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Doc Appt-chiari, Cranial Instability And Ans? Yikes


mkoven
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I just had an appointment with the neurosurgeon recommended by the eds geneticist. I'm frankly overwhelmed and not sure how to sort through everything he told me.

He says I do have a 5mm chiari malformation, an abnormally low clivo-axial angle of 135 (normal is 165), and Harris and Grabb-okes angles showing cranial-cervical instability. With these together, he thinks my brainstem is getting pinched and this is causing the dysautonomia. He also told me I have ossification of the stylar hyoid ligaments bilaterally that could be putting pressure on the carotids.He did an mra with my head turned both ways to look at that. He also said the spinal cord shows some change in signal, showing stress on it. I think these were the highlights. It came at lightening speed without a lot of elaboration about the significance of each of these findings.

I explained that we were leaving town on 7/6 for Illinois (I've been on sabbatical in DC). For now he has me in a hard cervical brace till I return on Monday. He then said, depending on my response, he would consider a halo vest , which as I understand involves screws to the skull. He then talked about thepossibility of a suboccipital decompression-fusion.

He is of the opinion that the dysautonomia is a result of some combination of all this. I don't have a sense of how emergent/urgent this is. If it is not emergent, I don't think I'm ready for anything invasive, to include the halo vest, till I understand better, gather more information, and get back to the midwest. (He seemed to think I could continue to travel to DC to see him from Champaign, which frankly is not realistic). I really don't want surgery, or even screws placed into my skull unless it's absolutely necessary. But I don't want to do myself any harm either. Feels like a whole can of worms just got opened.

What do you guys think? ? He came highly recommended

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It seems to be a connection between chiari and pots.

I just been refer to the chiari institute and I don't even know how to react to the option of chiari.

I really hope that everything will be solved on the right side and that you feel better.

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Chiari is somewhat common in EDS ... in fact I think the chiari institute now automatically screens all of their cases for EDS.

I have actaully been wondering if it is in fact chiari that is the reason for autonomic issues in folks w/ EDS.

Now I will be looking around for info on folks that had both chiari and aut issues and went on to do the decompression surgery.

I wonder how many had improvements.

I have chiari symptoms, but I have said I would never iot for the surgery. I have heard it is not always has helpful in EDS cases.

Who are you seeing for the chiari?

Who did you see for the EDS?

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I?m in the process of making an appointment to the chiari institute in long island. My dr. refers me there less than 2 weeks ago. My muscles are very weak and I use crutches.

I have been told by this cardiologist that he know a case of someone that had pots and chiari ? and after the surgery all the symptoms have been solved ? both the chiari and the pots.

What are your symptoms?

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Mkoven, I'm sorry to hear you've joined the instability club. It's definately not a fun club to belong to. It does sound like you have a good NSG who understands your situation, and is willing to try some non-invasive treatments for now. Well somewhat non-invasive. The traction with the screws is invasive.

I hope you can put off surgery as long as possible, or not have to deal with it at all, but it sounds like you'll need to have something done the way your MRI sounds. At least he has experience with EDS and chiari.

A lot of people have chiari surgery who also have dysautonomia, and they end up with worsening of symptoms. Some do get better, and some only have slight improvement.

If you have EDS and have a chiari decompression, you would need to have it done by an NSG with experience with EDS, as a fusion needs to be done along with the decompression. Many folks with EDS have had failed chiari surgeries because the NSG didn't know they had EDS. You can imagine how poorly you would heal if you have EDS, and all that muscle area is cut in the back of your head and neck------which in turn you would lose some of the support-----many have ended up with cranial settling. It's a big decision on weather or not to have the surgery, as EDS in pretty complicated, and we need more surgeons who have experience on what complications can some with EDS------and that just doesn't mean chiari surgeries.

My problem is more cranial instability issues then the chiari, as I only have 1mm herniation. However, I have a small posterior fossa, the instability, a missing left vertebral artery, and an enlarged right vertebral artery making up for the one missing on the left. The right vertebral artery is also being kinked from the instability. My odontoid bone has pannus growth---(aka tissue growth), and my cerebral spinal fluid is partially blocked. I have pressure on my brain stem also, and it is though my dysautonomia is related to the instability, but I also have vascualr involvement from the EDS whihc causes blood pooling. I'm having a lot of problems lately, and my orthopedic surgeon wants to do a fusion of c1 & 2. I have 3 other opinions from genenticist, neurosurgeon, and another geneticist at NIH validating the instability, but my PCP would like another NSG opinion, but there isn't anyone so far that has taken it all that seriously. They don't understand the EDS/instability connection. I have a lot of subluxating in the cervical spine/cranial area----several times a day. My PCP doesn't want me walking around like this. He has seen the instability.

Maybe check out the EDNF (ehlers danlos nation foundation) website in the message boards to see of any expereinces with The Chiari Institute--------you would find it under the neurological section.

Personally, I have decided to avoid TCI.

Wishing you the best Mkoven.

Maxine :0)

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thanks for your replies. curious, maxine about your wanting to avoid tci. you can pm me if you'd prefer not to get into it on the forum. this guy does have experience with eds and chiari, but sometimes i wonder if in talking to a surgeon, you get to hear about surgery. I'd like to hear more about the "natural history" of these issues without surgery, and examples of good and bad experiences with surgery. i haven't healed well during prior surgeries. and i;m also concerned that a fusion would mess up the rest of my neck, where most of the discs are already bulging or herniated. wouldn't that put more stress on them?

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Maxine,

Here are two articles that may be able to help you and your PCP to understand the connections bewtween, chiari, EDS and cranial instability.

The first article is a paper written by Nazli McDonnel, Clair Francomano (two of the worlds leading EDS authorities) and then several NSG from the chiari institute.

The second article is a translation from the first article.

SO here you go:

Syndrome of occipitoatlantoaxial hypermobility, cranial settling, and Chiari malformation Type I in patients with hereditary disorders of connective tissue

Translation

My problem is more cranial instability issues then the chiari, as I only have 1mm herniation. However, I have a small posterior fossa, the instability, a missing left vertebral artery, and an enlarged right vertebral artery making up for the one missing on the left. The right vertebral artery is also being kinked from the instability. ..... They don't understand the EDS/instability connection. I have a lot of subluxating in the cervical spine/cranial area----several times a day. My PCP doesn't want me walking around like this. He has seen the instability.

Personally, I have decided to avoid TCI.

Wishing you the best Mkoven.

Maxine :0)

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Hi,

I have chiari too, but they did a CINE (?) scan and found my spinal fluid was moving, so no need for surgery. Boy was I glad. I consulted with 3 neurosurgeons, one said surgery, two said no surgery. I would definitely get a second and possibly a third opinion before going ahead with anything.

I have also been counseled to avoid TCI.

Angela

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Two questions-

-where does one get cine mri's done?

--to Maxine, and anyone else with cranial instability, I've noticed I have trouble lying on my side--it seems to push my head over, make me dizzy and give me a headache. How do you sleep? (Back? side? what type of pillow(s)?)

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I can't sleep on my side either, as I feel the same sensation.

Today I have mt Nephew's graduation party, so I'm in a rush, and it's blowing me away today.

My husband can't help becuase the post office is not allowing him the time off, so i'm on my own.

I'll get back to you with more information soon, :)

Maxine :0)

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I have a terrible time sleeping. My sleep is quite fragmented because I have to continuously shift positions.

Laying on my sides causes numbness in my arms from my shoulders to my hands. On my stomach ... I get a pinching sensation in my neck (I suspect from my tortuous cartoids), my back ... forget it! I get tons of nausea and congestion in my neck & head if I fall asleep on my back. I do need to lay on my back some to relieve my shoulders ... I just have to be sure I do not fall asleep like that.

One pillow that I found helped some was a side layers down pillow.

Eventually I want to get this chiari pillow.

Here is a link:

http://www.chiaripillow.com/

Two questions-

-where does one get cine mri's done?

--to Maxine, and anyone else with cranial instability, I've noticed I have trouble lying on my side--it seems to push my head over, make me dizzy and give me a headache. How do you sleep? (Back? side? what type of pillow(s)?)

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"Cine MRI (as in cinema) is taken the same way a traditional MRI is, with the addition of either a wristband or EKG leads on the patient's chest to measure the heart rate. Each time your heart beats, the cerebrospinal fluid is forced out of your brain, down toward the spine in response to the flow of blood that enters the brain with each beat. The MRI machine is equipped with an additional software package that allows the images to be put together, showing the flow of the cerebrospinal fluid (CSF) as it is moving."

Maxine :0)

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Mkoven,

I have no idea------------mine was done in NY. When they measured my CSF flow a pulse meter was put on my finger.

I don't know if my CSF is still partially blocked. I imagine it is since my symptoms are worse with the instability.

You were fortunate in that it has actually showed up on your MRI. Was contrast used? Did they put you into different positions?

Do you feel abnormal movement----subluxating ect.

My PCP is very concerned, and he and my general surgeon who was going to do my colonoscopy have both agreed I should have a virtual one. My concern is that if they find polyps I'll have to go through the whole thing again. Hopefully they'll find them low enough and I won't need to be sedated, but I hope they don't find ANY. When I had the barium enema, they weren't sure. The reason all this hoopla on the colonoscopy is because of sedation. We have no idea how I'll react, as my body is so sensitive, and intubation would be tricky, if not dangerous.

Today I'll see my PCP to discuss going to the Cleveland Clinic to have more tests. I simply can't continue to be dismissed locally. Dr. Grubb is not dismissive, but he's my POTs doc, and had way to much on his plate to help me with finding a decent neurosurgeon. My PCP is trying.

If thery're seeing instability on your MRI it sounds pretty significant. Do they know if you have cranial settling? Cranial settling is dangerous, and anyone that has this is advised to have surgery. The instability alone is reason for surgery.

Maxine :0)

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What shows up on mri is a 4mm chiari and an abnormally acute clivo-axial line of 135 degrees (normal is 165). This sharp angle compresses the brainstem when I flex my head. I also have a retroflexed odontoid. There were two other measurements that indicate instability and hypermobility (Harris and Grabb-Okes?) where I was into the abnormal range. If I do have surgery, it will be both a decompression and a fusion of skull to top two vertebrae.

I saw the surgeon again today and together we decided that I'm not bad off enough for surgery. I have to learn to keep my head in a neutral position and he wants me to wear a hard collar in any potentially neck stressing situation. It's up to me to determine if/when things are bad enough for surgery. When I get back to the midwest, I'll seek out an opinion there.

I'm relieved that surgery is not emergent. But he does think a lot of my neurological symptoms, some of my headaches, nausea, tingling tongue, vertigo, and ans stuff are from the instability. But I don't show any gross weakness.

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