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

[Guest 102]

My experience with a neurologist back in 1992 was positive. He ordered a lot of blood work to r/o other illnesses and sent to the hospital for various tests for vertigo. I was never sent to a spine specialist/surgeon, thank goodness.

Mine too, Mij, a positive experience with neurology, thankfully.

During period 1982 - 1987, I attended the late Prof Behan's clinic in Glasgow (west of Scotland). He was also having referrals sent from north of England at this time, such was his 'expertise' in ME then. I will be forever grateful to him for diagnosing me with 'presumed' ME. I, of course, had no idea then that he worked with Melvin Ramsay, we had never heard of ME before, any of us. Or Coxsackie. I also had no idea that, in thirty years time, I would have to bore myself stupid (and believe me, I really do bore myself!) highlighting what RamsayME is amidst a proliferation of criteria that have since been introduced.

I was never investigated for CCI or tethered cord or anything structural as it was clear to Behan (after EMG and muscle biopsy and immune profiling) that I had severe ME - and there has been nothing remotely since then to suggest I have any mechanical issues. I have also never heard Prof Chaudhuri, Behan's predecessor for a while, mention mechanical issues and ME either. He is the cons neurologist who did autopsy on the late Sophia Mirza. He also lists Chiari as a differential diagnosis in the most recent MEA clinical guideline. I tend to trust what he says.

In the 1980s pwME were told they had candida overgrowth, that was the root of ME! We all gave up bread and cheese and of course it made no difference if you actually had ME and not candida overgrowth. Hypotheses come and go, treatments are in vogue, but at least not eating bread was not going to endanger you in even the slightest way. FWIW I remained neutral about both XMRV and rituximab, waiting to see how they panned out. I never got carried away by hope. Or hype. One has to protect oneself from false hope with this illness.

Of course, we know that many neurologists in UK currently - Behan was bravely with a few others going against the establishment thinking - are still utterly clueless - and we have the FND club right here in Edinburgh (Stone and co).

Edit: Just to add, I attended the late Prof Behan's service recently, was important to pay my respects and a friend/colleague of his - who is actually professor of psychiatry - made reference in his eulogy to Behan's sterling work on ME in 1980s, when very few doctors were giving the illness the respect it deserved.
*edited to add 'eulogy'

 

[Mij]

In the 1980s pwME were told they had candida overgrowth, that was the root of ME!

Yes of course, here in Canada as well

 

[Guest 102]

My experience with a neurologist back in 1992 was positive. He ordered a lot of blood work to r/o other illnesses and sent to the hospital for various tests for vertigo. I was never sent to a spine specialist/surgeon, thank goodness.

Puts me in mind of the 'snakeoil' salesmen that we have come into contact with over the past decades. Several of whom, when questioned, would say, 'Oh yes. We plan on a proper clinical trial.' It looked good on their websites but they never did them of course. It would eat into the profit margins. Still, I do hope this team in Spain - especially as it does affect people who are raising funds from the UK - are FORCED to comply with whatever health agency there might be over there, and publish something useful BEFORE performing any more operations on people with M.E.

Edit to add: If all this talk about ME, CCI and surgery continues, perhaps a statement should be sought from the neurologists or their agencies here in the UK. Maybe they can shed some light on why such surgeries are not being recommended at home? Perhaps, as @Jonathan Edwards says above, we don't need a clinical trial at this point, maybe it would be sufficient if neurologists could all have access to the scans and determine if the diagnosis was correct and surgery was warranted in these individuals?

It's interesting that the resolution of ME is being experienced as a *biproduct* (is that the correct word?) of this surgery, which is being performed to resolve/treat structural issues. I just cannot see why misdiagnosis of ME is not being considered more. It's not rocket science. And ME is misdiagnosed all the time, it is not infrequent.

FWIW, I would love to have been misdiagnosed with ME, and although this surgery sounds so very frightening - and I salute anyone who goes through it - how unspeakably wonderful to wake up and be instantly free of the yoke of ME (whether misdiagnosed or correctly diagnosed). I cannot even begin to imagine.

 

[Trish]

None of the treatment that has helped me is generally available in the UK for people with ME (or EDS). For better or worse, we have a different healthcare system. Please recognize that this is some of the gap.

The only way for patients to improve from this surgery is if they have CCI/AAI. This is not a generic treatment for ME/CFS, but rather for a specific pathology that some people can have.

@JenB that is a very odd pair of statements from the same post. You rightly say CCI/AAI treatment is for CCI/AAI not for ME (or EDS). These treatments are available in the UK for people who have the conditions they are suitable for. Of course they are not available for people for ME/EDS because they are not treatments for ME/EDS. It is possible to have more than one condition at once, and if so, at least in theory, the treatment is available.

I am not offended—was just hoping for some substantive engagement with the recent posts.

Patience, please @JenB, I doubt any one of us with ME has had time to read and be ready to discuss the details of what has just been posted on this and other threads in the last few days.

Just because we don't immediately discuss it at a technical level doesn't mean we aren't interested and trying to read and understand it.

Speaking personally, I have managed to watch one video in which it was made clear that:

- Amateurs should never try to measure angles or guess at diagnoses from scans. It takes many years of training and experience to know what you are doing.

- Amateurs should not try to work out whether someone's symptoms are relevant or specific to a particular diagnosis.

- Scans alone are not diagnostic - eg healthy gymnasts might be diagnosed on the basis of scans because they are very flexible, so may have larger range of movement than is considered 'normal'.

- Only neurological symptoms specific to the particular diagnosed structural problem are relevant,

- Only if symptoms are very disabling should surgery be considered.

- Even then there is a protocol of physical therapy to try first, with surgery as a last resort.

So I learned a lot from that particular surgeon's experience.

I have no idea why Mattie was under anesthesia for 14 hours.

Do you have individuals' permission to share their medical information on a public thread?

You are fortunate to not have problems with your spine—they can be quite debilitating!

I'm sorry, Jen, I'm sure that was meant as a kind remark, but it came across as belittling the level of debility of those of us with ME without spinal neurological symptoms. Calling someone fortunate when they have severe ME may not be a good move.

 

[JenB]

@JenB that is a very odd pair of statements from the same post. You rightly say CCI/AAI treatment is for CCI/AAI not for ME (or EDS). These treatments are available in the UK for people who have the conditions they are suitable for. Of course they are not available for people for ME/EDS because they are not treatments for ME/EDS. It is possible to have more than one condition at once, and if so, at least in theory, the treatment is available.

I’m not sure why this is odd. I and others with ME diagnoses have benefited quite a lot from Mestinon and antivirals, for example, which are generally not available in the UK for people with ME diagnoses. (Neither of these are, by the way, generic treatments for ME, either.) So whether it’s medication or surgery, nothing I have benefited from in the last seven years is available on the NHS. This can be viewed as terrible or just so, depending on your point of view. I am grateful I have been able to access the treatment I have needed. Others may feel differently about their own cases.

Speaking personally, I have managed to watch one video in which it was made clear that:

- Amateurs should never try to measure angles or guess at diagnoses from scans. It takes many years of training and experience to know what you are doing.

- Amateurs should not try to work out whether someone's symptoms are relevant or specific to a particular diagnosis.

- Scans alone are not diagnostic - eg healthy gymnasts might be diagnosed on the basis of scans because they are very flexible, so may have larger range of movement than is considered 'normal'.

- Only neurological symptoms specific to the particular diagnosed structural problem are relevant,

- Only if symptoms are very disabling should surgery be considered.

- Even then there is a protocol of physical therapy to try first, with surgery as a last resort.

So I learned a lot from that particular surgeon's experience.

That’s a great, accessible talk! So glad you had a chance to see it. I do think it encapsulates many ideas we have been trying to convey here for some time.

Do you have individuals' permission to share their medical information on a public thread?

I’m not sure what you are referring to.

I'm sorry, Jen, I'm sure that was meant as a kind remark, but it came across as belittling the level of debility of those of us with ME without spinal neurological symptoms. Calling someone fortunate when they have severe ME may not be a good move.

Mij was stating how glad they were not to be referred to a spine specialist. I would have been VERY glad to have been referred to a spine specialist because that is what I needed all along. If you don’t need it that’s a good thing because it can be really shitty to have to deal with all this. It is fortunate not to need surgery, especially if you have severe ME. Mij themselves said that they felt fortunate not to be referred to a spine specialist so I thought I was just reflecting back Mij’s feelings on the subject. At least—I thought this is what Mij was saying!

@Mij sorry if I got that wrong!

 

[Mij]

@JenB I was reflecting on my own personal experience when I wrote that I was thankful that I was not referred to a spine/neck specialist. I was a gymnast when I was younger and the radiographs might have indicated an 'abnormality' when in fact it might have been normal in my case.

 

[Cheshire]

@JenB that is a very odd pair of statements from the same post. You rightly say CCI/AAI treatment is for CCI/AAI not for ME (or EDS). These treatments are available in the UK for people who have the conditions they are suitable for. Of course they are not available for people for ME/EDS because they are not treatments for ME/EDS. It is possible to have more than one condition at once, and if so, at least in theory, the treatment is available.

Yes, there is a strong problem here that has never been addressed.
CCI/AAI is an official diagnosis, recognised by mainstream medicine. Why do people have to fly to Spain to get treatment?

 

[JenB]

It’s also worth noting that Dr. Bolognese is talking about CCI/AAI. Obviously Chiari, cervical stenosis, subaxial instability, Eagle’s Syndrome, spinal fluid leaks all present differently but can also have overlapping symptoms.

 

[Trish]

Amidst all this enthusiastic promotion of the surgical route for those diagnosed with CCI/AAI, chiari etc, it has been very interesting to read a personal account on PR of someone who was diagnosed some years ago, but opted not to have surgery, and is very glad not to have done so.

By contrast they are in contact with many others who did opt for surgery and have had ongoing problems including needing further surgeries.

We don't allow copying or screenshotting of posts from other forums here, but since it is in the public area there, we do allow links:
https://forums.phoenixrising.me/thr...cci-as-a-cause-of-your-cfs.56908/post-2240206

Please don't copy any excerpts from the post here. Perhaps someone could encourage the individual to join us here and tell us more.

 

[JenB]

@JenB I was reflecting on my own personal experience when I wrote that I was thankful that I was not referred to a spine/neck specialist. I was a gymnast when I was younger and the radiographs might have indicated an 'abnormality' when in fact it might have been normal in my case.

It’s very common among people with EDS (which I am not saying you have) to be gymnasts or ballerinas when young, due to their gift of flexibility. I think that is very much Dr. Bolognese’s point: that flexibility/hypermobility and instability of a joint are not the same thing. People with EDS seem to be more prone to developing instability for reasons we don’t yet completely understand (it’s not just about being “bendy”) but this insight also means that perhaps people who are not hypermobile can also develop instability, for reasons we don’t understand.

His POV is to be much more careful re: MRI measurements and to use them as a first step screening tool, not an end all, for exactly the scenario you describe.

 

[JenB]

It’s very common among people with EDS (which I am not saying you have) to be gymnasts or ballerinas when young, due to their gift of flexibility. I think that is very much Dr. Bolognese’s point: that flexibility/hypermobility and instability of a joint are not the same thing. People with EDS seem to be more prone to developing instability for reasons we don’t yet completely understand (it’s not about being “bendy”) but this insight also means that perhaps people who are not hypermobile can also develop instability, for reasons we don’t understand.

His POV is to be much more careful re: MRI measurements and to use them as a first step screening tool, for exactly the scenario you describe.

And there is also a tendency in the EDS community to view hEDS in particular as a total explanation for people’s symptoms. I fight strongly against this because, as I am sure others have pointed out, there are many with this same phenotype who are perfectly healthy and not chronically ill at all. Even when traits are inherited, when is something rightly considered a “disease” if so many people are asymptomatic? I sometimes think there is a tendency within hEDS to “select on the dependent variable.” That is, to observe that all of these people who are hypermobile are sick, therefore their sickness is due to hypermobility. It is true that people in the hEDS community are very sick. I just wish there was more work done to compare people with hEDS who are sick to people with the same physical traits who are not. Just as I wish there was better subsetting of ME data into people who meet hEDS criteria and those who do not. (The research on people with ME w/ w/o POTS has I think been very illuminating.)

 

[JenB]

Amidst all this enthusiastic promotion of the surgical route for those diagnosed with CCI/AAI, chiari etc, it has been very interesting to read a personal account on PR of someone who was diagnosed some years ago, but opted not to have surgery, and is very glad not to have done so.

By contrast they are in contact with many others who did opt for surgery and have had ongoing problems including needing further surgeries.

We don't allow copying or screenshotting of posts from other forums here, but since it is in the public area there, we do allow links:
https://forums.phoenixrising.me/thr...cci-as-a-cause-of-your-cfs.56908/post-2240206

Please don't copy any excerpts from the post here. Perhaps someone could encourage the individual to join us here and tell us more.

Oh...yes, but she also has some confusing ideas about CCF surgery. For example, that most people benefit initially from fusion surgery because they develop spinal fluid leaks during their surgeries, which relieves intracranial hypertension. (Spinal fluid leaks generally cause horrible symptoms but I am also not entirely sure how this can regularly happen as this surgery does not involve opening the dura or doing any laminectomies.)

There are so many stories of people who have CCI who do not have surgery. Diagnosis /= surgery, not by a long shot. Diagnosis often means that you have the means for prevention of further injury. There are also many people who have benefited from PT or experimental or alternative non-surgical treatments, which I won’t describe here. There are a lot of people who are enthusiastically not pursuing surgery, but are glad to have been diagnosed with the things they have, whatever they are. I would say that the majority of people who have been diagnosed with CCI/AAI are unlikely to be having surgery anytime soon. They have either decided or have been advised by their surgeons to try other things first. (People with severe neurological deficits are more likely to be having surgery.)

And obviously if your main symptoms are from intracranial hypertension, as they were in this person’s case, there are medical options* for managing that. As well as other surgical treatments, depending on the cause.

(*by this I mean medication)

 

[Dx Revision Watch]

...Finally, I am not sure that “distinct from ME/CFS” is exactly right. Some symptoms overlap, as is clear from the Venn Diagrams. I had neurogenic bladder due to tethered cord syndrome. The symptoms of neurogenic bladder are mentioned in the ICC, for example. As are gait abnormalities, lack of coordination and dizziness. Still other CCI symptoms like tinnitus and parasthesia are mentioned by Ramsay.

Gait abnormalities, lack of coordination, dizziness, tinnitus and parasthesia are symptoms common to a number of diseases/disorders.

 

[JenB]

Gait abnormalities, lack of coordination, dizziness, tinnitus and parasthesia are symptoms common to a number of diseases/disorders.

Absolutely! Most symptoms associated with ME are symptoms of other conditions. The same symptoms can have many different causes, even within the same person.

 

[JenB]

Yes, there is a strong problem here that has never been addressed.
CCI/AAI is an official diagnosis, recognised by mainstream medicine. Why do people have to fly to Spain to get treatment?

I don’t know why people in the UK have to fly to Spain. I obviously did not.

I will also repeat: this is just one of many diagnoses.

 

[Hutan]

Moderator note: please focus on the topic. The topic is not whether people like to post on S4ME.
The thread is open again.

 

[Guest 2176]

Since the threads keep going so far off topic of the questions relating to the anatomy it's hard for me, with brain fog, to totally rewrite my sentences. I hope it's all right I i copy some from the other thread that was on similar topic and closed:

One thing I would like an answer to is whether traction is really supposed to separate the occipital condyles from the atlas, so that fixation takes weight entirely through metal rather than articular surface. That would seem to me very risky indeed in the long term and I do not understand the rationale if CCI is judged on the angles that indicate forward/back slip rather than vertical translation

This is a question that I would be interested in the answer to as I don't quite understand the anatomy of cranial settling and vertical instability.

However it seems clear in the literature (https://link.springer.com/article/10.1007/s10143-017-0830-3) that traction can correct a kyphotic cxa, which is a measurement that's a proxy for brainstem compression via the odontoid bone, and that this measurement is associated with pathology, and that intraoperative traction is used to correct the cxa and fuse in a better position.

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.

So, the cranial settling anatomy is something that I can't speak to. But if many of the people with a CCI diagnosis have a kyphotic cxa I don't see how it is somehow unbelievable that traction would tell us something about their symptoms , and mimic the effect of a fusion.

 

[Guest 2176]

I did not say that. If we are to have an intelligent discussion it is important to understand what the other person actually said.

. I apologize for misrepresenting your position by saying you said traction could not alleviate brainstem compression at all. I did not mean to misrepresent you. I inferred that you thought this because you said traction wouldn't be a useful test.

You recently said: "Traction will relieve brainstem compression by reducing forward-back displacement. The comment I made previously was that I though ti very unlikely that it had anything to do with raising the skull up in relation to the cervical vertebrae (certain not in relation to the head as JenB had originally said)."

And at another point said: "Symptoms of CCI by and large do not change with traction. Even with surgery they mostly do not improve but stabilise. I see no reason to think that traction is a useful guide to diagnosis. "

So I am confused by the contradiction. Are you saying that you think traction could indicate something about brainstem kyphosis in the context of horizontal but not vertical instability? Or just that the effects can be salutory for CCI but not specific enough to CCI to indicate something about the pathology ?

 

[Guest 2176]

Here are a collection of summaries of where j addressed various questions re anatomy in the other thread.

In addition that paper provides justification for setting the range of the cxa how the consensus statement has set it, both from some kind of modelling of neural stretch injury based on angle, and also from the study on patients who have had their cxa surgically corrected.

(The Henderson one i posted above)

Abstract
There is growing recognition of the kyphotic clivo-axial angle (CXA) as an index of risk of brainstem deformity and craniocervical instability. This review of literature and prospective pilot study is the first to address the potential correlation between correction of the pathological CXA and postoperative clinical outcome. The CXA is a useful sentinel to alert the radiologist and surgeon to the possibility of brainstem deformity or instability. Ten adult subjects with ventral brainstem compression, radiographically manifest as a kyphotic CXA, underwent correction of deformity (normalization of the CXA) prior to fusion and occipito-cervical stabilization. The subjects were assessed preoperatively and at one, three, six, and twelve months after surgery, using established clinical metrics: the visual analog pain scale (VAS), American Spinal InjuryAssociation Impairment Scale (ASIA), Oswestry Neck Disability Index, SF 36, and Karnofsky Index. Parametric and non-parametric statistical tests were performed to correlate clinical outcome with CXA. No major complications were observed. Two patients showed pedicle screws adjacent to but not deforming the vertebral artery on post-operative CT scan. All clinical metrics showed statistically significant improvement. Mean CXA was normalized from 135.8° to 163.7°. Correction of abnormal CXA correlated with statistically significant clinical improvement in this cohort of patients. The study supports the thesis that the CXA maybe an important metric for predicting the risk of brainstem and upper spinal cord deformation. Further study is feasible and warranted.

There's the abstract but I recommend reading the full paper.

The same paper discusses cranial settling and something called "functional cranial settling" that I don't quite understand the difference between that and cranial settling.
Here's an example: "One large series reported “kinking of the medulla” (kyphotic CXA) in 140 of 364 of Chiari patients [111]; these were recognized as a form of “functional cranial settling” [110] in one series, and cranial settling or basilar invagination occurred in 25–30% of cases of Chiari malformation [26]. Kim et al. described “nontraditional basilar invagination” as the underlying cause of recurrence of pain and disabling symptoms in Chiari I patients after conventional suboccipital decompression, and reported substantial neurological improvement after intraoperatively correcting the CXA from an average of 127° to 147° [81]. Functional cranial settling, such as occurs with connective tissue disorders [110], may invite inordinate aggregate translation and flexion and subsequent basilar invagination with compression of the spinal cord or medulla [53, 54, 61, 64, 65, 116, 165]."

I'd like to dive further through the references to understand cranial settling Better though , but it may be above my pay grade

So it definitely doesn't seem like Henderson is the first to discuss cranial settling in the context of connective tissue disorders.

 

[Guest 2176]

So, the cranial settling anatomy is something that I can't speak to. But if many of the people with a CCI diagnosis have a kyphotic cxa I don't see how it is somehow unbelievable that traction would tell us something about their symptoms , and mimic the effect of a fusion

In fact that same Henderson paper has detailed description and diagrams indicating the use of intraoperative traction, and showing how it corrects a kyphotic cxa.