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

@Hip I think we have talked about this before but within weeks of getting ill with this my gums receded massively. I remember thinking all my teeth where going to fall out if it continued but it leveled off and remained roughly the same since.

Just gona throw out this as well. My lower back and lower neck start to ache at late afternoon / night. Now this could well be because I have been upright all day but this also coincides with a change of symptoms. I generally feel better at this time of day, more so than any other time.

 

Just on the joint laxity thing, I’ve definitely felt like my joints are more lax since being ill. Often feels like the muscles aren’t strong enough to support the joint properly so you get a lot of movement that you shouldn’t have. Maybe it could be due to deconditioning, maybe something else. Would be interested in other members’ experiences, particularly at the severe end where deconditioning is likely to come into play.

 

then there was this tethered cord, in 2 out of 3 patients (so far, faik)

it seems a consequence of the surgery, rather than a previous overlooked thing.

what were the symptoms that lead to the discovery of the tethered cord ?
when did they start ?

 

Hi @JenB,

Since you plan to write more blog posts on Medium about your CCI diagnosis and treatment, is it possible to add a disclaimer at the beginning of those articles as suggested at the beginning of this thread?

Many thanks in advance,

Kind regards.

Michiel.

 

Jonathan, however implausible, can you conceive of any possible way in which CCI could cause ME symptoms in some people without it having been noticed by those specialising in CCI?

As I don’t know enough about anatomy or medicine, I’m trying to think about this from a purely logical perspective. How could CCI cause ME symptoms without it ever having been noticed by CCI specialists? If CCI caused ME symptoms, why would none of the CCI patients they saw ever describe having ME type symptoms, or being relieved of them following surgery?

If there was a sub-group of people for whom CCI resulted in predominantly ME symptoms rather than known CCI symptoms then it seems likely that they would not be seen by CCI specialists, and that the association could therefore have been overlooked. But how could there be a sub-group of people whose CCI results in such different symptoms? Is it conceivable that these people could have some physiological difference which results in a different response to CCI? Is it conceivable, for instance, that a mild form of CCI, which might not be severe enough to cause classic CCI symptoms, could, for some unknown reason, perpetuate ME symptoms (which may have been triggered by something else either before or after the CCI)? Are there any other possible scenarios, however improbable?

I’m not trying to you coax you into making wild speculations about things which you consider to be highly unlikely to be true – I’m really just trying to ascertain whether “totally implausible” allows room for any highly improbable possibilities in your mind.

 

Returning to my comment about the number of patients in our ME support group – I was thinking about this on my slow amble with an elderly dog around the block, and realized that I should have been more mathematically explicit.

Members of our support group do not represent the ME community. Those who are mildly affected (and that's a pretty foul way to describe people who are very ill and struggling to survive) will be doing everything they can to earn a living, so are unlikely to become members. Those who are severely affected are unable to attend meetings, even if it is "only" for a coffee, so again are unlikely to want to become members. I'd be surprised if we represented even a half the ME community. So if 1/500 have a diagnosis of ME, we probably at best only represent 1/1000 at best.

With only 30 members in the group, and with only 3 with EDS, the figure of 10% is very nebulous: we can't extrapolate from that. The numbers can draw attention to a possible problem, but cannot sensibly be used in a calculation.

The prevalence of either ME or EDS rely upon the diagnosis of these conditions from a small number of specialists, and a handful of studies. The reliability of these figures is very poor, particularly with no agreed set of criteria or diagnostic test. (Diagnostic tests have to be interpreted and are much less reliable than the public would like to believe, but with ME we have nothing at all.)

If I were to use the appropriate variety of values for each of these, the results would be wildly different.

But the biggest problem by far would be caused if the numbers of people with EDS were underestimated and the number with ME were overestimated because, like the three members of my group, their first diagnosis was ME rather than EDS, and this simply meant that no further options were considered. This would not have a dramatic effect on the prevalence of ME (which I think is very unreliable anyway), but it would have a significant effect on the estimate of numbers with EDS.

Is it possible that having EDS could result in someone being misdiagnosed with ME? Probably not if they were first sent to someone specializing in EDS, but if they were sent to an ME centre, I have much less confidence that it would be spotted. (Remember that there are areas of the country not covered by such centres, that a proportion of such centres do not have a specialist, and that those that do have specialists have them often from a psychological background). In addition, people with EDS but not yet experiencing problems are just as likely as anyone else (or more likely?) to go down with ME and have that diagnosed first.

Although we can speculate about whether EDS and ME have some form of link, or whether ME could cause similar problems in the neck, it seems to me that the big issue to be addressed is the probability of misdiagnosis.

My friend's GP put in a request for her to see one of the EDS specialists: the funding authority refused it. If access to such specialists is limited, again, how reliable are reports of prevalence and of misdiagnosis?

She did see him privately. He saw the Medserena scan (again that had to be done privately) and commented that the flexion and extension was one of the highest he had seen in EDS.

I wonder why those of us with ME have such mistrust of the system?

 

g

Jonathan, hEDS is not simply hypermobility plus pain and fatigue.

The NHS Choices website page on EDS says:
"People with hEDS may have:

• joint hypermobility
• loose, unstable joints that dislocate easily
• joint pain and clicking joints
• extreme tiredness (fatigue)
• skin that bruises easily
• digestive problems, such as heartburnand constipation
• dizziness and an increased heart rate after standing up
• problems with internal organs, such as mitral valve prolapse or organ prolapse
• problems with bladder control (stress incontinence)"

I know people who have a diagnosis of hEDS. Their pain is very definitely related to their hypermobility - their joints are repeatedly coming out of place and causing them great pain.

 

No, totally implausible is totally implausible.

I need to explore some further arguments when I have time but I have been busy in the last 24 hrs.

I agree with Michiel that it would help for Jen B to add a disclaimer, or preferably stop posting about these things until we have some evidence that makes some sense. I am getting very concerned that people are walking into a massive scam.

Immobilising the neck may put ME into remission but that does not mean there was ever anything wrong with the neck and immobilising necks can kill people.

 

Dear @Sarah94,

I don't want to seem unreasonable but just in case you had missed it.

I was involved in the setting up the hypermobility research programme in the 1970s that led on to the new term 'hEDS'.

I have looked after a large number of people with these diagnoses.

When Rodney Grahame, who has been the undisputed central figure in 'hEDS' in the UK since 1980, was due to retire from his hypermobility clinic in 2000 I was chosen as the most appropriate person to take over the clinic because of my expertise. I declined because I did not think there was anything useful to be achieved by such a clinic.

I know as much about the history of 'hEDS' in the UK as almost anyone. I don't think it is a helpful diagnosis. There are certainly people with recurrent dislocations but most of them have local idiosyncratic laxity problems in specific joints. Adding the term hEDS does not alter the management. There are a very small number of people with specific gene types who have generalised laxity but I think they would better be described by their gene type than by EDS.

 

@Graham,

I agree with your caveats about the maths but I think my original point stands. Maybe I was not clear.

The whole business of the link between 'EDS' and ME is shrouded in mystery. The ME research community is snatching around for any tiny clue yet there appears to be an elephant in the room in the form of a link with a genetic connective tissue condition that if real would provide a massively powerful lead for starting research. But as you say, there are no reliable data.

There are three groups of people who might be said to have 'EDS' with symptoms of ME. The first would be people with recognised genetic variants - now identified in most of the extra-articular EDS types. The puzzle is that several genetic screening studies of PWME have now been done and there is no mention of these EDS gene variants coming up. The only gene found recently was a prolyl hydroxylase 4 variant. PH4 is involved in collagen synthesis but it is not an EDS gene so it does not support a link to EDS. We have nearly 2000 members here and my impression is that not more than maybe two or three families are documented with EDS gene variants. Moreover, these variants will be quite a bit commoner than the EDS type itself if they are recessive or partial penetrance.

The second group would be those with clinical signs of extra-articular EDS type - like the woman in the photo with skin folds or someone with cardiac defects. My impression is that these extra-articular types are not particularly associated with ME though. The tales in the literature are of a link to type III (articular) EDS or 'hEDS'. There have never been any threads here on what to do about your skin folds or mitral valve disease. From what I can glean it seems likely that maybe a handful of people here may have extra-articular EDS features but even there I am not sure. So many of these things are common enough in the general population anyway.

The third group are those with hypermobility, who if they have chronic pain and fatigue will be diagnosed as EDS by private physicians with 'an interest in EDS'. The sad thing is that pretty much all private physicians with an interest in EDS have followed the makeshift approach to diagnosis that I decided not to get involved in. What Jen Brea calls the 'EDS space' is almost entirely populated by physicians who do not really know what they are talking about - like the Swedish people we have seen on a recent thread.

I have developed huge respect for PWME, and their intelligence and tenacity in trying to get at the truth. ME is a real illness that destroys lives. I can see no plausible reason why any form of EDS should cause the core symptoms of ME and what I can see of the story of a link between EDS and ME looks more like a phantom than an elephant. There are no data worth taking seriously.

Again I must say that I think it entirely plausible that neck fixation may induce remission in ME but that is not necessarily anything to do with CCI or EDS or anything similar. The current situation worries me greatly but maybe it will lead us to a genuinely useful understanding if we continue to argue it through. After all, there must be a truth in there somewhere.

 

Is it at all plausible that neck pain could come from poor posture, which in turn comes from weakness? In the sense that we are exhausted and don't have a good posture because of that. Or much of a posture at all if we lie down a lot.

This is relevant because if PWME have neck pain they now might be wondering if they something like CCI.

That said I find it interesting that I have hypermobile shoulders. Which are anatomically right next to the neck. There is actually a subtle divide between my upper body which is slender and mobile and my lower body which is fatter and stiffer.

 

I had neck pain which got so bad I was having to spend much of my time lying in bed with blinding headaches.

I got the chance to go to a session of aromatherapy massage. She said that the muscles in my neck were solid and she could not get my neck to move from side to side. For myself, afterwards it felt like a tight button on a shirt had been undone. i carried on with the sessions and after months she was able to get my neck to have full mobility.

I have carried on and the pain has never returned. I think it was caused by using a wheelchair where you are always straining to look up and look round - I have to be careful even now but I now recognise the pain when I stretch my neck too far. Also my muscles are always wanting to stiffen up. I feel I would curl up like a hedgehog if I did not keep deliberately relaxing them.

Anyway, my problems were muscular, directly from the ME and as a consequence of the ME, but the symptom was severe neck pain.

 

@Jonathan Edwards

I think I fit into both your groups 2 and 3 of people who may have EDS but with symptoms of ME.

I have mitral valve prolapse and regurgitation, diagnosed in 2016. I was initially told I needed open heart surgery, but am being managed for the time being conservatively.
I also have a diagnosis of hypermobility, but not so far hEDS, although a NHS Consultant for yet another condition has suggested that they are all linked under a syndrome mentioning EDS including ME, and has referred me on his own initiative to a NHS geneticist. Whether the EDS issue will be resolved then I don’t know.


As far as I am concerned I have ME, diagnosed 2008 but probably existing mildly since 1986. PEM is my worst symptom as well as breathlessness and OI. I take betablockers which has reduced tachycardia. I think the biggest help would have been a full skilled assessment for dysautonomia. It is becoming increasingly hard for the cardiologists to know whether the breathlessness is linked to the heart or the ME.

I do not want to have a CCI diagnosis but have symptoms which could relate to neck instability, and of course I may have EDS which has a link.

It is all a terrible muddle.

As far as I am concerned I should have been properly physically assessed when diagnosed with ME. At the very least I should have been treated with respect and accurate knowledge by all the doctors to whom I was referred.
Instead two Consultants told me there was no such thing as ME, and a third that “ people with ME are mad but you are not mad”. His testing for POTS was completely inadequate so I was told I didn’t have POTS. It seems that his preconceptions about ME overrode the diagnostic process.


I’m pleased to say that all the doctors I see currently accept the existence of ME, and take it into account in my health history.

But it is still a terrible muddle.

eta: I may have been inaccurate in separating hypermobility and hEDS. I wrote from memory but this area seems particularly confusing. Need to read more.

 

Not wishing to get into the CCI/ME discussion I just want to thank you for these words above, Jonathan. They are powerful and actually very moving, to me, at least. Thank you.

 

Lying down with my head propped up looking at my tablet caused neck pain for 2.5 years back in 2005.

FIR sauna helped it disappear after 2 sessions.

 

Yes, I am thinking maybe I ought to try to do something about it. I am not that good at getting around to things like this but I have been pleased to notice that most times I say I might get round to something someone else pops up getting around to it - like the long actometry study. Still this time I might to try get some data to support what I have been saying - or not support it if that is the truth.

Mitral valve prolapse is quite common anyway. Which is why our study actually found the same rate in 'benign hypermobility syndrome' (now supposed to be called hEDS) people and controls.

 

This I find fascinating, fully acknowledging all the caveats and concerns of course. If neck fixation actually did induce ME remission for some people, then of course the $64k question would be by what mechanism? Would it necessarily be due to the neck actually becoming fixed? Or alternatively perhaps due to something else, done as part of the process of the surgery or aftercare? Purely as a thought experiment (obviously being much too risky / unethical / unevidenced in reality), would pwME not suffering from CCI/EDS, potentially still experience ME remission if they underwent the same process?

How could one ever go about trying to gather good evidence on something like this, beyond the current very limited anecdotal indications? Is it possible that stronger evidence potentially does already exist, but hidden, simply because no one has to date known to go looking for it amongst medical science archives?

 

That is the suggestion. But it might achieved with much less risk.

By careful analysis of all the steps in the scenario. The first thing is to evaluate the evidence for CCI in the first place. Another thing is to do a much more limited immobilisation procedure.

 

Jonathan,

EDS diagnostics have changed since then. See this document. https://www.google.com/url?sa=t&sou...rs-danlos.com/wp-content/uploads/hEDSvHSD.pdf

 

The emphasis being on the 'all' I imagine. I could imagine the slightly crazy, but not that crazy, possibility, that "the something" could be something not even formally specified to be part of the process, but that might nonetheless typically be done during most such procedures. Being non-medical I can't imagine what, but it must be impossible to pre-specify exactly everything that gets done in practice.