The science of craniocervical instability and other spinal issues and their possible connection with ME/CFS - discussion thread

[Guest 2176]

As to the anatomy of cranial settling and vertical Instability, i understand this less, but i found another paper that addresses this , as well as addressing the need for uprigjt scans



The title of the paper is
Syndrome of occipitoatlantoaxial hypermobility, cranial
settling, and Chiari malformation Type I in patients with
hereditary disorders of connective tissue

And if you search for it on Google scholar there's a free PDF available.
I'm attaching some images that I think are relevant.

 

[Guest 2176]

Additionally, as to the diagnostic methods and reasoning behind them, i did find tbis image but can't find the original source--can anyone help me with that? It must be from a paper or case study of some kind.

 

[Mfairma]

I want to add a perspective that I think many patients share, but which has not really been fleshed out, at least not on the pages of threads I’ve read on this subject. To me, the central issue in this discussion revolves around the willingness to suspend doubt about health and treatment claims and how that willingness impacts our discourse...

Moderator note: The rest of this post and responses to it have been moved to this thread:
Medium Blogs by Jennifer Brea and general discussion of ethical and other issues.

 

[Guest 2176]

One final thought. @JenB you have mentioned you believe you might have a connective tissue problem seperate from eds. Do you have anyway of objectively figuring out what that might be? Is a biopsy of connective tissue done and would it be relevant?

I think that the type of collagen in skin differs from that in ligaments and tendons so a biopsy might be very invasive. But agree this wouls be interesting thing to study. I have to take a break before digging some more but i think the Bolognese paper discussed a biopsy of connective tissue but i may be wrong.. and the problem may havw been w rhe elastin not the collagen

 

[JenB]

@Jeff_w @debored13 Interestingly, someone in our group just had a consult with a neurosurgeon who recognized that this patient had CCI based on the same measurements that we’ve all been talking about. They are in the US but had consulted with Gilete. They took Gilete’s report to a local NS who then did their own measurements, found the same measurements as in the report, and agreed they had CCI.

However, this neurosurgeon said that they would just fuse that person exactly in place without correcting their pathological measurements. This is anathema to everything I know about this surgery. Maybe this is how some neurosurgeons have been trained to do it? If I had been fused in place, without intraoperative traction, my life would be a living hell.

 

[Jonathan Edwards]

Hi @debored13,

I have been away from the forum for a day or two. Thank you for your apology a few pages back. I also apologise for sounding grumpy and condescending much of the time. Trying to make sure people do not misinterpret clinical evidence is hard for at times. However, some of the recent posts are very helpful in terms of showing what I am trying to get clear.

Here's the first thing.
View attachment 9083
I would like to copy across these photos of MRIs but have not found out how to yet. They should come up with a click.

Let's look at the first three pictures, ignoring the authors' numbers to begin with. They show the relation of odontoid peg of axis to the front lip of the skull opening (foramen magnum). The reason for considering the position of the odontoid peg in this context is the worry that it could move upwards and press on the brainstem.

Which picture looks the safest position? I think it has to be the sitting position because in this position the odontoid peg is directly underneath the lip of skull bone and so cannot move up and press on the brainstem. On the other hand in the supine (lying face up) and traction positions the odontoid is further back and if it moved up it might slide up behind the bony lip and press on the brainstem. The brainstem is in the black space to the right of the bony lip on the first picture.

So it looks as if fixation in a traction position is a bad idea. This is something that used to worry me twenty years ago when patients came back from surgery apparently fixed in a worse position than before.

Traction will pull the neck straight and reduce kyphosis due to slippage at lower levels (not shown here) and it will pull the head back from the maximum flexion position that would be most dangerous (and associated with the smallest clivo-axial angle) but unfortunately it also seems to pull the head away from the truly safe position of normal sitting bringing it a little bit further forward.

So why do the numbers seem to improve. They seem to improve because the wrong thing is being measured. You can see that on the supine and traction pictures the measurement is oblique - as much front and back as up and down. All we interested in is the up and down position. The distance in the sitting position is only less because the odontoid has moved forward into a safer position. The actual plane of the Atlanta -occipital joint where forward -back movement occurs is, on these pictures sloping down to the right a bit. That means that the up and down position of the odontoid relative to the skull base plane does not actually change in any of these pictures. Traction achieves nothing in terms of 'lifting the head off' in this case. It just moves the odontoid to a rather poor position too far back.

JenB had suggested that traction lifts the skull off the head, by which I think was meant the skull off the neck. But this seemed to me highly unlikely since distraction at the atrlanto-occipital joint would create a vacuum. What is now clear to me is that the intention is to lift the skull up in relation to the axis and its odontoid, but not in relation to the atlas above it. It is intended to reduce upward subluxation of the odontoid or 'basilar impression' or 'cranial settling'. In these pictures it does not do that because the up and down position does not change - as you can see by comparing the position of axis and atlas - which is where the reduction would occur.

It might be that in this case or other cases the reason to operate is to protect the brainstem from future upward subluxation of the odontoid but clearly if so the traction position front and back is bad.

There is a lot more detail to go into but that is enough for one post. It looks as if this study has made exactly the sort of mistake that I worry radiologists and surgeons often make.

 

[Guest 2176]

Hi @debored13,

I have been away from the forum for a day or two. Thank you for your apology a few pages back. I also apologise for sounding grumpy and condescending much of the time. Trying to make sure people do not misinterpret clinical evidence is hard for at times. However, some of the recent posts are very helpful in terms of showing what I am trying to get clear.

View attachment 9083
I would like to copy across these photos of MRIs but have not found out how to yet. They should come up with a click.

I'm on mobile, only, but i just click on the attachment, then hold my thumb on the screen after it's enlarged , and then am given an option to download it.

 

[Guest 2176]

Oh if you mean rhe specific figures you'd have to go to the original paper to do that, i just screenshotted an excerpt that contained some of the photos

 

[Jonathan Edwards]

Just a note about 'CXA'. I had not heard this term before coming on ME forums. It means clove-axial angle and I can see why it might be thought relevant to CCI. A small angle would indicate the head flexing forward on the spine and that is the worst position for CCI by and large. But I would be sceptical that it would be a reliable guide to anything being wrong with the actual structure.

I googled CXA and nearly all the mentions are in papers by Henderson, with some of the top hits being via ME forums. That worries me because I have a strong feeling that the 'consensus' about this measurement is isolated to a group of surgeons who cluster around Henderson and have an interest in clinically marginal CCI-related problems. Why are no other neurosurgeons writing papers about CXA?

I have so far not found anything that convinces me there is any evidence for this angle being useful. There is a study by Henderson of people with small (i.e. 'bad') CXAs having surgery with several getting better. However, this is not a 'correlation' between CXA and improvement. `To show a correlation you need a variable and in this study all the CXAs were small. We would need a study of people all with similar clinical signs of CCI but some with smaller CX and others with larger CXA and a result showing that those with the smaller CXA gained more benefit than those with large CXA. Otherwise there is no point in measuring CXA because benefit depends on the signs of CCI, not the CXA.

 

[Jonathan Edwards]

The Henderson paper suggests that intraoperative traction is used. It even gives a diagram of this process. I don't understand how a surgery like this wouldn't improve symptoms , but simply try and prevent worsening, like @Jonathan Edwards has suggested , if what Henderson is saying is true and they are correcting kyphosis.

The reason why surgery mostly prevents worsening, rather than improving symptoms is that the symptoms and signs of CCI, at least in cases with structural changes, are largely due to long term irreversible death of nerve fibres from repeated persistent compression. Brain tissue tolerates pressure for short periods but not for long periods. Transient pressure on the brainstem and cord is mostly silent, although it can cause blackouts. In people with CCI with rheumatoid arthritis there was no particular history of symptoms going up and down with head position. I wouldn't expect traction over a day or two to alter symptoms or signs due to CCI much if at all except in catastrophic cases that had recently progressed and even there one would not be optimistic.

 

[Trish]

Moderator note:

We now have 3 threads on related issues:

Concerns about craniocervical instability surgery in ME/CFS
for discussing medical diagnosis, surgical methods and research

Medium Blogs by Jennifer Brea and general discussion of ethical and other issues.
For discussing wider issues relating to how this impacts the ME community and ethical issues

And this thread in the members area for sharing personal stories

Some recent posts have been or will be moved to the appropriate thread. It would help members if you keep your posts to the appropriate thread. If you think your recent post needs to be moved, please use the report button and ask moderators to move it.

 

[ME/CFS Skeptic]

I googled CXA and nearly all the mentions are in papers by Henderson, with some of the top hits being via ME forums. That worries me because I have a strong feeling that the 'consensus' about this measurement is isolated to a group of surgeons who cluster around Henderson and have an interest in clinically marginal CCI-related problems. Why are no other neurosurgeons writing papers about CXA?

Isn't it also the case that this notorious consensus statement was never published in a scientific journal?

I had to go find it on page 25 of some internal document of the Chiari & Syringomyelia Foundation but it seems that even that link no longer works. I think the organisation renewed their website as they are now called The Bobby Jones Family and the Chiari & Syringomyelia Foundation.

Can the persons who think this consensus is important point out where it can be found and who the exact authors were?

 

[Cheshire]

Can the persons who think this consensus is important, point out where it can be found and who the exact authors were?

And official guidelines refering to this consensus...

 

[Jonathan Edwards]

It looks to me from the Bolognese paper that 'functional cranial settling' means where the clivo-odontoid distance reduces on sitting but there is no actual upward movement of the odontoid on the atlas. It seems that the term functional acknowledges the absence of any actual upward movement. As far as I can see from the pictures shown the changes in distances are not a sign of anything that would cause concern - simply a measure of backward sliding of the occipital condyles, which would be normal.

 

[ME/CFS Skeptic]

Isn't it also the case that this notorious consensus statement was never published in a scientific journal?

I also want to point out that that consensus statement only speaks of CXA angles that are "potentially pathological". It reads:

The literature suggests that a clivo-axial angle of 135 degrees or less is potentially pathological. That is a CXA of135 degree, may in some circumstances, result in harmful deformative stress upon the brainstem and upper spinal cord and, therefore, warrants consideration for further evaluation and possible treatment.

So a CXA of 135 is unusual but they don't really know what it means yet, it depends on circumstances.

In this video about the 2013 consensus statement, Henderson explains that "there was no attempt to discuss what constitutes a surgical indication." https://bobbyjonescsf.org/csf-video/review-colloq-2014/

The video is interesting because Henderson says that Rodney Graham and Peter Rowe were at this 2013 consensus meeting. And at the end of the video one of the guests remarks that "calling it the Cervical Medullary Syndrome sort of implies cause and effect. It's a little bit worrisome to me to lead that impression, it may well be right and the more I think about it the more likely it is that it is right, but it is certainly controversial. I think we have to prove that this is cause and effect rather than just something that does happen. So I'm a little bit troubled by the Cervical Medullary Syndrome with all of these things like dysautonomia. I think it's true, I just don't think we can't say that."

 

[Jonathan Edwards]

So I'm a little bit troubled by the Cervical Medullary Syndrome with all of these things like dysautonomia. I think it's true, I just don't think we can't say that."

It also raises the issue that surgeons may be operating on the basis of 'CCI' indications that form part of a syndrome they have recently invented but which does not in reality exist.

 

[ME/CFS Skeptic]

Could you update this with the information from the largest metastudy @Michiel Tack?

This one, 2274 surgeries, zero deaths:

I have this review in my folder and although I didn't cite it directly, I used it for writing my forum posts back then. So I'm not sure what you want me to update. The review writes what I wrote, namely:

Surgical management of craniovertebral and cervical instability using screw- and wire-based fusion is associated with a variety of perioperative risks. Injury to the vertebral artery, nonfusion, hardware dysfunction, deep wound infection, and dural laceration and CSF leak are among the more serious risks associated with such procedures.

Perhaps you want me to write that death is very rare with these surgeries? If the review had explicitly made that conclusion and written about it then I might have too but it simply doesn't mention mortality if I remember correctly. It's quite likely that's because it's so rare but there might be other reasons (for example difficulty distinguishing deaths from the underlying condition and deaths related to complications of surgery). There are other studies that mention death as a complication such as Kukreja et al. Given that most studies and reviews do not make clear conclusions regarding mortality following fusion surgery and that this risk probably depends on the underlying condition, I've tried not to focus on this or try to give an indication of how common this is. The death in the Goel study is probably the only time I've mentioned this at all (actually I remember that you have been more outspoken about the risk of CCI-related death than me!).

It's difficult to write about high-impact outcomes that have a very low probability, but in retrospect, I don't think I've written something wrong. I suspect that with a major surgery like this there's always a small chance for the most dramatic outcome and some neurosurgeons probably tell that to their patients as well.

You might also note in the n=779 study you quote that 81.58% of patients had improvement in neurological outcomes. This is a little more concrete than the Goel study, about which you remark that outcomes are “generally good.”

Regarding the highlighting of improvements, I can be brief: most of these are changes over time not compared to a control group. So it doesn't control for placebo effects or the natural course of the illness and can only give a rough estimation of treatment effects. So I think that writing that "outcome is generally good" is actually more accurate than the specific numbers you would like to add.

Regarding the rib bone harvest: this is from the recent Henderson study (a 5-year follow-up), probably the most relevant study that we have that was published this year. So it's probably not that old or irrelevant. I hope that new techniques will reduce the complication rate, but until we have some published data on the complications of those techniques I think it's ok to cite the relevant data that we do have.

Finally, I am not sure that “distinct from ME/CFS” is exactly right. Some symptoms overlap

I think this has already been addressed earlier in this thread. It is certainly possible that patients with CCI have symptoms that patients with ME/CFS also have. Such overlap is probably possible in all medical conditions. What matters is the clinical picture and the main impairments patients have. As I understand it people like Jonathan Edwards or Todd Davenport who have cared for CCI patients say it doesn't look like ME/CFS and that was also my impression from reading some of the literature. The clinical characteristics that clinicians use to recognize and diagnose a condition are clearly different in ME/CFS and in ICC. I suspect that CCI experts agree with this and that they wouldn't suspect CCI when presented with a typical ME/CFS patient.


Your comments have made me reflect on whether it was appropriate for me to write about this subject back in May, something Hip brought up earlier. Given that, except for Jonathan Edwards, no medically qualified person or group have come forward to thoroughly discuss this and provide a sceptical view to the strong claims being made (there were only some brief comments to be cautious) seems to suggest that it was. Because at the time there were statements such as the one from Jeffs website below that I think are misleading.


Practically no information was being given about clinical presentation of CCI itself; it was being confused with the recent reports about EDS with CCI so that POT, mast cell problems and irritable bowel syndrome were presented as symptoms of CCI. The complications of fusion surgery were only briefly or simply not mentioned. So in that context, I think it was appropriate to collect and share information to provide some more background with the caveat that I’m not medically qualified (I wrote: "I am obviously no expert in these complex surgical procedures and it's possible that there are some inaccuracies in the text above. I simply did some research and read some studies. I hope the information I collected will be helpful for patients to interpret the recent ME/CFS recovery stories.") That's still the case.

The information I collected was related to concerns about CCI diagnosis and surgery, things that I think you and Jeff should have made clearer as you presented your hypotheses and recovery stories. So it was written in response to other information, which explains why it isn't an encyclopedic lemma or comprehensive overview of information on CCI as you seem to want it to be. It focused on things such as the difference between CCI and ME/CFS, the trustworthiness of the CCI experts and the complications with fusion surgery that in my view were not properly discussed at the time.

I'm disappointed that few of the concerns I've tried to raise have been taken seriously and that Jeffs website, which you @Jrehmeyer describe as "indispensible", still makes the claim posted above. EDIT: the fact that even Bolognese has warned to avoid 'amateur hour' on Facebook suggests that things have moved too fast and with too little caution.

 

[JenB]

I’ve said this several times but I suspect it has gotten lost (sorry for the repeat photo but I think it is important to this conversation). I did have many symptoms that were “classic” for CCI going back to the beginning of my illness. However, I had many other symptoms besides. This is because I had at least three different conditions (CCI, tethered cord syndrome, MCAS) that were all contributing to a very complex clinical picture. If you weren’t used to these diagnoses frequently co-occurr, you might fail to see them at all.

I suspect that CCI experts agree with this and that they wouldn't suspect CCI when presented with a typical ME/CFS patient.

No, I don’t think the “CCI experts” would agree with this. First, they are really “Chiari, tethered cord, CCI and the occasional Eagle’s Syndrome plus other things that people with EDS seem to have in high numbers” experts. Because they almost exclusively see EDS patients, many of the patients they treat have MCAS, SIBO, gastroparesis, POTS or other dysautonomias alongside whatever their neurosurgical diagnoses might be. They are used to seeing very complex cases, and would not be surprised to see “classic” Chiari or CCI symptoms jumbled together with many other symptoms in a patient who has a connective tissue disorder and multiple co-morbidities. This is why diagnosis can be so challenging...unless you are used to seeing this patient population. My neurologist back in 2012 never imagined I had a neurosurgical condition. I simply had far too many symptoms. Remember that in many doctor’s offices, they ask you, “What are your three most important symptoms?”

(The symptoms I personally had are below in bold. I would also add that sensitivity to food and chemicals can be symptoms of MCAS and MCAS can also contribute to intestinal problems.)

 

[JenB]

I'm disappointed that few of the concerns I've tried to raise have been taken seriously and that Jeffs website, which you @Jrehmeyer describe as "indispensible", still makes the claim posted above. EDIT: the fact that even Bolognese has warned to avoid 'amateur hour' on Facebook suggests that things have moved too fast and with too little caution.

No one can diagnose or operate on themselves. The only practical harm I can see from this is a random person on Facebook tells a person they *might* have something they don’t actually have and that person feels anxious until they go see a doctor. (Obviously, that’s not great if you have an anxiety disorder.) This happens on Facebook several times a minute, across thousands of patient groups, across hundreds of diagnoses, and was happening long before Jeff ever told his story.

This is again an ethics conversation and goes back to what @debored13 was saying earlier about consequentialism. https://www.s4me.info/threads/mediu...al-and-other-issues.11138/page-30#post-217828

As for too fast, a very large proportion of the PWME who have been diagnosed with one of the above conditions has EDS. Some already knew this but never considered the role EDS might play in their health. Others are being diagnosed for the first time because of this conversation. Or discovering they have a strong family history of tethered cord, for example. People with EDS have been getting these diagnoses and these surgeries for decades. Consider that some of these concepts and conversations are new to us and so it may seem that things are unfolding faster than they actually are. If we were to transpose this thread onto an EDS forum, the people there would be very confused by this entire conversation.

 

[Guest 2176]

Hi @debored13,

I have been away from the forum for a day or two. Thank you for your apology a few pages back. I also apologise for sounding grumpy and condescending much of the time. Trying to make sure people do not misinterpret clinical evidence is hard for at times. However, some of the recent posts are very helpful in terms of showing what I am trying to get clear.

Here's the first thing.
View attachment 9083
I would like to copy across these photos of MRIs but have not found out how to yet. They should come up with a click.

Let's look at the first three pictures, ignoring the authors' numbers to begin with. They show the relation of odontoid peg of axis to the front lip of the skull opening (foramen magnum). The reason for considering the position of the odontoid peg in this context is the worry that it could move upwards and press on the brainstem.

Which picture looks the safest position? I think it has to be the sitting position because in this position the odontoid peg is directly underneath the lip of skull bone and so cannot move up and press on the brainstem. On the other hand in the supine (lying face up) and traction positions the odontoid is further back and if it moved up it might slide up behind the bony lip and press on the brainstem. The brainstem is in the black space to the right of the bony lip on the first picture.

So it looks as if fixation in a traction position is a bad idea. This is something that used to worry me twenty years ago when patients came back from surgery apparently fixed in a worse position than before.

Traction will pull the neck straight and reduce kyphosis due to slippage at lower levels (not shown here) and it will pull the head back from the maximum flexion position that would be most dangerous (and associated with the smallest clivo-axial angle) but unfortunately it also seems to pull the head away from the truly safe position of normal sitting bringing it a little bit further forward.

So why do the numbers seem to improve. They seem to improve because the wrong thing is being measured. You can see that on the supine and traction pictures the measurement is oblique - as much front and back as up and down. All we interested in is the up and down position. The distance in the sitting position is only less because the odontoid has moved forward into a safer position. The actual plane of the Atlanta -occipital joint where forward -back movement occurs is, on these pictures sloping down to the right a bit. That means that the up and down position of the odontoid relative to the skull base plane does not actually change in any of these pictures. Traction achieves nothing in terms of 'lifting the head off' in this case. It just moves the odontoid to a rather poor position too far back.

JenB had suggested that traction lifts the skull off the head, by which I think was meant the skull off the neck. But this seemed to me highly unlikely since distraction at the atrlanto-occipital joint would create a vacuum. What is now clear to me is that the intention is to lift the skull up in relation to the axis and its odontoid, but not in relation to the atlas above it. It is intended to reduce upward subluxation of the odontoid or 'basilar impression' or 'cranial settling'. In these pictures it does not do that because the up and down position does not change - as you can see by comparing the position of axis and atlas - which is where the reduction would occur.

It might be that in this case or other cases the reason to operate is to protect the brainstem from future upward subluxation of the odontoid but clearly if so the traction position front and back is bad.

There is a lot more detail to go into but that is enough for one post. It looks as if this study has made exactly the sort of mistake that I worry radiologists and surgeons often make.

Im replying partially just to remind myself to reply later as I do not know how to bookmark a part of a thread.

This is an interesting discussion and i would like to fully understand the anatomy. Just woke up and a little tired though.

I would like to read those papers in full and also to ask Bolognese ans Henderson by email what their thoughts on this is.

Btw, wrt "cxa" , one justification in the study was not simply the case studies in which cxa was corrected and the patients improved. The other justification was based on some form of modelling (i dont quite understand whether it was a computer model or what) in which cxas under 150 started to be associated with some kind of sign or proxy of stretch injury. Also, that particular Henderson paper on the cxa cites a lot of literature. I'm aware that quantity is no substitute for quality and i haven't yet read those sfudies , but it does seem to suggest Henderson did not simply invent this measurement. Perhaps he revived a lesser used measurement because he thought it deserved more prominence. I would be interested in a clear history of measurements used in CCI. One of the studies, iirc, is from 2004