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

[Jonathan Edwards]

Dr. Edwards, you mentioned that ligaments more or less (I am paraphrasing here) either snap or are the way they are predestined by your genetics and that you doubt that that an inflammatory process could increase the laxity of this type of tissue.

I have to say I that my own experience (and that of a hundred patients with EDS that I spoke too, not exaggerating here) says otherwise, but I can absolutely not prove it, I also do not know if that process that changed my ligaments and or joint mobility is of an inflammatory nature, but I do know the process exists, such as there is a sudden worsening in my and other patients joint laxity after „crisis“ like infection and/or antibiotic use.

This is an observation that many EDS clinicians also made.

I am one of maybe no more than a score of musculoskeletal physicians worldwide who have made connective tissue structure, pathology and biomechanics a special interest. I used to write the chapters in textbooks on connective tissue physiology. I still sometimes get things wrong but the situation here is pretty clearcut I think.

Along the length of a ligament the maximum stretch is set by the collagen fibres - like a rope. Just like a rope you cannot lengthen it without damaging so many fibres that it tears. So we can discount inflammatory or enzymatic effects leading to ligament lengthening without snapping along their length.

The place where a ligament gets longer during growth, and yes, @Skycloud, during pregnancy a bit, is at its attachment or enthesis. There is a transition zone of cells that are neither bone cells nor ligament cells but sort of 'superglue cells'. In inflammatory diseases like rheumatoid arthritis this area can undergo change so that the bone at point of attachment is eroded away and the ligament may possibly lengthen. I say possibly because the normal mechanism for ligaments getting lax in inflammatory conditions like RA is something quite different. It is that the bearing surface of joints and discs are eroded away, making them loose in the way that a worn axle gets loose - the ligaments are actually unchanged.

The long and the short of this is that for basic physics reasons ligaments can only get laxer if bone or cartilage is removed either at the attachment or a bearing surface. (During growth the situation is different because the attachment is growing - paying out new ligament on demand. So during childhood ligaments can become lax through growth.)

Removal of bone or cartilage shows easily on an x-ray if it is enough to allow laxity. In RA and pregnancy we see the bone dissolved. In EDS as far as I know there has never been any suggestion of this. There would be no reason because the genetic basis is in defects in the structure laid down during growth. There is no suggestion of a genetic tendency to inflammation.

I do not know how to interpret a sense of increased laxity after an acute illness. The only thing I can think of is that if one loses weight the mobility of certain joints increases because they are naturally limited by muscle or other tissues (elbow flexion for instance).

I would be very surprised if real EDS specialists thought this could occur. But then I would distinguish EDS specialists in the sense of clinical geneticists, who by and large do not see hEDS because there is no gene, and the 'EDS specialists' who diagnose all hypermobility as 'hEDS' and associate it with widespread pain and fatigue. I have reason to think the latter mostly do not know anything much about connective tissues.

 

[Skycloud]

Interesting, thanks @Jonathan Edwards.

 

[butter.]

Very interesting, thank you!

What is the actual mechanism in ballet dancers when they do become more flexible due to stretching?

I will add this study, they are partly geneticists, if they are „the real thing“ is absolutely not for me to decide, after all, I am a baker.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335943/

I do absolutely trust your expertise insofar that what you say has merit, but I definitely trust my own experience and those of other patients more, why is that?

It is relatively clear cut, if your hands could not touch the floor at all and your joints did not subluxate before a certain factor like mentioned above and afterwards they did and you miracoulously are bendy like a yogi and this happened on 3 separate instances and many other people report the same, than it is more than just a coincidence. (not definitely but likely)

50 so called specialists told me that it is impossible to get sicker by moving my body and that there is no cfs, 99% these of all specialists would tell me the same thing at this point in time after being bedbound for 2 years.

I do not believe that you must have a genetic disease for having EDS like symptoms and pathology and that secondary factors could lead you there.

Also having talked to THE TOP EDS GENETECISTS, the picture is not as clear as many present it, there are reported cases with only heterogeneous mutations in any given EDS identified variant that still have the full picture of the disease! Interesting isn‘t it?

I am grateful for your input. ty.

 

[Jonathan Edwards]

What is the actual mechanism in ballet dancers when they do become more flexible due to stretching?

A very good friend of mine was once a soloist at the Royal Ballet. She and my wife go to open days at the ballet school to spot new talent. To have any chance of success ballet dancers have to start as children, often quite young. As far as I am aware all the flexibility they show is a combination of genetics (mobile children find ballet easy, non-mobile children give up early) and training the tissues during growth. I do not know of any data but it is likely that ligament lengthening during growth depends on use. We see excessive laxity into certain positions in some cases of infantile paralysis for instance.

Most ballet dancers that will go on to be professionals are identified for their talent by age about 12 and certainly age 15. I do not think there is any need to suggest that they get more flexible after that, once they have stopped growing.

I will add this study, they are partly geneticists,
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335943/

Also having talked to THE TOP EDS GENETECISTS, the picture is not as clear as many present it, there are reported cases with only heterogeneous mutations in any given EDS identified variant that still have the full picture of the disease! Interesting isn‘t it?

I think there may be a confusion here. In hypermobile people, some of whom are classified as hEDS, there is every reason to think the mobility is genetic (unless they have been to ballet school maybe). They do not have single gene mutations of a dominant type as in classical EDS. But that just means that their mobility is determined by several genes making a small contribution, with different ones contributing different amounts in different individuals. That is still genetic but not Mendelian genetic.

 

[JemPD]

Ok so following on from my previous post here

Perhaps there is something else going on, other than her having been misdiagnosed or CCI=ME?
Perhaps the act of having a CCI surgery cures ME (or something manifesting as similar to ME) but has nothing to do with the neck, or CCI directly?
From what i can glean, (in layman's terms) from @Jonathan Edwards & what I have read of Jen's story & reading on here generally...

a) CCI symptoms/clinical presentation generally bears no resemblance whatsoever to symptoms of ME.

b) Jen & the others were dx with ME using ICC/CCC - which means they did have symptoms of ME (or at least of what is currently seen as ME - ie resembling ME & not resembling CCI)

c) they developed new symptoms after an unconnected surgery (thyroidectomy in Jen's case) that were suggestive of the usual clinical picture/presentation CCI/tethered cord - hence it being looked for & discovered.

d) they get surgery for the CCI/TC

e) all symptoms remit.

That seems to me to suggest that either it was the most spectacular & unexpected placebo effect ever encountered, or something ELSE happened/was given to them as part of/in conjunction with the CCI surgery. Curing either their ME (or whatever they had that resembled it).

Because it doesn't seem logical to me, to

1) be very ill with an illness dx as ME (which may have easily been misdiagnosed but that involves no symptoms suggestive of CCI)
2) then develop symptoms of CCI, such that it is looked for, discovered & treated
3) in addition to the new symptoms caused by CCI, all your other symptoms from the previous illness disappear as well...

& then conclude that either the original illness wasn't what you thought it was & must have been CCI instead, or that CCI must have caused the original illness. Because the first illness bore no resemblance to the second & so while the first illness may not have been dx correctly, it had no cross over symptoms with the second so could never have been an appropriate differential dx that was missed.

It would be similar to having a stomach ulcer (in 1975 before H-Pylori discovery), then developing a cellulitis infection on your leg & being given antibiotic treatment for it, & happily discovering that your ulcer had disappeared at seemingly the same time as the cellulitis.

- The conclusion that either you never had an ulcer but rather your stomach pain must have been misdiagnosed cellulitis instead (which does not cause stomach pain), or that cellulitis is the true cause of stomach ulcers...
Neither conclusion really fits & you would be missing the big red flag that amoxicillin (or whatever) had cured your stomach ulcer. Of course when you reported that to yr Dr they would have dismissed it as being due to lowered stress levels or something silly but the real point would still be missed.

Personally I would like to see some serious study done into exactly what drugs were given: when, why, how much & in what order/combination. Transfusions? What position were they lying in, for how long, what kind of room were they in, did the nurses have their scrubs washed in a certain chemical... I don't know, but for all we know the answers could well lie in something that happened between entrance to the hospital for surgery & waking up after it. something completely unrelated & not imagined by anyone doctors or patients alike, it could be something so innocuous that it wouldn't even be remarked on.
Is anything unusual/unique that goes on connected with CCI surgery that isn't in other surgeries? Did the serious physical trauma of a fusion in conjunction with any of these things reset something in the system?

I cant imagine what it could be but that unknown 'something' is the only explanation that logically fits. In my mind anyway.

And now having caught up with what you said earlier @Barry & @Jonathan Edwards too....
here

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.

Barry responds to JE...

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?

would pwME not suffering from CCI/EDS, potentially still experience ME remission if they underwent the same process?

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

I wonder... Do people think - & I ask this mainly of @Jonathan Edwards since he is likely to be the one answering most of the questions if we did it, if it would be worth having a kind of brainstorming thread along the lines of 'what else could be causing the "CCI" remission"?'
I know it'd be unlikely for laymen to come up with the answer, but sometimes the less expert can spot something that's so obvious to the expert that they don't even notice it.

I dunno it's just a thought.

ETA I'm sorry I appear to have gotten confused & posted this in the wrong thread, we appear to be having almost the same conversation on 2 diff thread & i cant work out whats from where now.... it's the follow on from the thread about Jen's blog on medium I think, but i'm so sorry I am too fried & exhaust to work out how to move it all now it took agaes making all the quotes follow on properly I think I've mistakenly quoted from 2 threads at same time. I'm so sorry for the confusion, if any mod has time to move the whole post for me i'd be most grateful

 

[Jonathan Edwards]

I am not sure I have a lot more useful to say on this. Too many unknowns.

 

[JemPD]

I am not sure I have a lot more useful to say on this. Too many unknowns.

No worries it was prossibly a daft idea. Clutching at straws.

 

[Grigor]

What is the actual mechanism in ballet dancers when they do become more flexible due to stretching?

I do not know of any data but it is likely that ligament lengthening during growth depends on use.

I started at a quite young age but was rather on the stiff side. I'm more of a pirouette turner and high jumper. The dancers that are flexible fit more the hEDS picture. Actually many of my former colleagues do. It also makes me wonder where to draw the line between hEDS and healthy Über flexible dancers.

My hamstrings were always very short. I could only touch the floor with my hands if I was very warmed up but even then barely and only occasionally. They did get a bit more flexible at a later age but that felt more from training my big muscles rather than the tendons or ligaments. A bit the same way as beefing up but then in flexibility if that makes any sense. My tendons and or ligaments/joints however felt stiffer as I got older. So ligaments/joints wise I think Jonathan is correct!

 

[Keela Too]

As a student I found touching my toes nigh on impossible. I was fit, healthy and did all that ‘80s Aerobics stuff complete with leotard and leg warmers. I once decided that I “needed” to be able to touch my toes. It took me 6 weeks of daily stretching and effort to finally manage it. As soon as I stopped this I lost that ability.

Yet now in my 50’s after having ME for 7 years, I have found that I can touch my toes! (Okay I can only just touch them, but even trying to dab down on a sort of bounce I could never get even close before now.)

Why is that?

Is it that inactivity loosens ligaments etc? I certainly am much less active than previously, and my strength is much reduced too. Did those 3 years when I was walking only 500 daily steps have an effect? I would have thought that would have made me MORE inflexible, not less!

??

 

[Trish]

Maybe reduced muscle strength leading to less power in muscles that come into play to protect joints when you overstretch?

 

[Jonathan Edwards]

A very interesting question.

So to answer it we want to know what limits toe-touching.

Toe-touching, or more than that, palm to floor touching, is one of the criteria for hypermobility and is usually assumed to be a sign of lax ligaments. But toe or floor touching is, I suspect, almost entirely dependent on maximum hip flexion. Knee extension beyond straight ('swayback') would not help much. Spine flexion would help a bit but to really get down to the floor you really need your whole spine vertically downwards - i.e. to have more or less 180 degrees of hip flexion.

Now hip flexion is not determined by ligaments because the hip has no surrounding stabilising ligaments because it is a ball and socket. Its range is entirely limited by how much the muscles on the outer side of a movement can be stretched or how much muscles on the inner side get in the way. That may be why we can improve toe-touching with exercises but we cannot, for instance, increase knee or elbow extension (fortunately).

Spinal ligament range probably does play a part but I am pretty sure that being able to touch toes when one has been doing a bit of gym work has nothing to do with ligaments. And maybe if muscles are not trained mobility can actually increase because they do not get in the way so much.

 

[oldtimer]

It makes sense to me that the ability to put one's palms flat on the floor comes from the hips. I have always been able to do it easily. After a hip replacement less than a year ago and the other one probably not too far off, and quite advanced spinal degeneration, that hasn't changed.

I was an aerobics fanatic for years and for long periods have done no exercise at all. It never changed my palms on the floor party trick. Don't ask me to sit cross-legged though. Never could.

 

[Annamaria]

palm to floor touching, is one of the criteria for hypermobility

I could always touch my palms to the floor but have never been very flexible otherwise. Puzzling. Long legs and arms but comparatively short back.

 

[MandyME]

As a student I found touching my toes nigh on impossible. I was fit, healthy and did all that ‘80s Aerobics stuff complete with leotard and leg warmers. I once decided that I “needed” to be able to touch my toes. It took me 6 weeks of daily stretching and effort to finally manage it. As soon as I stopped this I lost that ability.

Yet now in my 50’s after having ME for 7 years, I have found that I can touch my toes! (Okay I can only just touch them, but even trying to dab down on a sort of bounce I could never get even close before now.)

Wow that is interesting. I've always had difficulty touching my toes. As a kid, after doing daily stretching, I could only touch them for a split second at a bounce! I tick every other box in the hEDS criteria for hypermobility plus hernia/prolapses/gut issues/slow healing sores and bruises/stretchy skin etc... I've never bothered to get a diagnosis as I don't think there are any local specialists educated in it and they'll just tell me to see a physiotherapist, who were the ones that suspect I have it in the first place.

I just tried to touch my toes on the off-chance I could touch them now, but nope! Although, I was surprised at how far I could go considering my tight hamstrings and sore back. I've had ME for 6 years, maybe I should try again in 12 months

But seriously, I find it really fascinating that you can now touch your toes Keela. And with the recent research mentioning collagen degradation, there might be something to it.

 

[Diluted-biscuit]

Wow that is interesting. I've always had difficulty touching my toes. As a kid, after doing daily stretching, I could only touch them for a split second at a bounce! I tick every other box in the hEDS criteria for hypermobility plus hernia/prolapses/gut issues/slow healing sores and bruises/stretchy skin etc... I've never bothered to get a diagnosis as I don't think there are any local specialists educated in it and they'll just tell me to see a physiotherapist, who were the ones that suspect I have it in the first place.

I just tried to touch my toes on the off-chance I could touch them now, but nope! Although, I was surprised at how far I could go considering my tight hamstrings and sore back. I've had ME for 6 years, maybe I should try again in 12 months

But seriously, I find it really fascinating that you can now touch your toes Keela. And with the recent research mentioning collagen degradation, there might be something to it.

I’ve had ME for over 25 years, I still can’t touch my toes.

 

[lansbergen]

I’ve had ME for over 25 years, I still can’t touch my toes.

Before I fell ill I could touch my toes. Now it is imposseble.

 

[Diluted-biscuit]

Before I fell ill I could touch my toes. Now it is imposseble.

This is why I’m staying sceptical about all this stuff.

 

[Mij]

I have always been able to put my palms on the floor with ease.

 

[ME/CFS Skeptic]

I have always been able to put my palms on the floor with ease.

Normally the test is without bending your knees.

(this was intended as a joke)

 

[Mij]

@Michiel Tack I meant without bending my knees