Signs of Intracranial Hypertension, Hypermobility and Craniocervical Obstructions in patients with ME/CFS (Pre-print 2019/published 2020) Bragée et al

[Midnattsol]

from article posted by @mango


I haven't read either article in detail; just querying the above discrepancy ie Were the patients only recently diagnosed with ME?

I'm not sure I see the discrepancy, I have had symptoms for 10 years soon, but was only diagnosed three years ago.

 

[saltcaramel]

You would think so.
Personally I would like to see these surgeons given 500 MRIs of healthy people and 500 MRIs of those with CCI or even ME type symptoms. And see them calculate measurements (CXA, the other measures they use) for both groups. In one study. Without bias. Without knowing who is in which group. Or even, get outside people to calculate these.

Then I’d like to see these people - both healthy and ME undergo traction, dose dependent like J Edwards mentioned, so they don’t even know what amount of traction is applied, and Id like to see what both healthy people and those with ME say about it.

Just don’t understand why the surgeons don’t have this data. And why people are ok with that. It’s also in my mind probably what the Bragee study should’ve done first - especially the measurements.

Personally, I would also LOVE to see this kind of study!! I’m not sure why it hasn’t been done, many patients are longing for this! We can only hope that it might come.

At the same time, it’s very important to remember that CCI is essentially a clinical diagnosis. The surgeons performing these surgeries state again and again in their video lectures, measurements aren’t everything! Super fit healthy people can have terrible looking MRIs; very sick people can have what looks on MRI to be a super mild problem and then have their symptoms resolve when that “minor” problem is fixed.

I’m personally excited about this Swedish study. Of course it has its limitations. But I believe it’s an important arrow, pointing in a direction and saying “Look, here!” I don’t believe it’s more than that, at this stage. But I believe it would be a grave mistake not to follow that big red arrow and investigate further, with an open mind to all possibilities! I am really grateful to Dr Björn for uncovering this arrow.

 

[Jonathan Edwards]

In a generally hypermobile female group, where only a minority have problems with repeated subluxations from normal movements, it's noticeable that the joints involved are only the ball-and-socket and condyloid types. I suspect some of our joint sockets might just be a bit shallow, which in someone who didn't also have laxity would never cause any issues at all.

I don't think the mechanics work that way at all to be honest. The problem with all this EDS theorising is that you can think up whatever possible mechanism you like but unless in reality it applies to the human body it is of no interest. The two ball and socket joints have completely different ranges of problems because the shoulder is very shallow and stabilised by muscles and tendons and the hip is deep and pretty much never subluxes unless there is major bone dysplasia. eDS is just one of a range of reasons for having mobile joints. but unfortunately has become a catch all wastebasket diagnosis.

 

[Jonathan Edwards]

At the same time, it’s very important to remember that CCI is essentially a clinical diagnosis. The surgeons performing these surgeries state again and again in their video lectures, measurements aren’t everything! Super fit healthy people can have terrible looking MRIs; very sick people can have what looks on MRI to be a super mild problem and then have their symptoms resolve when that “minor” problem is fixed.

The problem with this is that it allows the surgeons to sidestep any suggestion that they are operating on imaging that does not mean much. They so, oh but what matters most are the symptoms. That is absolutely true if they really are symptoms of CCI but symptoms of CCI are quite distinct from symptoms of ME. Feeling as if your head will fall off is not a symptom indicative of CCI. Nor is tinnitus. Nor are many of the things that have been suggested by some of the surgeons doing these operations.

I have not seen any evidence for anyone with ME having symptoms or signs that would indicate surgery for CCI.

 

[Jnoelle]

My belief is primary based on reading the literature on classification and knowing the people who have been involved in creating this diagnosis over the last few decades. I co-authored one of the first papers putatively linking hypermobility to other problems and as part of that I learned that physicians working in this area tend not to understand how reliable evidence works.

My own clinical experience over 30 years is also relevant in that I never met a patient who I thought this diagnosis would be useful for. I worked alongside a clinic where the diagnosis (or an older equivalent) was repeatedly made for reasons that were never clear to me.

I find your statement "I never met a patient who I thought this diagnosis would be useful for" unfortunate for your patients.

I was diagnosed by Prof Grahame in 2019 with hypermobility. During that encounter, he helped me understand what it meant for my body, including explaining a lifetime of seemingly unrelated issues that no other doctor could bring together - the most recent of which was given my level/type of activity, how did I manage to tear my rotator cuff and why isn't it healing on a 'normal' timeline in spite of PT?

Prof Grahame also sent me for an upright MRI to investigate possible CCI/AAI. Medical professionals who understand hypermobility (whether hEDS or hypermobility spectrum disorder) use that as a spring board to help patients manage symptoms, wide ranging as they are. https://www.ehlers-danlos.com/ is a great educational resource.

Just because there is no genetic test (yet) to identify this connective tissue disorder, knowledge (in the right hands) is empowering.

 

[Jonathan Edwards]

Prof Grahame also sent me for an upright MRI to investigate possible CCI/AAI. Medical professionals who understand hypermobility (whether hEDS or hypermobility spectrum disorder) use that as a spring board to help patients manage symptoms, wide ranging as they are. https://www.ehlers-danlos.com/ is a great educational resource.

As I pointed out somewhere Rodney Grahame and I set up the first hypermobility research clinic in the UK in around 1980. I have known Rodney as a departmental colleague for many years. He would like to think he understands hypermobility but the problem is that he has never done any valid research that would tell us whether he understands or not. Most of what I have heard being said about hypermobility is just made up. The population studies that have been done do not bear it out.

I am sorry to sound mean but if people are having unnecessary operations because they are being sent for upright MRIs, which are generally regarded as worthless in this context, I have to be very frank. I personally do not think Rodney Grahame has ever known what he was talking about.

It is not easy for me to say that because Rodney was very kind to me early in my career and helped me get key jobs for training. I owe him a great debt of gratitude. However, my gratitude to a colleague cannot affect my responsibility to be honest when it matters in terms of patient care.

 

[Sly Saint]

I'm not sure I see the discrepancy, I have had symptoms for 10 years soon, but was only diagnosed three years ago.

just trying to get clarification.

I've just re-read BB post a couple of pages back and he says the same as in the article, that all the patients were newly diagnosed.
I'm not querying the diagnosis, just curious to know what they were diagnosed with/treated for before the ME/CFS diagnosis (particularly as they were severe and many had been ill for some time).
But obviously I realise that that information is private.

 

[Kitty]

The two ball and socket joints have completely different ranges of problems because the shoulder is very shallow and stabilised by muscles and tendons and the hip is deep and pretty much never subluxes unless there is major bone dysplasia.

Interesting, we do have different problems with hips and shoulders. Hips sublux, in my case so often that I had a physio train up friends to shove them back in. They would never self-right, and if I left them 'out' I'd get sciatica because the muscles went into spasm.

We seem to sublux less after middle age, perhaps because joints are a bit stiffer or we move more cautiously. Eventual femoral head replacement surgery helped a lot in my mum's generation, with some of them reporting less pain than they'd had since they were teenagers.

With the shoulders it's more a tendency to injure them doing everyday things, and subluxations don't need intervention to right them. The instability tends to get worse with age, possibly because of repeated injuries.

I'm not arguing that it's EDS – I think we may have trait that manifests as some kind of hip joint abnormality.

 

[Jnoelle]

The problem with this is that it allows the surgeons to sidestep any suggestion that they are operating on imaging that does not mean much. They so, oh but what matters most are the symptoms. That is absolutely true if they really are symptoms of CCI but symptoms of CCI are quite distinct from symptoms of ME. Feeling as if your head will fall off is not a symptom indicative of CCI. Nor is tinnitus. Nor are many of the things that have been suggested by some of the surgeons doing these operations.

I have not seen any evidence for anyone with ME having symptoms or signs that would indicate surgery for CCI.

I have ME (dx by Dr Weir).

A group of US ME specialists have published this which lists, among differential diagnoses on p3: CCI, CSF leak, Chiari and others

The neurosurgeons' intake forms I have seen (Henderson + Gilete, Malik did not have any at the time) request information regarding symptoms, many of which are identical to ME questionnaires.

I do not know what 'the many things that have been suggested by some of these surgeons" might be. I was not guaranteed by any surgeon that surgery would resolve all my issues, though was told it may well be causing some or all them.

All this to say, there are other medical professionals with different views to yours.

All patients want (or at least this patient) is to be well or better. Each person's path is unique in getting there.

 

[Jonathan Edwards]

All this to say, there are other medical professionals with different views to yours.

Yes, I think that list could be added to the names I gave.
The reason the surgeons' list looks like ME is because Henderson has invented this syndrome that sounds like ME which he says is due to CCI, but those of us who have looked after CCI for years have never come across anything remotely like ME.

These are the people I regard as on the fringe of medicine in this respect.

 

[GloriaDG]

The problem with this is that it allows the surgeons to sidestep any suggestion that they are operating on imaging that does not mean much. They so, oh but what matters most are the symptoms. That is absolutely true if they really are symptoms of CCI but symptoms of CCI are quite distinct from symptoms of ME. Feeling as if your head will fall off is not a symptom indicative of CCI. Nor is tinnitus. Nor are many of the things that have been suggested by some of the surgeons doing these operations.

What if the medical literature on CCI has been wrong or simply incomplete up until now? What if there are different manifestations of CCI, depending on the personal unique physiology of each individual (maybe mechanisms as well, ex autoimmunity and bone erosion Vs genetic laxity)? Fighting in order to preserve certain established medical ideas, despite being outdated, has already happened in medicine. Thousands of times.

In the 17th century William Harvey has been the first one to affirm that blood circulates in the body and is pumped to the brain and the rest of the body by the heart. Up until the 17th century, they believed therw were two separate systems involved in blood circulation: the natural system, containing venous blood which had its origin in the liver, and the vital system, containing arterial blood and the 'spirits' which flowed from the heart, distributing heat and life to all parts. Like bellows, the lungs fanned and cooled this vital blood. Crazy, right? Many of these beliefs were based on Galean's hypothesis.

Well, Harvey encountered incredible backlash, disbelief and controversy for his discovery. Discovery that for us today is just as obvious as 2+2=4. Some doctors even affirmed they would "rather err with Galen than proclaim the truth with Harvey." I feel like this is good old history repeating itself.

 

[lunarainbows]

I have ME (dx by Dr Weir).

A group of US ME specialists have published this which lists, among differential diagnoses on p3: CCI, CSF leak, Chiari and others

The neurosurgeons' intake forms I have seen (Henderson + Gilete, Malik did not have any at the time) request information regarding symptoms, many of which are identical to ME questionnaires.

I do not know what 'the many things that have been suggested by some of these surgeons" might be. I was not guaranteed by any surgeon that surgery would resolve all my issues, though was told it may well be causing some or all them.

All this to say, there are other medical professionals with different views to yours.

All patients want (or at least this patient) is to be well or better. Each person's path is unique in getting there.

I’ve seen that document before but only just had a look at the names. I’m not sure I am completely happy to trust that document because of who it is written by. This name popped out at me:

Dr Ilene Ruhoy, who runs or is heavily involved with the Centre for healing neurology, which among other things, uses Ayurvedic healing (completely alternative, and I say this as someone whose family literally grew up with Ayurvedic medicine), Regenerative medicine (which includes injecting dextrose, exosomes into a person - maybe this is related to the neck injections we discussed elsewhere?), and finally Autism treatment where they “dive deep to look for potential underlying triggers” (their own words).

She has also written a blog about CCI before if I remember correctly, and has shadowed Dr Bolognese? So I’m not certain she’s truly independent in this case.

 

[Jnoelle]

I’ve seen that document before but only just had a look at the names. I’m not sure I am completely happy to trust that document because of who it is written by. This name popped out at me:

Dr Ilene Ruhoy, who runs or is heavily involved with the Centre for healing neurology, which among other things, uses Ayurvedic healing (completely alternative, and I say this as someone whose family literally grew up with Ayurvedic medicine), Regenerative medicine (which includes injecting dextrose, exosomes into a person - maybe this is related to the neck injections we discussed elsewhere?), and finally Autism treatment where they “dive deep to look for potential underlying triggers” (their own words).

She has also written a blog about CCI before if I remember correctly, and has shadowed Dr Bolognese? So I’m not certain she’s truly independent in this case.

The authors include numerous medical professionals/scientific researchers, including many who have received NIH grants and are at the forefront of the ME/CFS field in the US.

We will each have our own opinion and let's leave it at that.

 

[Jnoelle]

Yes, I think that list could be added to the names I gave.
The reason the surgeons' list looks like ME is because Henderson has invented this syndrome that sounds like ME which he says is due to CCI, but those of us who have looked after CCI for years have never come across anything remotely like ME.

These are the people I regard as on the fringe of medicine in this respect.

What populations of CCI patients have you looked after exactly? RA? Downs syndrome? Connective tissue disorders? traumatic brain injury following an accident? any others?

 

[Grigor]

we will do our best to take our responsibility, and not claim or advocate for treatments based on the findings, but for hypothesis testing and further research.

Thank you @Bjorn Bragee. I'm personally very concerned about the surgeries at the moment so I welcome research without it.

 

[Jonathan Edwards]

What if the medical literature on CCI has been wrong or simply incomplete up until now? What if there are different manifestations of CCI, depending on the personal unique physiology of each individual (maybe mechanisms as well, ex autoimmunity and bone erosion Vs genetic laxity)? Fighting in order to preserve certain established medical ideas, despite being outdated, has already happened in medicine. Thousands of times.

The puzzle for me is why people should want to keep trying to find reasons for thinking CCI might be important in ME when we have absolutely no published evidence of any value that indicates that. The onus is on people developing such ideas to present some evidence in support.

It would not simply be a matter of things being incomplete but of all sorts of basic understanding of connective tissue being wrong. The problem with throwing in all these things like autoimmunity and metalloproteinases and whatever is that if you actually work in the field you can see that the proposals for spinal involvement in ME just do not add up - because of the DETAIL of what would have to happen. It is hard to give a sweeping explanation but I have over the last two years thought hard about the plausibility of all the things that Jennifer Brea has come up with and they simply do not add up. I spent my life working out the role of B lymphocyte in autoimmune rheumatic disease and it takes years and years to get to a point where you can reliably predict how one part of a process can be expected to relate to another. The level of analysis used by Brea is simply hopeless.

But why, why, why do people want to go on pursuing this when we have no evidence for it at all? We just have pseudo-evidence from uninterpretable prevalence studies and recovery stories from some patients where we have no objective information.

The irony is the idea that I might be a conservative reactionary dismissing a new theory. If you ask anyone in UK academic medicine who I am then if they know they will say I am a loner maverick who causes trouble by criticising received wisdom. The reason I am critical of the spinal story is that I am critical of everything. If you look at threads here you will see that I have been just as critical about almost all other popular approaches to ME, because they are all pretty much nonsense. The truth is that we have no idea what ME is due to and we need to find out with proper science.

 

[Jonathan Edwards]

The authors include numerous medical professionals/scientific researchers, including many who have received NIH grants and are at the forefront of the ME/CFS field in the US.

I know. This is part of the problem.
I am sorry to say that PWME have been served badly in that a number of physicians who have moved in to the field are pretty uncritical about what they believe in. I never practiced in the field and when I started getting involved in the ME research scene I recognised maybe three or four competent groups - really no more than that. Almost the whole academic scene in ME has been 'fringe' for too long. We need some proper science.

 

[chrisb]

What if there are different manifestations of CCI, depending on the personal unique physiology of each individual (maybe mechanisms as well, ex autoimmunity and bone erosion Vs genetic laxity)?

If you believe that surely you have to believe that it would be wrong to extrapolate from your own experience and, based on that, suggest to others the cause and possible cure of their problems.

 

[lunarainbows]

The authors include numerous medical professionals/scientific researchers, including many who have received NIH grants and are at the forefront of the ME/CFS field in the US.

We will each have our own opinion and let's leave it at that.

If people are happy to work with and edit and produce a document with someone who provides treatments includes injecting unproven things into people’s bodies and says they take a “systematic approach to assess symptoms of autism“, then I think this is part of the problem, and I would also question the other researchers too. This doesn’t help the ME community. The fact that quackery has been allowed input into any document regarding ME is a huge problem. I think we deserve better.

 

[GloriaDG]

If you believe that surely you have to believe that it would be wrong to extrapolate from your own experience and, based on that, suggest to others the cause and possible cure of their problems.

I did not do or say that. This is completely irrelevant to the point I was trying to make, please let's avoid confusion.

What I was trying to say is: I agree that this is just an hypothesis that needs to be researched and demonstrated. However, I don't understand why in this forum the hypothesis that the CCI historical literature could be incomplete is so difficult to accept. What is being repeated over and over in this forum is that this hypothesis is nonsense.

It would not simply be a matter of things being incomplete but of all sorts of basic understanding of connective tissue being wrong.

Please note that I'm not endorsing Jen B hypothesis. I just believe, based on how history has always repeated itself and based on the observation of several anecdotal experiences (because like Ron Davis said through his wife, the scientific process can't start without observing) that some spinal/vascular pathologies could sometimes manifest in ways and with symptoms that just haven't been described in medical textbooks yet. This would happen for reasons that we still don't know, considering that the human body is so extremely complex. Why, for example, some people show all sorts of spinal problems on their imaging (non related to CCI) like stenosis for example, but are asymptomatic, while others with the same presentation on imaging are symptomatic? This is not something that the CCI neurosurgeons have made up, this is a mainstream concept. I've even had a look at CCI articles related to Rheumatoid Arthritis (so not related to the world of the EDD neurosurgeons) and they say exactly this:

"According to Rosario Maugeri, MD, PhD, of the University of Palermo, “Cervical spine involvement in RA is often a silent condition. Even in the presence of severe cervical spine damage, many patients may be asymptomatic.”

So, purely basing epidemiological studies on the prevalence of spinal issues in the ME community on the comparison of measurements between patients and healthy control, like it's being suggested here, would not be enough, as we don't understand yet why some people are asymptomatic and some not having the same spinal presentation.

The human body is so extremely complex.