of drop

fits,that dont seem to last very long,she will first,stare into space,jerk

alittle

then drop to the floor and then pick herself up again all within say 10-15

seconds.if she is holding anything it will fly out of her hands and many a time

she has had her breakfast over her.Do you or does anyone on this sight know

why these children are the way they are?Are they born either with the

epilepsy,doose or mae sydromes or is there some reason that she is so

poorly.She is

such a beautiful child who looks like an angel,but she also has these bad???

behaviours,where she will just lose it and goes mad.Headbutting,kicking,biting

and punching us and this can go on for hours.the doctors have told us that

this is to do with her epilepsy and that we will just have to live with

it.Since starting on the diet(10th august)she is alot better although its still

early days and still lots of drops.i will mention to the consultant about

doose,and mae syndrome and see if he knows much about them,when she last had an

eeg

they told us that it was " remarkardlby abnormal " whatever that is supposed to

mean?I love her to bits but it is so hard watching her when she

fitting.Anyway will go for now and hope to hear from you soon ,take care and

god

bless,Leeanne and

August 24, 2004

-

I think you may have something there with Ethan. It is interesting, we

tried Trileptal at first with him and that is when all the drop attacks

and head drops started, before that he only had the tonic clonic

variety! I feel like this has cleared up a lot of the mystery behind

his " seizure disorder " diagnosis. I am so happy we listened to our

inner voice that told us to go with the diet so early, even though it

was not the typical route after only 5 weeks of seizures. Thank you for

your info, and if anything starts up again (bite my tongue) I will

certainly have that checked out. Did you say there was a similar list

for Doose on the onelist? XOX-Tina

Re: just checking in...Tina/Leeanne-MAE

Sorry, not been checking mesages for a few days and I am in catch up

mode, so not sure of this is the first in this thread?

Anyways - Leeanne - LGS and MAE are very similar syndromes, similar

age of onset, similar seizure types, but with subtle differences on the

EEG and often in development etc.

TCs are usually (but not always) the first seizure type, then the rest

of the nasties creep in over time (varies with each child, can be days,

can take weeks/months), which include drop attacks - often by way of

myoclonic astatic drops (which the syndrome is named after) - also known

as 'stare jerk and fall' seizures, atonics, absences and myos.

LGS on the other hand often includes (as well as the above) atypical

(rather than typical) absences, focal, and tonic seizures. These ones

can occur in Doose as well, but rarely, ( started with tonic

seizures last yr but his were due to a pardoxical seizure response to

benzodiazepine medications) and their (unprovoked, ie - not med related)

inclusion in the range of seizures usually points more to LGS.

LGS also has a fairly definitive spike and wave on the EEG, MAE also

has EEG characteristics which help with diagnosis.

The correct diagnosis is important more from the treatment point of

view, as many of the AEDs that are often prescribed for LGS are big no

no's for MAE - they can and will excaerbate Doose seizures, mainly

vigabatrin, phenytoin, phenobarb and tegretol. Benzodiazepines are also

making a name for themselves as invoking (over time) tonic seizures in

MAE kiddies, and lamictal has in quite a few cases aggravated myoclonics

(can happen in generalised primarily myoclonic seizure disorders, which

MAE is...)

MAE carries a variable prognosis, with permanent remission (sometimes

regardless of which treatment is used) a possibility in the more

favorable prognosis group. Most MAE children appear to respond very well

to the keto diet, which is why it is now becoming known as the first

line treatment for this disorder, even before traditional AEDs.

I am in contact with many MAE parents round the world, (a lot of whom

also participate in this keto webring) and the developmental progress

and 'catch ups' that these kids are capable of if the seizures can be

stamped on, is really quite remarkable.

It sounds like may have this diagnosis based on your seizure

type description, but v hard to know without more detail - perhaps

asking your Drs outright to review her EEGs, this time bearing Doose in

mind...may see you finally get an answer.

was misdagnosed with LGS at seizure onset, we asked for a 2nd

opinion 5 mths later, (from reading screeds about LGS we felt he just

didn't 'fit' this one) and his diagnosis was subsequently changed to

MAE. At that point he was only the 2nd case diagnosed in NZ, I think the

number is now 4, but I'm not 100% sure. In other words - it is quite

uncommon, but my money says there are many more kiddies out there

misdiagnosed, unfortunately it is often not until a ped

neuro/epileptoligist has actually had a case, that they become aware of

the subtle differences that can occur, hence a lot of children are not

treated correctly at the outset. The keto diet in my opinion is also the

best treatment for the usually meds intractable LGS, so either way the

diagnosis is not that important in that respect, but the meds issue is a

biggie. We could have saved and ourselves (!) a lot of torment

had we known earlier how these kids tend to respond to AEDs - more often

than not, adversely

Let me know if you need further info or advice, not getting here a

heck of a lot lately, but will try and keep up to date with messages

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

From: FitzGeralds

Leeanne

hill just educated me on Doose syndrome, I did not really know

of

it before that, so maybe she will pipe in on this syndrome. She

told me

it had a usual onset btw 2-4 years old, and the best first treatment

is

the keto diet, as many antiepileptic drugs exacerbate the problem.

I

think that may be why Ethan did so well after we started the diet 5

weeks after the seizures started.! That's all know. Sorry -Tina

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

From: ardwickpat@...

Sent: Sunday, August 22, 2004 9:33 PM

Hi,tina,

just read your email and im probably going to sound really stupid

now

but

what is MAE/doose syndrome and what is LGS.My daughter is 12

and

has just

started the keto diet on the 10th August this year,she has tonic

clonic,myclonic and absence epilepsy.Her eeg reports have told us

that

her eegs are

remarkably abnormal??? and nobody can tell us why she is this

way,she

also has

learning disabilities and language delay.

hope to hear from you soon,Leeanne,mum to aged 12.

August 24, 2004