lennox gastaut syndrome - i need information

[Nat1H]

i posted a thread a few days ago about my DS2 (cp, epilepsy) who has started having 'unusual' absence seizures.
He has been having 2 - 4 absences a day, lasting betwenn 2 and 5 minutes each. One day he had a 'cluster' of 3 seizures, where he just about came round before slipping back into another.
He has been very 'drunk' looking for the past few days - not quite as alert as usual.
His absences have started with a head drop, his mouth makes an 'o' shape and his breathing is shallow. His right side starts to twitch. He has NEVER had siezures like this before, and I am worried that it may be Lennox Gastaut Syndrome.
We are going to see the neuro on Tuesday, but I wanted to see if anyone here had any ideas.
Am I over-reacting?
I have scared myself silly by reading stuff on the internet, the problem is, he seems to fit the criteria.
Cheers. Nat.

My son has epilepsy. A few months ago he had some of these drop seizures over 2-3 weeks. I read stuff on the net an was scared it could be lennox gastaut - because drop seizures can be a feature of lg. But my son does not have lg.

So try not to jump to this concusion. Yes it does need to be investiagted. But talk it over with your neuro and get their opinion.

HI Nat. My son was dx with Infanitle spasms at the age of 5 omnths and we were told that one day this may morph into LGS, it scares me shitless.

Needmorecoffee, has been through the worry of LGS with her dd recentyl so she may be able to help you out.

Agree to post for needmroecoffee- I will e-mail her a link to this, if that is OK?

I hear my name....
A neuro will generally want 3 criteria for LGS. Seizures that are a bugger to stop (including status seizures), signs of losing skills/mental regression and a slow spike wave pattern on an EEG. 2 out of 3 will get youa LGS dx although its not always correct.
DD had infantile spasms at 3 months then 3 years with only a couple of absences. This Jan she started myoclonics and complex partial seizures plus status seizures. An EEG (emergency my arse, they asked for it in Jan) showed the slow spike wave pattern of LGS in about a third of her brain, over the damaged regions. Her doc consulted with Prof Neville who is Mister LGS in the UK because the pattern wasn't everywhere (rare) and she wasn't having tonic seizures (nearly always present). We decided to treat with a 2 week course of ACTH to prevent it turning into true LGS. She was still learning well but looking back compared to how she is now, her vision was much much worse (the pattern was over the back of her brain and obviusly interfering with her sight)
2 weeks of ACTH (and hell) and the EEG is clear and we've seen no seizures since July. I have no idea if we've beaten it. I am still terrified cos dd only has her intellect. She has total body CP and can't even lift her head.
Nat, I don't know if this is LGS but you need an amergency EEG. And that means this week, not 6 months. If the slow spike wave pattern is seen he should go straight onto ACTH (I'm sure dd's neuro wont mind discussing it with your lad's doc. Its an experiemntal treatment. ACTH has always been given for infantile spasms but most neuro's with LGS tend to slog through 17 pointless drugs, none of which work for more than a few months. Using ACTH for LGS is pretty new).
One thing though, brain damage and some anti-epileptics can cause a slow spike wave just to be confusing. Epilim (sodiumvalproate/depakote) is the main contender so he shouldn't be on that.
One good thing I've been told, if you have had a seizure free gap between IS and LGS or suddenly develop LGS after no previous seizures (happens even to NT kids at 3-5 years) then the chances of beating it are higher and of not losing skills.
I'm still in the middle of this, wondering and waiting and its bloody awful. DD will have EEG's every 3 months now but when she had the EEG after the ACTH treatment and it was clear I burst into tears. you want the EEG but also you don't want it IYKWIM.
I feel like IS and LGS expert here but I've read every reserach paper on them. There are worse epilepsy syndromes but those two are right up there
Good luck with your neuro and BE PUSHY

Thanks needmorecoffee.
I am going to push BLOODY HARD on Tuesday and ask if it may be LGS. Unfortunately he has CP and is on epilim, so from what you have said, we may not get a proper reading from the EEG.
What does ACTH stand for?
You mention your DD's vision. What happened with that - how could you tell it was worse? DS2 keeps saying 'where are you' even when we are sat really close to him. I don't know if this is just something to say (he often repeats things he has learned) or if he actually can't see us. I haven't taken much notice of what his happening with his body/eyes when he says this, but now I have something else to worry about!! Bugger!!

You might need to remind your neuro that epilim can cause a slow spike-wave. You'd be amazed how many don't know this. There's some research out there. Epilim doesn't always cause slowing though, which complicates things but it can.
There's a thing here the answerer is a neurologist.
ACTH (syncathen) is a steroid that is given by injection. Its the first line med for infantile spasms and other intractable epilepsies. It doesn't have hideous side effects though. When dd was on it (at 3 months and again at 3 years) she screamed 24 hours a day, her BP went through the roof and needed medication and her electrolytes went haywire. Both times she had to remain in hospital. But when it works, it works and is worth it.
DD has cortical visual impairment that varies daily. When she was a baby she was blind because the electrical crap going on inher brain. we noticed this year her vision decreasing and the EEG showed the slow spike waves and polysynchronous spikes in her visual cortex. I imagine the signal from her eyes was getting lost in the static from the sub-clinical seizures.
Has your ds had his vision checked? Many kids with CP and brain injury have vision problems. It can be sorted with visual therapy (hollow laugh. We're still waiting). A friends son who is 11 and communicates with a letter board (severe CP) says when his seizures are bad he can't see.
If you get an EEG and it doesn't show the slow spike wave pattern, ask for a sleep EEG. Early LGS will showthere first which is why many status seizures and tonics happen at night. Does your lad have tonics? Look like stiffenings that last 20 seconds or more. Not conscious of surroundings usually.
Hope that all helps.

Thanks needmorecoffee. Am quite new to epilepsy (only diagnosed last November) and he hasn't had many seizures up until now, so am still learning what all the terms mean.
His seizures are as described above - more like long absences with some tremor in his right side. His head sometimes drops at the beginning of these.
We have been keeping our eye on him very closely this week, and are beginning to think that he is usually having these seizures at nursery. If he has one at home, they are nowhere near as severe as nursery are describing (touch wood).
We are beginning to wonder if there is some sort of stimulus at nursery that is causing these. Can overstimulation cause fits? Or flourescent lights?
Am going round and round in circles now - need to see Neuro!!

needmorecoffee I am in awe of how god damn knowledgeable you are!!!! Amazes me, but I guess you have had to mkae sure that you are on the ball in orsdetr to challenage of those docs....!!

I dont want to highjack Nat's thread but would you mind if I emailed you could do with your wise wisdom ( as if you havent got anything better to do than responds to other peoples queries!!) but if you dont mind that would be great

Could be the way the nursery describes them. DD's respite carer came sccreaming in one day saying dd was having seizures. Heart in mouth I ran in but it ws her normal twitches and jerks from her CP. DD jerks her head back with her reflux and with her CP.
Friend of mine's son has seizures like you describe. His head goes down and his right arm sort of shudders. Lasts about 10 seconds I'd say. He's on epilim and topomax which clearly are not working.
Over stimulation can cause fits. I know one lad who has one every time he sees his mum. But then so can under stimulation, phase of the moon, telling your child you're going away (dd does this one. If I let it known I'm going away she always has a status seizure the night before reqyiring hospital). All sorts of things!
In my previous post it should have read 'ACTH DOES cause hideous side effects' D'oh

Don't mind at all. Used to be a neuroscientist so I know how to read research papers. I don't know everything though, I just read a lot.
You got my email address Louro?

I dont think I have....?!

its [email protected]