Possible Ehlers-Danlos Syndrome with craniocervical instability

<p>Cervical instability</p><p>I had Arnold Chairi type II decompression with c1 removal and dura placed 20 years ago. I did well for the most part. Mild headaches thereafter with se tingling in my arm sometimes over the years. Until 4 years ago. I stepped away from bedside nursing due to pressure headaches and tingling in my right arm, right should pain and clicking, tight neck and shoulder muscles and pain. Speed up to 4 mths ago. Constant every second of every day pressure and pain in my head and neck, dizziness, nausea, tingling in face and lips, coughing on food and drink, ringing in ears, fullness in ears and pain in right ear, difficulty concentrating, pressure and pain in head when I use my arms, used to be over 3 lbs now with 1 lb. Can't bend forward or look down or pressure is worse. I wear the cervical collar and this gives relief. Saw 2 neurosurgeons who said pain management and neurologist who said migraines who gave migraine meds which don't work<br />The one admitted me and did LP for hydrocephalus ind increased ICP which was negative and pumped me ful of migraine protocol for 48 hrs but gave no relief. Saw 3 rd neurosurgeon who said cervical instability. Is sending me for upright MRI flexion and extension. Said to wear cervical collar and will likely need cervical fusion. So I looked this up. Looks like I have vestibular nerve damage. I need to wear this collar. I am in IL. Put in request for 2 more neurosurgeons in Chicago to get surgical opionions. Has anyone had surgery for cervical instability?</p>

@jacklynn12 Welcome to Mayo Clinic Connect, a place to give and get support.

Your symptoms sound unbearable. I'm sorry you haven't found relief. Surely you would like to connect with members that can give you information, suggestions, and support as soon as possible. Due to this I added your question to a previous discussion on this topic. You may wish to scroll through the previous posts. Members like @chefgreg45 @wings89 @zebraspoonie @gldnrtrvrlvr @jenniferhunter @wittmack have posted to this thread and may be able to offer guidance.

This sounds like a serious surgery. I think it's smart to get more than one opinion on your surgical options. May I ask if any of these surgeons specialize in this sort of procedure?

@jacklynn12 From Chicago, it's a 5 hour drive to Mayo Rochester. I am in the Chicago area too, and I couldn't find a surgeon to help me, and going to Mayo made all the difference in the world. For me, seeing a Chicago surgeon just drug things out every time he wanted a test, I waited weeks for that and more weeks for a followup appointment. After 6 months of jumping through hoops, he decided not to offer surgery to me. I waited a month to get into Mayo, and they got all testing done in 3 days. I had an answer and an offer for help right away and just waited a few weeks to get on the surgery schedule. There may be longer waits with the pandemic, but it doesn't hurt to ask. Why do you think you have vestibular nerve damage?

I also have thoracic outlet syndrome which does cause arm and neck pain and lots of chest tightness. It affects my jaw and causes my cervical vertebrae to independently rotate, although that is much improved since I had a cervical fusion at C5/C6 and the muscle spasms have calmed down. I had similar issues with nausea and vertigo and I do have a slight bit of instability in my C spine. That was 2mm at C5/C6 which didn't prevent me from getting an artificial disc if I wanted that, but I wasn't a great candidate for that and had the fusion instead. Mayo was great place to go because they also evaluated the TOS to figure out if that or my spine issue was the major player in my symptoms. I had great results from my spine surgery and my neurosurgeon, Jeremy Fogelson is also trained in orthopedic spine, so he brings the best of both worlds.

If that is possible for you, you may want to get another opinion at Mayo since you have had the prior surgery, and with nursing work, it is possible that you could have TOS from repetitive tasks and raising your arms. This posture escalates TOS. IN Chicago, the places that treat TOS are Northwestern, University of Illinois, and the University of Chicago. Those were the only choices a few years ago, so look at the websites where you are searching to see if TOS is listed as a condition they would treat. TOS is misunderstood and missed most of the time unless you are a multidisciplinary facility that also treats TOS. I do physical therapy for my TOS with myofascial release. Surgeries create fascial scar tissue and tightness, and undoubtedly you will have tightness from your prior surgery and from any injuries, bad posture, etc you may have. Before you could try Myofascial Release therapy, you will need a complete understanding of spine instability and what risks it poses and if this is the right time to try it, or postpone until after a surgery. MFR may be able to help with all that tightness if you are able to try it. Let me know if I can be of further assistance. I would highly recommend my Mayo surgeon and he is a deformity expert and C spine expert, neuro/ortho, and teaches at Mayo and at surgeon's conferences. Mayo can evaluate TOS which causes nerve and blood vessel compression. Ask about it because you can have overlapping symptoms causing it to be missed. You will need copies of imaging DVDs to mail in if you want to seek an opinion at Mayo. Let me know if you need more information.

I have been dealing with issues since I was a child. I have dealt with severe migraines, that the doctors diagnosed me with menstrual migraines. Doesn’t make sense now since I have not had a period since November of 2010. Still have the migraines, although, much more severe and can last for weeks or even months. I also have limited motion of my arms and neck.

I fell on the ice, Christmas Eve of 2004, while carrying my 2 year old son. I fell right on the arm I broke when I was 7. That arm was set wrong. From then on my abilities with that arm slowly faded. Anything I do with that arm or hand causes severe pain with added vomiting from the pain. At the worst I can throw up every 10 minutes for hours. I have lesions in my throat from vomiting so much. Since I have been using my left arm more, I have severe pain when I use my left arm for the last 15ish years.

I have gone to many neurologists, orthopedics, 2 arm specialists and about 4 or 5 pain management doctors. I’ve been seeking help for over 18 years. Still no definitive diagnoses. In the beginning I would leave the doctor in tears because they would say it’s all in my head.

I have many other symptoms. Severe pain when I have to hold my own head up. I have to sit with my head supported or I am in severe pain. Which then leads into the debilitating migraines. It literally feels like my head will explode from the pressure. My pain alone can get so bad that I can vomit without having a migraine. But only if it is from my waste up, I broke my ankle and didn’t vomit from pain. I need to mention the severe neck pain I have on a daily basis. I haven’t been pain free for over 18 years, I’m not talking aches, I mean debilitating pain where I have to listen closely to my body. If my body is screaming, whether it’s only been 2 or 3 minutes, I must stop what I’m doing. Otherwise I pay dearly. If I’m pushing it, then I don’t have the help I need. I depend a lot on others. Most times people grow tired of you needing help all the time. So, there’s that too.

I also experience muscle jerking which is very painful and constant. I don’t get a break. The movement started about November 2006. That started at the elbow of the deformed arm and started out very slow. Looked like there was some creature in there moving around. It has gotten much faster and has spread up and down the arm. It can also happen in my left arm and both my legs when I’ve “overdone” it. The movement is worse at rest and when it looks like it has stopped it hurts worse. It’s like it’s tightening up really tight. If I lay my fingers on my right arm, pretty much anywhere on the arm or I rest that elbow on anything the movement intensifies and gets faster without any pressure. Just simply touching it.

I get all over body shakes when I’ve “overdone” it. When I get these body shakes I have to sit or lay down so I don’t fall down. My overdoing it is, doing something more than a couple minutes or something I shouldn’t do. Believe me when I say, if I do something I know I shouldn’t do, I pay big time. Sometimes I’ll “pay” the rest of the day. Which means I can’t do anything else for the rest of the day. Most times it affects me for days. When I really push it, it can take me weeks and sometimes months to recover.

I also have balance, dizziness and I stumble when I walk. Almost like I’m tripping myself.

Repetitive movements, not supporting head, sitting for more than 20-30 minutes depending on if I can support my head, doing anything above my head, looking up for longer than a few seconds, using scissors, writing longer than a few minutes, carrying anything over about 3 pounds, doing anything holding my arms out from my body are the worse things I can do. These acts cause the most severe pain.

Two more things. At one of my appointments with a neurologist he performed a full body reflex test. After that test the doctor sent me out to the waiting room to get blood work done. Before I left the examination room I started shaking uncontrollably from head to toe. They didn’t say anything about it. Also I felt like I had been hit by a truck. My body hurt so bad after that exam and the only thing he did different than any other doctor was the full body reflex test. It took me about a week or more to recover. I made the doctor aware of what happened at my next appointment and he said that’s impossible. A reflex test can’t cause that. I feel like the doctors are not looking at all my symptoms.

I have been dealing with this for so long and doctors will do one or 2 tests and they come back normal, they give up. I feel like if I don’t figure out what’s going on soon then I’m going to end up in a wheelchair soon.

I was denied genetics testing about the same time. Since then another doctor sent a referral to the same practice I had gone too before but to a different doctor. That doctor no longer practiced in the same field. So, I ended up with the same doctor. I told him last time I was there I was denied for the genetics test. He told me that genetics labs were now offering free genetic testing to individuals who’s insurance denies it. So, try again. Things may have changed in your state too.

Hi Bailey, has a diagnosis of Ehlers-Danlos Syndrome been considered or ruled out in your case?

No doctors have never mentioned that before but my cousin has. My cousin’s daughter was diagnosed and the doctor told my cousin that she most likely has it too with her symptoms.
What drew me to this site was the craniocervical instability. I read the symptoms on your Mayo Clinic’s website and a lot jump out to me.
I experienced early on an electrical sensation in my neck. It sounded sort of like what a cluster of electrical wires sounds like.

I should also add I was just diagnosed with mild tendenosis by an orthopedic. I have this cyst like bump on my wrist and I have severe issues with dexterity and strength in my wrist. I have more of these up my arm. The ones up higher are bigger. They didn’t show up on the MRI either. So he said he thinks they’re lipomas and I shouldn’t worry about it. I asked if he could help with the tendenosis and he said there was nothing more he could do. Didn’t tell me what to do next either.

Thanks for explaining. I wonder if @jenniferhunter or @jacklynn12 might have more thoughts for you about cervical instability. But it might be a thought to talk to your doctor about EDS.

I agree about looking into EDS. I will mention it to my doctor.
I’m also going to talk to him about testing my serotonin levels since I take meds that interact with each other. I try to be careful about how long I wait to take the meds apart from each other but not sure if that helps.