Head-drops, nonstop, all day long

[Marcus999]

Hi All

After five years (no diagnosis yet, although JME seems the closest) my 15-year-old daughter's seizures have 'stabilized' for the moment to (primarily) Atonic "head-drops". The thing is she has them every 10 to 30 seconds all day long. They last from three to ten seconds. Becomes basically symptom-free while she sleeps. Has anyone ever come across this? We can find no history, anecdotes, or medical literature about this kind of frequency.

She started out at 10 with drop attacks, then we saw eye-rolling, then head drops. She's been in status twice, hospitalized numerous times. Meds all seem to stop working. MCT diet worked for a year, then stopped (despite being 3+ all the time). She has no cognitive deterioration, genetic tests all negative, Bone marrow tests, MRI's and CT's all negative.

Currently on Valproic Acid, Keppra, Clonazepam, and weaning off Clobazam. Many micronutrients to treat anxiety and nervous system + Vitamins.

Any help or referrals to similar cases of intractable head-drop much appreciated.

 

[Fedup]

Marcus999

I am not a parent, nor a doctor. The thing is most epileptics eyes roll during a seizure, my head drops, that is if I am sitting in a chair my chin will hit my chest at the start of a seizure. I realy think you are looking for too many answers. This has to be scary but take a second. You might want to talk to your doctors again and explain what is happening and how concerned and scared you are.

 

[Marcus999]

Hi Fedup

Thanks for the reply. I'm sorry, I don't understand what you mean by "looking for too many answers". When you have a sec could you explain please?

Our Neuro is up to date with her condition... It's the frequency that mystifies us (and her Neuros). We're counting up to 2,000 of these a day.

 

[Cint]

The thing is most epileptics eyes roll during a seizure, my head drops, that is if I am sitting in a chair my chin will hit my chest at the start of a seizure. I realy think you are looking for too many answers.

Not really. I've had many SP and CP seizures and my eyes don't always roll back, nor does my head drop. I just stare ahead, as if in a dream-like state, unable to comprehend what is being said. Everyone's seizures are a bit different, so to say Marcus is looking for too many answers, is not quite fair, especially when it involves your own child. And many parents have children whose seizures go on and on and on....... without answers from docs.

 

[Fedup]

Marcus999

From what you wrote, you seem to be at your wits end and excepted without diagnosis that its epilepsy, its the closest. Now her head drops and then you noticed the eyes rolling, she has them ever 10 - 30 seconds all day long. You could be right, it could be juvenile myoclonic epilepsy, it is possiable for her at her age to grow out of it but this can only be a hope. If its epilepsy the first thing you did was to get control of the seizures, now the head droping and the eye rolling. They may not be individual but part of one thing, that is seizure. Your next quistion is are they realy under control, you do not know. You need to write down everything in a diary or notebook, when it starts and stops, time it starts and stops, what foods you think might be effecting it and so on. So insteed of answers for each indidual thing maybe its just part of one and so one one answer is needed. This is not something that is easy and I am not making light of what you say, I am just saying what I see in what you wrote and what I think might help you.

 

[Marcus999]

Hi Again

Thanks Cint. Still not sure what 'too many answers' refers to. Thing is, the eye-rolling stopped many years ago. Her episodes have been described to us as Myoclonic Absences. She is 'out' for the duration of the drop, eyes usually open but lidded. Like I said, it's the maddening frequency that's so... well, maddening. We've managed to cut out T-C's (maybe just for now - who knows) and drastically reduced the myoclonic jerking to a subtle twitching. If we could find an answer to the huge number of Myo-absences she'd be almost back to being "our girl" again. She still can't walk unaided and her motor control is still sketchy but if we could get the drops dealt with she'd have a pretty good quality of life. Hence our frustration. Maddening...

and thanks again for the attention to both of you.

 

[Marcus999]

Hi Fedup

Oh, I see what you mean now about too many answers!

"So insteed of answers for each indidual thing maybe its just part of one and so one one answer is needed."

Yes, (by the way - she definitely has epilepsy, we know that. It's just the exact type that eludes diagnosis) we started out her treatment by trying "one thing". That really didn't work in her case. After four years it became evident that we're dealing with multiple seizure types - maybe related to each other, maybe not. We're now having the most luck in treating each type individually with targeted medications.

It's an ongoing battle. And, yes, maybe she'll grow out of it. We're reasonable sure that once puberty is passed things will be a little tamer - who knows? We know it's just a hope.

Respectfully yours.

 

[Cint]

IMO, a caring parent is never looking for too many answers, especially when you've already observed T/C's and that jerkiness of a myoclonic.

Here's some info on the subject from the pros:
http://professionals.epilepsy.com/page/epilepsy_juvenilemyoclonic.html

 

[Cint]

So insteed of answers for each indidual thing maybe its just part of one and so one one answer is needed. This is not something that is easy and I am not making light of what you say, I am just saying what I see in what you wrote and what I think might help you.

There are more than one answers, tho, especially if one has several problems. I have several types of E, therefore I have lotssss of unanswered questions to this day. And I've had E for over 30 years!

 

[Nakamova]

Hi Marcus --

It must be tough when it happens so often, but have you been able to discern any secondary triggers for the head drops? Are they worse at different times of year or day? How does she do at night?

I think Valproic Acid and Keppra are considered the drugs of choice for Drop Seizures, so maybe a slight increase in the doses would help. A newer drug, Banzel, is suppose to help with them as well, so that might be worth a go: http://www.banzel.com/seizure-control.aspx Cataplexy, which manifests similarly to head drops, is usually treated with SSRIs, so that might be another option to discuss with the docs.

Hope you and she get some relief soon.

 

[Marcus999]

Hi Nakamova

Thanks for the reply.

We've looked at all the secondary triggers we can think of... Drops are constant and unremitting all day long. Treatments/drugs seem to work for a while and then cease to work. MCT oil diet included. We're hoping that as she gets older and she's done puberty that we may have more luck. It's as if her metabolism just won't stand for being 'handled' for long. It's like a switch turns her off every 10-20 seconds.

She's not had a nighttime EEG but all discernable seizure activity stops when she sleeps - it seems to be activated by the mere activity of conscious thought. As soon as she wakes it all starts again.

We've also tried Banzel, it was very much a no-go. It just increased the frequency and the duration significantly. We'd had high hopes for it.

Much appreciate the thoughts!

 

[Marcus999]

Diagnosis - 5 years on.

Hi to all who were kind enough to reply to my inquiries.

I wanted to let you all know that we got a diagnosis a few days ago:
Spinal Muscular Atrophy - Progressive Myoclonic Epilepsy (SMA-PME).

This is a very very rare form of SMA which manifests as (mostly) epilepsy. No wonder it was so hard to track down. It's not good news, obviously, but it's better to have a diagnosis than not.

We have yet to learn the details of how this genetic disease will play out in our daughter.

Good luck to you all.

 

[Nakamova]

Marcus, thanks for the update. I wish the best of luck to you and your daughter.