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

[Hoopoe]

Ron Davis on CCI: he talked with physicians that specialize in ME/CFS and they said it's only a fraction of patients that may have this. There is a problem because if only some have it, why are the two patient populations the same [I think he means in terms of symptoms, history, etc]. Something that is consistent with CCI is that in the severe patients some of the highest metabolites in the blood are lysine and hydroxyproline which are major constituents of collagen and that would be consistent with degradation of collagen. It's not proof that this is occurring, it could be the opposite as well, with the body increasing production of collagen. Either way this indicates there is something with connective tissues and they have to sort that out.

 

[JenB]

he talked with physicians that specialize in ME/CFS and they said it's only a fraction of patients that may have this

It is definitely a fraction but at this stage, I don't think anyone knows how large or small a fraction it is, including ME/CFS clinicians. This was not something they ever assessed their patients for in the past, so I don't know how they could possibly know.

 

[JenB]

So how do you respond to my comment above @JenB - that in fact there is no overlap in the discriminatory features?

These are not the definitions in 'the literature'. They are definitions in fringe publications as far as the medical community is concerned, and everyone knows that.

What I recommend as an alternative is

what is in the generally accepted literature. CCC and IOM are not bad for ME. The features CCI have little to do with the speculative 'cervical medullary syndrome'.

I don't want to lecture but I think it is important not to give the impression you are quoting accepted medical evidence when it is in fact highly controversial material from a few people with a very strong financial conflict of interest.

And the neurosurgeons?

Sorry, which definitions are fringe? I’m not sure if you are talking about certain ME definitions or descriptions of CCI?

Re: CCI, my Venn Diagram does not use the the CMS symptom list. It uses the symptoms on this page, which draw on a variety of sources: https://www.me-pedia.org/wiki/Craniocervical_instability#Symptoms I expressly tried to avoid touching dysautonomia! I completely understand that it might be difficult to arrive at a coherent picture/agreement of what constitutes a CCI symptom. I do not believe such a list exists.

If localising neurological symptoms PWME are attributed to ME because of ICC criteria, that is a good reason for ditching the criteria. There is no justification for attributing local neurological symptoms to ME.

This is not a bad argument. And a lot of people think the ICC runs the risk of picking up a wide range of additional diagnoses because of the sheer number of symptoms it includes.

However, Ramsay did describe some “localizing” neurological symptoms in his 1990 piece summarizing the most common symptoms:



And you see many of these symptoms (along with a couple of others) described in several of the pre-1980 outbreaks. I know you have previously said that the outbreaks have nothing to do with present day ME (per se) but I still think this context is important. For better or worse, there is more overlap between the ICC and Ramsay/outbreak descriptions than between the outbreaks and the CCC.

I agree that both the IOM and the CCC by and large do not include these symptoms. I did not do a CCC Venn Diagram simply because so much of the patient community discussion has been a around the ICC and/or Ramsay. However, it’s clear that the CCC is the strong preference of both the clinical and research communities.

If nothing else, perhaps this discussion can help to inform how to better define this diagnosis.

 

[Dx Revision Watch]

It is definitely a fraction but at this stage, I don't think anyone knows how large or small a fraction it is, including ME/CFS clinicians. This was not something they ever assessed their patients for in the past, so I don't know how they could possibly know.

Well, according to Dr Ilene Rohuy's blog post that you have yourself flagged up on Twitter:

"A subset of the population of those that suffer with hypermobility, mast cell activation, and dysautonomia will develop what has become to be known as myalgic encephalomyelitis (ME) and/or chronic fatigue syndrome (CFS). A certain percentage of these patients develop worsening and progressive symptoms due to cranio-cervical instability (CCI) or atlanto-axial instability (AAI). This instability is thought to cause a microshearing of the cranial-cervical junction where the medulla of the brainstem becomes the spinal cord top, thereby disrupting neural pathways that contribute to the patient’s symptoms and, in fact, making other forms of treatment either ineffective or suboptimal.

. . . I will be joining this neurosurgeon during intensive pre-op evaluation as well as the surgery that has helped many in the ME/CFS community begin to heal."

(NB: No references for these statements provided.)

 

[Hoopoe]

I agree that both the IOM and the CCC by and large do not include these symptoms. I did not do a CCC Venn Diagram simply because so much of the patient community discussion has been a around the ICC and/or Ramsay. However, it’s clear that the CCC is the strong preference of both the clinical and research communities.

Remember that diagnostic criteria are not supposed to be a complete description of an illness. The diagnostic criteria for multiple sclerosis for example do not list symptoms but focus on a pattern of brain lesions and attacks.

This point is I think important because the misconception that diagnostic criteria are a complete description will lead to patients feeling misunderstood, and to the impression that the various diagnostic criteria describe maybe very different illnesses.

 

[Jonathan Edwards]

Sorry, which definitions are fringe? I’m not sure if you are talking about certain ME definitions or descriptions of CCI?

ICC and Ramsay are fringe medicine. No reasonably intelligent well trained physician takes them seriously. Various descriptions of the clinical picture of CCI by private neurosurgeons are now regarded as fringe by neurologists and other physicians with an interest in necks. I met another academic neurologist yesterday who confirmed the general concern about these descriptions - which go beyond CMS.

I completely understand that it might be difficult to arrive at a coherent picture/agreement of what constitutes a CCI symptom. I do not believe such a list exists.

With due respect, Jen, you seem to have still completely missed the point made both by me several times before and Trish recently, that making a diagnosis is not about a list of symptoms. We know exactly which clinical features can be used to reach a diagnosis of CCI and they are all localising neurological symptoms or signs. They do not include neck pain or fatigue or tinnitus or visual disturbance (the last two are localising neurological features but in the wrong anatomical area).

As I said before, all the clinical features used to make a diagnosis of CCI are local neurology, none of the features that should be used to make a diagnosis of ME are. What could be simpler?

If you want to know if a pet is a cat or a dog you do not count the legs. There is no point in pointing out that a dog might be a cat because it has four legs. Nobody diagnoses dogs that way. I am afraid that there is a huge amount of disinformation going around in this area, typified by the post by Dr Rohuy. And I guess that we may agree that the point all this raises is that physicians dealing with ME may have contributed to that with ICC.

However, Ramsay did describe some “localizing” neurological symptoms in his 1990 piece summarizing the most common symptoms:

And everyone in academic medicine is agreed that Ramsay's account is of doubtful validity and of no relevance to ME in general. Any localising neurological feature is a pointer to an alternative diagnosis. PWME rightly complain that doctors put everything down to their ME, whether they believe it or whether they are trying to avoid further tests, when what they ought to be doing is considering something else.

And whatever may have happened in some outbreaks of viral illness in the past there is no reason to try to shoehorn people with ME into a pattern that may well have been idiosyncratic.

I did not do a CCC Venn Diagram simply because so much of the patient community discussion has been a around the ICC and/or Ramsay.

A few people on the internet like discussing ICC and Ramsay but I don't see how that makes them the choice for a serious discussion of medical management of ME when the medical community has move on.

 

[Guest 102]

Sorry, which definitions are fringe? I’m not sure if you are talking about certain ME definitions or descriptions of CCI?

Re: CCI, my Venn Diagram does not use the the CMS symptom list. It uses the symptoms on this page, which draw on a variety of sources: https://www.me-pedia.org/wiki/Craniocervical_instability#Symptoms I expressly tried to avoid touching dysautonomia! I completely understand that it might be difficult to arrive at a coherent picture/agreement of what constitutes a CCI symptom. I do not believe such a list exists.



This is not a bad argument. And a lot of people think the ICC runs the risk of picking up a wide range of additional diagnoses because of the sheer number of symptoms it includes.

However, Ramsay did describe some “localizing” neurological symptoms in his 1990 piece summarizing the most common symptoms:

View attachment 9343

And you see many of these symptoms (along with a couple of others) described in several of the pre-1980 outbreaks. I know you have previously said that the outbreaks have nothing to do with present day ME (per se) but I still think this context is important. For better or worse, there is more overlap between the ICC and Ramsay/outbreak descriptions than between the outbreaks and the CCC.

I agree that both the IOM and the CCC by and large do not include these symptoms. I did not do a CCC Venn Diagram simply because so much of the patient community discussion has been a around the ICC and/or Ramsay. However, it’s clear that the CCC is the strong preference of both the clinical and research communities.

If nothing else, perhaps this discussion can help to inform how to better define this diagnosis.

View attachment 9341

Can I just point out that PEM is a more recent description, Ramsay never spoke about PEM, rather fatigability on trivial exertion and ‘feeling awful’

So I do not think PEM should strictly be on the 1930-1980 outbreak part of the Venn.

Also, FWIW, as someone who actually got ill during a Coxsackie outbreak in early 1980s in west Scotland, in Helensburgh/Dumbarton region, documented by Ramsay, I doubt very much consultant neurologist Behan had to rule out tethered cord, in my own case, at least. I had no indication at all of having it.

 

[Guest 102]

ICC and Ramsay are fringe medicine. No reasonably intelligent well trained physician takes them seriously.

And whatever may have happened in some outbreaks of viral illness in the past there is no reason to try to shoehorn people with ME into a pattern that may well have been idiosyncratic.

So what do you make of my 36 year long illness?

 

[Mij]

I just want to point out from having this illness for almost 30 years that many of these symptoms are transient. I did not experience PEM for many years. I went through a period when it was difficult to hold my head up, neck pain, eye twitching etc

@JenB are there ME patients that you know of without POTS that meet the CCI criteria?

 

[Jonathan Edwards]

So what do you make of my 36 year long illness?

I don't know anything about your illness but my understanding is that ME/CFS goes on long term for the majority. I am not sure what that has to do with ICC or Ramsay.

 

[JenB]

I just want to point out from having this illness for almost 30 years that many of these symptoms are transient. I did not experience PEM for many years. I went through a period when it was difficult to hold my head up, neck pain, eye twitching etc

@JenB are there ME patients that you know of without POTS that meet the CCI criteria?

I just want to reframe slightly so we’re not confusing ME clinical diagnostic criteria based on symptoms with a CCI diagnosis (where CCI has no set symptoms).

@Hip is in a better position to answer this question because he has actual data. Hip, are there people in your survey who tested positive for CCI who don’t have POTS?

I personally don’t know of anyone with CCI who didn’t have POTS but that doesn’t mean that there aren’t any. It’s not something I’ve been particularly focused on.

 

[JenB]

So I do not think PEM should strictly be on the 1930-1980 outbreak part of the Venn.

Ramsay doesn’t which is why it isn’t in his part of the Venn. However, many different clinicians wrote about the outbreaks they observed and several of them described something that I think can rightly be called PEM. See the symptoms here: https://www.me-pedia.org/wiki/Epidemic_myalgic_encephalomyelitis#Symptoms with references to the original sources.

Also, FWIW, as someone who actually got ill during a Coxsackie outbreak in early 1980s in west Scotland, in Helensburgh/Dumbarton region, documented by Ramsay, I doubt very much consultant neurologist Behan had to rule out tethered cord, in my own case, at least. I had no indication at all of having it.

The type of tethered cord we see in hEDS and in pwME getting diagnosed with tethered cord is typically of the “tight filum” variety (i.e., occult tethered cord). I have definitely had subtle symptoms since I was a child but never knew I had them. I only started having overt symptoms after my viral onset. This is a theme I’m seeing in some patients: asymptomatic (or subclinical) variations in human anatomy that only become problematic after a massive inflammatory event. At least, that’s one hypothesis.

Tethered cord has some pretty obvious symptoms like frequent urination (although sometimes lack of urination); leg pain, weakness or numbness; sexual dysfunction; inability to lift heavy objects (specifically above a certain height); pulling sensation in your spine; spine stiffness; abnormal gait. Symptoms can come and go. If you are very symptomatic, it’s pretty clear. Also, everything gets worse if you walk on your heels. (If you are reading this DO NOT ATTEMPT TO DO THIS.) So if you never had the symptoms, then you’re right that you have no indication of having had it. It’s also right that Behan would have no reason to suspect tethered cord might play a role in an outbreak. However, tethered cord was not a condition that was at all well-recognized until recently. Now it’s getting diagnosed in newborn infants who show signs and you have neonates getting surgery. I’m not sure Behan would necessarily have recognized it even if someone had it.

 

[JenB]

With due respect, Jen, you seem to have still completely missed the point made both by me several times before and Trish recently, that making a diagnosis is not about a list of symptoms.

As I said before, all the clinical features used to make a diagnosis of CCI are local neurology, none of the features that should be used to make a diagnosis of ME are. What could be simpler?

Of course. Especially not a diagnosis like CCI. The point of the exercise is to show why patients with tethered cord and/or CCI could be diagnosed with ME/CFS by ME/CFS clinicians if they also have PEM. There is enough overlap in symptoms for ME/CFS clinicians (who are generally not neurologists) that when they look at people like us they don’t say, “Oh, that’s a totally different animal.” Or, “I am worried about these localizing symptoms. I think you need further investigation/imaging for those symptoms before I can give you a diagnosis.”

The Venn diagram isn’t about making a diagnosis. It’s about whether a patient presenting with “localizing” symptoms would raise a red flag for ME/CFS clinicians. Apparently, they don’t. Maybe all this will change that. That would be a very good thing!

We know exactly which clinical features can be used to reach a diagnosis of CCI and they are all localising neurological symptoms or signs. They do not include neck pain or fatigue or tinnitus or visual disturbance (the last two are localising neurological features but in the wrong anatomical area).

I had tinnitus and it also resolved with surgery. Tinnitus I got from these publications:
https://search.proquest.com/openvie...c50273d68313/1?pq-origsite=gscholar&cbl=47886
https://pdfs.semanticscholar.org/21c4/85984a6ebe07efed38cf82a2f7a49b2a644e.pdf

I am not sure why neck pain is controversial or even fatigue. You can experience fatigue from almost anything that affects your spinal cord enough. I say this having experienced all kinds of central fatigue from spinal cord issues (distinct from PEM).

Any localising neurological feature is a pointer to an alternative diagnosis.

What do you make of the radiculopathy and ulnar neuritis? https://www.me-pedia.org/wiki/Epidemic_myalgic_encephalomyelitis#Findings (the latter can be a little fuzzy https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3908974/)

 

[Jonathan Edwards]

Tethered cord has some pretty obvious symptoms like frequent urination (although sometimes lack of urination); leg pain, weakness or numbness; sexual dysfunction; inability to lift heavy objects (specifically above a certain height); pulling sensation in your spine; spine stiffness; abnormal gait.

How does anyone know these are symptoms of a tethered cord Jen?
To know that symptoms are caused by a particular pathology you need evidence. I think it highly unlikely that anyone has evidence for these being symptoms of tethered cord.

 

[Jonathan Edwards]

Of course. Especially not a diagnosis like CCI. The point of the exercise is to show why patients with tethered cord and/or CCI could be diagnosed with ME/CFS by ME/CFS clinicians if they also have PEM.
...

The Venn diagram isn’t about making a diagnosis.

I am afraid I have lost the thread of your argument now Jen.
People with PEM get diagnosed as ME because PEM is a feature of ME. If they have something else as well that should not interfere if it s symptoms are neurological.


Maybe we can agree that what the discussion really brings to light is the unsuitability of the ICC focus on neurological features when nobody has any evidence that ME/CFS causes local neurological deficits.

Lots of people have radiculopathy or ulnar nerve problems (I have both these days). If you were to gather together 100 physicians and present them with the historical literature and ask if they thought that it was reasonable to attribute local nerve lesions to ME, I think 99 would say no. The syndrome recognised by researchers and clinicians as ME/CFS does not include these things.

 

[JenB]

How does anyone know these are symptoms of a tethered cord Jen?
To know that symptoms are caused by a particular pathology you need evidence. I think it highly unlikely that anyone has evidence for these being symptoms of tethered cord.

I mean, I think this is basic info on many websites. I can dig into the literature but this is from the NIH for starters:

Tethered cord syndrome is a rare neurological condition. The severity of the condition and the associated signs and symptoms vary from person to person. In some cases, symptoms may be present at birth (congenital), while others may not experience symptoms until later in adulthood. Features of the condition may include foot and spinal abnormalities; weakness in the legs; loss of sensation (feeling) in the lower limbs; lower back pain; scoliosis; and urinary incontinence.

https://rarediseases.info.nih.gov/diseases/4018/tethered-cord-syndrome#diseaseOverviewSection

And from NORD

The most important signs that can be found in late teenagers and adults are back pain aggravated immediately on flexion of the lumbosacral spine, which elongates the lumbosacral spinal canal, simultaneously stretching the lower spinal cord. The 3-Bs sign is useful for initiating suspicion of tethered cords syndrome. They include 1) bending slightly (over the sink), 2) Buddha sitting with legs crossed (like the Yoga pose) and 3) Baby holding (or equivalent weight) at the waist level.

https://rarediseases.org/rare-diseases/tethered-cord-syndrome/

Are there specific symptoms you are skeptical about? Or skeptical that we know enough about tethered cord to identify symptoms?

 

[JenB]

I am afraid I have lost the thread of your argument now Jen.
People with PEM get diagnosed as ME because PEM is a feature of ME. If they have something else as well that should not interfere if it s symptoms are neurological.

All of these people diagnosed with ME/CFS who have subsequently been diagnosed with Chiari, CCI/AAI, stenosis, IIH, Eagle’s, tethered cord, etc. do have PEM. (By definition and by self-reported fact.) What I am saying is that there is a tendency for physicians (including ME/CFS specialists) to observe “localizing symptoms” that began after onset, alongside all the other symptoms, and say, “Yep, that’s your ME/CFS, too.” Or to think that sending you for a brain MRI to rule out MS was the extent of the neurological workup you require. I have had “localizing” symptoms since my onset and reported them to ME/CFS doctors. No one ever said, “That doesn’t sound like ME.” Or “you could have something else in addition, you need to get that worked up.” A part of the reason for this (I suspect, hence the Venn diagram) is that some of these symptoms overlap with what they routinely see in their patients, and so they don’t jump out at them.

Conversely, if you go to neurologists and report your whole bucket of symptoms, they are unlikely to see the “localizing” symptoms and isolate them and diagnose them for what they are. Throw PEM, POTS, MCAS on top of CCI/AAI, IIH, TC symptoms and they are likely to think you have a somatoform disorder. Say it all began after a viral infection and you are likely to confuse them some more. Who could possibly have that many symptoms or that many diagnoses at once? All beginning at more or less the same time?

Maybe we can agree that what the discussion really brings to light is the unsuitability of the ICC focus on neurological features when nobody has any evidence that ME/CFS causes local neurological deficits.

I don’t want to comment on specific criteria at this stage but I do think it’s worth asking how these symptoms ended up in the criteria in the first place. Clearly some clinicians are observing these symptoms in their patients. It’s important to figure out why. (Even if the answer is that we have major problems of differential diagnosis.)

 

[Jonathan Edwards]

I mean, I think this is basic info on many websites. I can dig into the literature but this is from the NIH for starters:

So where does the evidence-base for that information come from?

Medicine seems to have moved into a commercial phase where anyone wanting to sell a treatment can invent a syndrome to treat. And maybe it isn't such a new thing. For about a century surgeons invented chronic tonsillitis and did tonsillectomies for no good reason. People with rheumatoid arthritis had their teeth removed for 'focal sepsis'. Maybe there was just a window in the 1980s-90s when quality of evidence was taken as important.

I do keep trying to make the point but maybe need to keep making it. So much of medical information is bullshit, no matter whether it comes from an osteopath in Bristol or the NIH or Mayo.

I make statements that I think I can justify on the basis of decades of practical experience tempering what I read. I urge you to step back and realise that a lot of what you are quoting may be nonsense.

 

[Jonathan Edwards]

All of these people diagnosed with ME/CFS who have subsequently been diagnosed with Chiari, CCI/AAI, stenosis, IIH, Eagle’s, tethered cord, etc. do have PEM.

So presumably they have ME/CFS since PEM is not a feature of the other disorders.

 

[Jonathan Edwards]

The NIH note on 'tethered cord' is interesting in that it only gives two categories:
In some cases, it may be the result of improper growth of the neural tube during fetal development, which is closely linked to spina bifida. Other potential causes include narrowing of the spinal column (with age), spinal cord injury, tumors, and infection.

Spina bifida clearly involves tethering and distortion and there are radicular and/or cord problems like urinary incontinence but why hnot just call it spina bifida?

The other examples are not actually tethered cord but compressed cord syndromes - again you might as well call them what they are.

It looks to me as if NIH have felt the need to have a note on 'tethered cord' because it comes up in the literature and have cobbled together a note that does not add up to much other than spina bifida and cord compression.