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

My main concern at the moment. Thank you!!

 

@Jonathan Edwards

Thank you for noting problem after effects - the downsides of this surgery. In my limited experience, some practitioners do not inform of downsides. Bony overgrowth causing nerve damage is not a small consideration.

 

As I understand it - and I should emphasise again that I have no medical background, so I'm very happy to be corrected if I've got the wrong end of the stick - 'cervical medullary syndrome' is a concept that has been drawn up by neurosurgeons dealing with CCI to describe and explain a symptom cluster that they frequently see in a subset of patients. It is - again, as I understand it - a concept that's in its infancy, having only been codified in the last 7 or 8 years, but it's drawn from their clinical experience and supported (or so I understand) by the published literature.

I'm happy to accept that the list is unhelpful and that the name is potentially misleading. I'm also happy to accept that the vast majority of neurologists (and other clinicians who deal with CCI) would not recognise this 'syndrome' as a good guide to the usual clinical picture of CCI. However from my point of view (which I acknowledge is only that of a layman) if the consensus view of a group of neurosurgeons who specialise in this area is that this is a recognisable group of symptoms which can accompany CCI, I think that's something that should be taken seriously and investigated further - even it doesn't fit with the historic and mainstream understanding of the condition.

I'm not saying this because I believe that cervical medullary syndrome and ME/CFS are definitely the same thing - I'm simply not in a position to make a judgement on that question - but because if there are overlapping symptoms then this seems (to me) to be an area worthy of further research, especially given Jen and Jeff's accounts of their symptom remission.

The former of the two - I've seen/read individual neurosurgeons give descriptions of cervical medullary syndrome which seem much closer to ME than the consensus statement.

 

Thanks @Stewart (sorry not Stuart),
It is useful to get your impression on the history.

The clinical picture of CCI has been around for 100 years. Every medical student is taught it. So it is interesting that neurosurgeons have been proposing a 'new syndrome' in the last 7-8 years. It is interesting and worrying because I think it is our old friend expectation bias coming in.

Why would neurosurgeons recently think they were seeing a new pattern of symptoms? I think much the most likely reason is because they are being referred people with that pattern of symptoms exactly because they have developed a reputation for being interested in that pattern. The 'syndrome' is self-fulfilling.

Maybe this is the place to add a b it of relevant history about 'EDS'.

At least in the UK until 1978 nobody was much interested in hypermobility. Then Rodney Grahame got interested. peter Rowe was also getting interested around that time or maybe a bit before. When we started the clinic at Guy's there was no symptom pattern recognised - we just collected people who were hypermobile. Then Rodney started talking about people with hypermobility having pain from it.

But how did he know that? The problem is that he only ever saw hyper mobile people referred to a rheumatology clinic. People are referred to a rheumatology clinic because they have pain.

Anyway, the story at that time was that the pain was due to the increased laxity in ligaments causing repeated low grade trauma. Rodney continued his hypermobility clinic for another forty years while I followed other things. Some time around 1990 the story changed. It seemed that people with hypermobility had pain all over - they seemed to have 'fibromyalgia'. They seemed to be hypersensitive to stimuli. It seemed that the pain could no longer be explained just buy the low grade trauma.

And the something else changed. Maybe around 2000 people started saying that people with hypermobility had chronic fatigue. Back in 1979 nobody had ever mentioned being fatigued. So why now? I am pretty sure because Rodney had gained a national reputation for seeing people with hypermobility and pain and more recently pain sensitivity and fibromyalgia and so he found his patients were fatigued - simply because those were the ones physios and doctors thought he was the expert for.

This continued to the extent that when Rodney retired those who had helped out in the clinic reported that, despite being a hyopermobility clinic, all the patients had the symptoms people lump under 'fibromyalgia'. In 1979 nobody had those symptoms.

I think Rodney may have held on to the 'hypermobility syndrome' term longer than some others but this group of patients are now often referred to as hypermobility type EDS or hEDS.

My strong impression is that the history of 'hEDS' is dominated by this circular self-reinforcing referral bias that creates whatever 'syndrome' the specialist says they are interested in. The promotional material I have seen on the net regarding CCI certainly looks that way.

So I think it highly likely that this new 'spinal medullary syndrome' is nothing more than the same self-reinforcing referral pattern. And it is not just that patients with these symptoms get referred. They see a physio who has heard of the syndrome and who encourages them to believe they have this syndrome so when they turn up at the neurosurgeons office they are another perfect case!

That might seem far fetched but, as I say, my experience with 'hEDS' in the UK is exactly that. When Rodney's clinic got overfull and the hospital admin insisted on the referrals being seen in a specified time the rest of us got a taste of his referrals. I got a few. They had been 'educated' by their physios to believe that they had pain due to hypermobility that required special management that only professor Grahame could provide.

 

big stuff, @Jonathan Edwards

so it works for mecfs, too... ?

 

No, I think not. Both doctors and patients get the syndrome of ME right before they ever heard of it. There were people with ME in 1979 (and neither they nor I knew it was called ME).

 

The idea that there is a kind of bias where people with the cervical medullary syndrome are finding their way to clinicians that believe in this syndrome strikes me as possible, but I also think that it’s equally possible that people with these neurological symptoms from instability have been ignored and then finally find doctors willing to take their symptoms seriously and treat them.

I think that the link between cervical medullary syndrome and dysautonomia /weakness is something that could be very promising to follow up on, in light of the connections between dysautonomia and ME/CFS and I’m glad that some clinicians think so too. Hopefully this will translate to more research.

When a new syndrome seems to explode in prevalence, there are more than one possible explanation. Reflexive skepticism along the lines that it is a fad is one. The idea that it has always been prevalent but is only being properly diagnosed is another. A third explanation is that there is some environmental variable that is changing rapidly.

The supposed iatrogenic harm from this diagnosis being publicized and conjectured as a cause of ME/CFS has to be weighed against the harm that would come if it was indeed a) a cause of me/cfs that was ignored and b) increasing in prevalence due to some environmental factor that has yet to be discovered or even addressed.

The arguments that hEDS and cervical medullary syndrome are basically being created by practitioners who convince patients they have them strike me as similar to arguments that “CFS” is a fabricated diagnosis, especially when it’s argued that specialists who diagnose and treat it are enabling the patients, and that the patients spend too much time googling their symptoms, or that enabling parents help their children fabricate the symptoms.

That does not mean that the argument is invalid but I would like to see hard evidence supporting that opinion/conjecture.

Also I think the literature around this syndrome is worth reading beforehand people make up their mind either way. This paper mighjt be a good start: https://www.omicsonline.org/open-ac...ive-disorders-2165-7939-1000299.php?aid=71754

 

Secretly, or not so secretly now I am writing it on the WWW, I am wondering if PWME with more neurological symptoms might experience a subjective improvement simply due to widening of the spinal canal in this procedure.

We know there have been studies on decreased blood flow rates in the brain stem in ME, also that Sophia Mirza's autopsy showed inflammation of the ventral dorsal ganglia, is it a stretch to suppose that moderate inflammation in the spinal cord could produce CCI/adhesion like symptoms which might be relieved by aligning and fixing these vertebrae?

Just thinking out loud. If that was the case then you would not expect total remission, they would still get PEM, but they might be relieved of more severe neurological repercussions of PEM.

 

I'm wondering if there is any data on the prevalence of asymptomatic CCI or AAI.

The Medscape article below says:

It also says:

The issue being that if some significant portion of the population has asymptomatic CCI or AII how can you tell if a positive finding on imaging is causing ME/CFS symptoms or is just another asymptomatic case of CCI/AII?

If a much larger percentage of ME/CFS patients showed positive imaging results than the asymptotic background percentage that would certainly be suggestive, but I'm not sure that the asymptomatic background numbers are even known.

 

I seem to recall an Italian doctor was doing surgery 'unblocking' veins restricting blood flow to the brain on MS pts. He claimed a large success rate, including for his wife.

Later a study found that there was no evidence of impaired blood flow in MS pts.

 

Thanks for pointing out the citation.

Henderson again.

I find this underwhelming. There is pretty much nothing in it I have not been aware of for most of my career. I routinely did flexion imaging in the 1970s. He admits that he has no data other than on osteogenesis imperfects and Down's syndrome. The stuff about measurement lines is arbitrary as far as I can see. His attempt to give a basis for symptoms seems if anything rather naive. The optic nerve is not a lower cranial nerve and even the acoustic and vestibular nerves enter the pons (or PMJ) rather than medulla. He seems to make no clear distinction distinction between Chiari descent and CCI per se.

I am afraid it confirms my impression that this 'medullary syndrome' is an artefact of referral and fashion. Why was there need for a new 'consensus statement' about something students have been taught about for decades? Why would only people with no well defined pathological basis for their CCI have this new syndrome when those with a known underlying disorder have the old fashioned symptoms and signs? I may have missed something but it does not seem to be in this review.

My scepticism is not reflexive. I am all for frank debate but it is best not to assume other people's ways of thinking. I have no scepticism about ME/CFS. I believe in it enough to want to spend an hour or two a day trying to do what I can to improve the research situation for PWME. But I am sceptical about speculations that sound superficially plausible but do not seem to have any substance beneath the surface.

And as Michiel says, Henderson's results suggest that surgery is not that good for what sound like ME-type symptoms if they do occur.

 

these are good questions. What I wonder about is why some people have a worse clivo axial angle than others, or similar measurement, and yet are less symptomatic or vice versa.

However, I think the problem of assuming rarity here is that so many people in the general population have never had the specialized imaging needed to establish a real baseline. Thus it is only assumed or estimated to be rare. Unlike some disorders in which routine labwork would show a problem or lead to further labwork, this one requires specialized imaging that is expensive , unlikely to be ordered by the vast majority of specialists, etc. in addition, the reason this condition is thought to be rare is that it is thought to only occur as the result of head trauma or connective tissue disorders—most of these congenital. When someone doesn’t have a known connective tissue disorder, it would be relatively unheard of to have this condition. ANd yet, this seems to be the case in many of the people recently diagnosed with it. Which suggests that true prevalence may be underestimated but also that investigation is needed into the cause in these cases.

So, in short, your questions are good but we have no idea what the true prevalence of this condition is.

 

I don't really understand this. CCI is a situation where bones are moving abnormally in relation to each other - right? Bones show better on plain old fashioned x-rays than on MRI - much better resolution. Flexion x-rays of the neck for CCI have been around since I was a student at least. You do not need the extension picture because any slippage always reduces on extension. The peg moves back towards the medulla when you nod forward. I remember discussing all these lines with my coauthors when we were writing a book on rheumatoid surgery in the mid 1980s. We didn't have MRI then. Normal values for distances were in the radiology books.

I thought the standing MRI was to show the Chiari descent rather than the CCI. I find the whole thing very confusing. Maybe I have missed something but it all seems unclear.

 

I can tell when i'm building up to PEM or its coming on or i'm borderline. I assumed most other ME/CFS patients can as well.

This is the thing, i suspect that some of us may not have ME but have CCI. If our goal is to talk people out of getting tested "for the children" or because its a serious surgery or you could die from infections or it can't be involved then what we are really saying is we don't believe CCI is a concern and want to scare you away.

 

Anyone who can afford this surgery has a different threshold of what recovery means. Plus you have to sustain it years without relapse.

My peers in the United States are working 60 hour work weeks, have active social lives on top of raising families. I do not think they are doing this effortlessly honestly, but that is the standard of recovery, because I do not exist in a vacuum. My parents are doing that in their 70s, minus the families.

 

I'm pretty sure all that needs to be asked at this point is two related questions.

1) Can CCI produce the symptoms of ME/CFS?

and 2) Do ME/CFS patients have CCI at the higher rates than healthy controls?

The only way to do this is a blinded study of the MRIs and a critical review of the measurements used to diagnosis. My concern is the surgeons are very unlikely to do this, possibly ever. And probably someone open-minded but expert in neuro-surgery other than than them needs to do it. The only other options are NIH study and private funding.

Questions about relationship and pathology seem premature.

 

Is it relieved after lying down for a while, or does it take 48+hrs to feel back to your baseline?

 

It depends how bad the PEM episode is. I try not to get into monster PEM anymore but my PEM episodes typically take 1-4 days to pass. I've had 1-2 week PEM before, hideously awful.
As for lying down helping if i am near the threshold i can sometimes lie down it off. Thats rare because i either stay below the threshold or massively overshoot it because i had to go out somewhere exhausting so i can't stop and keep below threshold

 

@Alvin do experience delayed PEM? Where you can feel baseline after activity and PEM only hits 16+hrs later?
The PEM that starts to build up gradually (for me) is relieved by lying down, so for me it's not really what I would describe as PEM.

I only experienced one episode of PEM in 28yrs that lasted over a week, it was brutal. I went waaay over with exercising. It was very distressing and I almost called 911. Never again

Ok, I'm getting off topic now.

 

It used to be often delayed several days, a week once but these days usually next day, the odd time 2 day delay. Sometimes instant but not often