To those with craniocervical instability

[eaudire]

I'm curious about the connection between craniocervical instability and POTS in relation to my own symptoms, and wondered if those with the condition could answer a few questions.

1. What are your most prominent symptoms and which did you notice first? (i.e., did you start having terrible head and neck aches, or did you notice heartbeat irregularities first?)

2. Which tests confirmed the diagnosis? (i.e., an MRI/X-rays/other imaging, and were they done supine or upright? Did they include both the brain and spine?) 

3. Did they determine the cause of the instability? 

Many thanks for any and all replies! Trying not to have too many unnecessary doctor visits and be as strategic as possible about the ones that I do have.

[DizzyGirls]

My daughter has all-out cervical instability, not just craniocervical.  Her biggest symptom and probably the earliest was her vertigo.  They think it is due to the spinal cord being pulled due to the way her head sits.  Also, horrible headaches, migraines, neck pain.  All right there together.  Also was diagnosed with pseudotumor cerebri with blurred optic disks and optic nerve inflammation.  (How's your vision?)  POTS stuff came later.

The gold standard is a flexion/extension MRI of the cervical spine.  My daughter's neck has a kyphotic curve instead of the normal lordotic so they didn't feel comfortable doing the flex/ext MRI on her.  When her head flexes too far forward, it impinges her corotid arteries and she passes out.  They didn't want that happening under anesthesia. 

The cause of her instability if muscle weakness due to ligament laxity and the instigator in all that is Ehlers Danlos Syndrome.

I totally understand the information gathering before hand.  A very smart thing to do!  I'm happy to answer any more questions you might have.  We've been dealing with this for a while and her surgery is in a couple of weeks.  She will have C3-T3 fused with the possibility of O-C1 and C2 also if they don't get the stability that they need.  It's a two neurosurgeon deal and roughly 8 hours long!  Going to be a grueling day and following days for her.

Best wishes to you! 

[bombsh3ll]

On 7/19/2017 at 8:00 PM, DizzyGirls said:

She will have C3-T3 fused with the possibility of O-C1 and C2 also if they don't get the stability that they need.  It's a two neurosurgeon deal and roughly 8 hours long!  Going to be a grueling day and following days for her.

Hi do you mind me asking how this procedure went and what improvements your daughter has gained from it? I was recently diagnosed with hypermobile EDS and paid for upright scans (I am in the UK). They were reported as normal but apparently craniocervical instability can be missed unless a neurosurgeon trained in this field looks at the films. I am currently in the process of getting an opinion from Dr Gilete in Barcelona. I would love to be able to find a correctable cause of my severe POTS & don't believe it is down to lax veins as my onset was very sudden after a forceful valsalva, whereas veins would surely tend to stretch out over time. I am sure something shifted or became squeezed in my brain. I get a lot of headaches and never had any prior to POTS onset. I hope your daughter is doing better after her surgery, it must be especially difficult dealing with these problems at such a young age.

[DizzyGirls]

It's been 6 months since her surgery.  We were told ahead of time that it would be a "monster" surgery with a "brutal" recovery.  It has not disappointed.  She ended up having C3-T4 fused.  They went in through the front (anterior) to reconstruct the curve of her neck and fused C3-T1, they flipped her over (said it took an hour just to do that!) and went in through the back of her neck (posterior) and fused C3-T4.  Bone grafts, titanium rods, plates, screws.  She did have two neurosurgeons and their prospective teams as well as a hand picked anesthesia team because of her POTS.  You should make sure they are really up to speed on POTS.  She was in the hospital for 10 days and then acute rehab for another 7.  It's been rough.  She did well for a while.  But, the last several months things have been getting worse again.  A lot of muscle spasms.  The knots at the base of her skull are causing migraines and really intense skull cracking headaches, and her muscles on the upper back and shoulders are really giving her fits.  She had in-home PT for about 8 weeks after and did really well, then the PT got transferred and he said that there wasn't really much else for her to do at home.  She needed to go to an outpatient PT.  The problem was, one of her neurosurgeons was being really conservative in getting her out to PT and waited too long.  Her muscles have all tightened up and are like ropes.  Her upper back is very heavy now and solid from being fused.  Takes a lot of strength to carry it.  The pain from this is beyond what we ever expected at this point.  We did find out from a spine site that after 3 months, there can be an upswing in pain.  That was definitely the case for her.  So, with PT, without PT, whose to know if it would have still ended up this way.  Her younger sister has EDS, dysautonomia, and was just diagnosed with dystonia.  We are thinking the older one has it as well.  Hers follows the description of people with EDS and dystonia.  Just read a really interesting paper on it.  Anyway, I hope I didn't scare you.  I just want you to go into it with all the cards on the table.  Her POTS has not changed, however, from the surgery.  Many of the neurological symptoms did, but some are starting to creep back.  I don't want you to think your POTS is going to magically get better with this surgery.  It will most likely get worse for a while because of the stress on your body.  One of my daughter's neurosurgeons is from peds and he is especially aware of POTS, so he kept hers under control really well after the surgery.  More than I can say for the PTs, but that's another story......  Good luck to you and if you have any more questions, please don't hesitate to ask!

[bombsh3ll]

Thank you so much for replying with so much detail. I am sorry your daughter is still struggling. I appreciate your honesty regarding the POTS - it is my only reason for considering this surgery, IF the neurosurgeon feels I have something anatomically correctable by surgery. Other than permanent lightheadedness, presyncope and inability to be upright, I have no neurological symptoms and believe my head and neck pain to be related to hypoperfusion. 

Hearing this, I think I would want to try a halo vest brace first if possible, to see if my condition improved with the pressure off the brainstem, and only go for the surgery if it helped. 

[Edsia]

Hi!  Did your daughter's pseudotumor cerebri  improve after the surgery? 

 do the doctors think the pseudotumor cerebri  was caused by the cervical instability?

 I suffer with pseudotumor cerebri as well and I'm wondering if it's connected to the Craniocervical instability. 

I also have ehlers-danlos syndrome,severe M.E and POTS and I'm bedridden. Unfortunately I live in a country where there's no knowledge or treatment options available for these conditions.

Wishing your daughter a speedy recovery!

 

[DizzyGirls]

Hi Edsia!  Welcome to Dinet!!  Did her pseudotumor cerebri improve after surgery?  Yes and no.  She had to have a lumbar puncture a few months after surgery, but her ped neurosurgeon said it's not uncommon to have that happen after a cervical surgery like my daughter's.  So now a few months later, she doesn't seem to have that problem anymore.  Different problems have surfaced, just not that one.  We are dealing with myofascial pain as well as severe muscle spasms.  Also trying to rehabilitate muscles that have not been used correctly over the past couple of years.  I'm sorry you are so ill.  It's got to be incredibly difficult to live where there are no options.  I get really frustrated at doctors here in the US who aren't familiar with EDS, POTS, Dysautonomia, craniocervical instability because I think after all the schooling they've been through to get to their specialty, they ought to know this stuff.  But most don't.  We had wanted to see Dr. Frasier Henderson for her spinal fusion surgery because he is the best of the best, but he was so far away.  We found a pediatric neurosurgeon at Lucille Packard Children's Hospital at Stanford University (he's actually the Chief of Neurosurgery) and I would not trade him for the world!!!  He grabbed a colleague in the Adult Spine Department (heard from some of the interns that he is world renowned for cervical reconstruction), and, with their collective teams, put my daughter back together.  I hope that you might be able to find someone soon.  There has to be somebody that researches the latest and greatest medical challenges.  I wish you all the best!!