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

[JenB]

Sorry @JenB but you are illustrating just how little you understand about this.

Is a dog clearly distinct from a cat?
Are their features non-overlapping? Of course not. They both have four legs, but the shape of the leg is always rather district. The both have two eyes but the retinal structure is rather different. And so on.

I still don't understand the nature of the ignorance you say I am revealing. I did not understand what Michiel meant by distinct. You can say I don't have a firm grasp on the English language, which is fine, but that is an honest misunderstanding. It stems in part from the fact that I don't think you can definitively distinguish between all cases of CCI and ME based on symptoms. Good thing we don't have to.

A skilled neurologist has an almost zero chance of confusing symptoms due to CCI and due to ME. I was never in a situation where the situation was unclear for one of my patients.

I would like to see one single skilled neurologist endorse your analysis.

Well, then clearly I have come across many unskilled neurologists. (I can't disagree with that! )

I'm sorry, one skilled neurologist endorse which analysis? That I had CCI and tethered cord? Those are my diagnoses, not my personal analysis.

What I *think* you are saying is that the textbook CCI case is clear and the textbook ME case is clear based on symptoms alone (but really? where? when? and what doctors have been trained adequately to diagnose ME? isn't that what we are all fighting for?). My experience is that textbooks are often wrong and do not encapsulate the range of symptoms patients actually experience and that clinically, there can be a lot of variation in symptoms.

I still don't think I can say that CCI is "distinct" because I cannot distinguish between the symptoms I had prior to my thyroidectomy and ICC-ME or Ramsay ME or another flavor of ME. Neither could Donna Felsenstein, Nancy Klimas, Derek Enlander, John Chia, Jose Montoya, Dan Peterson or David Kaufman. (Yes, I know it is ridiculous that I've been able to see that many doctors. It's part of why I am putting all this out there--I know how unfair it is.) Neither could any of the many neurologists I have seen over the years.

What I will say is that the neurosurgeon that operated on me was skeptical CCI was causing my symptoms to the point of being almost hostile. He did not recognize my symptom picture as consistent with CCI and I think thought I was wasting his time. It was only when he ran me through the gauntlet of testing that he surprised himself and saw that objectively, yes, I did meet the criteria. For him I was an alien, a highly unusual, once or twice in a career case. Among his patients, I was an outlier. Among ME patients, I am not. I was a dog in cat's clothing, as it were.

So in the end, I can't really blame the neurologists although as a lot, I wish they were a little more curious and a little less prone to diagnosing people with conversion disorder.

Again, so much more to write...

 

[Guest 102]

The faster we can do research in this area, the sooner we can begin to make recommendations (if any) and fight for access to better diagnostics for CCI and other conditions that can cause the symptoms of ME.

You seem to be broadening ME, more and more, Jen.

ME is a discrete neurological illness, but you seem to be now speaking from under the CFS umbrella.

RamsayME has been so thoroughly neglected since my own diagnosis, there was a window of research in 1980s, but that all disappeared with Wessely & co - and now you are passionate about CCI diagnostics, which is important, I am sure, but you seem to have lost focus because of your own very idiosyncratic experience. I just feel really baffled. Truly.

 

[JenB]

Lastly--if we think that CCI has nothing to do with ME, then we should look with more rigor at the ICC criteria. There are so many symptoms in the ICC criteria that are symptoms of intracranial hypertension, CCI, tethered cord, and MCAS, all of which we observe (yes, the research is spotty!) co-occurring with greater frequency among people with hEDS. Whether we think that what the clinicians who wrote the criteria describe as ME is actually a patient population in which these conditions also co-occur OR we are talking about distinct but symptomatically overlapping syndromes that need to be ruled out (exclusions to add to the primer as Colleen writes) I think we could be doing a much better job at serving patients.

 

[Grigor]

but really? where? when? and what doctors have been trained adequately to diagnose ME? isn't that what we are all fighting for?

Well again Prof Todd Davenport definitely is knowledgeable enough about ME and CCI.

What I will say is that the neurosurgeon that operated on me was skeptical CCI was causing my symptoms to the point of being almost hostile.

So you're proving here exactly that CCI does have it's distinct clinical picture. That doesn't mean you can't have both ME and CCI simultaneously.

Again distinct not overlapping

 

[JenB]

You seem to be broadening ME, more and more, Jen.

ME is a discrete neurological illness, but you seem to be now speaking from under the CFS umbrella.

RamsayME has been so thoroughly neglected since my own diagnosis, there was a window of research in 1980s, but that all disappeared with Wessely & co - and now you are passionate about CCI diagnostics, which is important, I am sure, but you seem to have lost focus because of your own very idiosyncratic experience. I just feel really baffled. Truly.

No I am not. Many people on this thread have argued that cervical stenosis (which can cause the symptoms of severe ME, at least according to Peter Rowe) and Chiari (which can also cause similar symptoms) need to be ruled out. Perhaps CCI does as well. However, most people who have ICC-ME (that is, meet the clinical/symptom criteria) do not have access to the imaging that could rule out these conditions. In order to ensure that people who meet the ICC-ME clinical criteria do have access to this imaging, we'd first need to establish how frequently these conditions occur in our population in order to justify recommending that all patients or patients with certain additional symptoms be referred for cervical imaging. Otherwise "rule out Chiari, cervical stenosis and CCI" is abstract and will almost never happen in practice.

This does not change my assertion that I had ME for all of the reasons of onset, symptoms, response to drug treatment and lab findings I have previously described.

 

[JenB]

Well again Prof Todd Davenport definitely is knowledgeable enough about ME and CCI.

So you're proving here exactly that CCI does have it's distinct clinical picture. That doesn't mean you can't have both ME and CCI simultaneously.

Again distinct not overlapping

Right, the CCI clinical picture my neurosurgeon is familiar with was different from mine. Perhaps this is because his patient population is almost entirely comprised of people with EDS, Marfan's and other genetic connective tissue disorders. I still had CCI.

I still am not entirely certain why my clinical picture looked so different to him. The major piece was my central apnea, which my surgeon called a kind of "partial Ondine's syndrome" that he had only seen once before. It's difficult to have a clear conversation about this with him as he (at least at the time) didn't really understand ME, PEM, etc. and was completely unfamiliar with our diagnostic criteria and clinical picture. There may have been other things that struck him as odd. I don't know.

What I am trying to say is my clinical picture did not look like a "typical" EDS CCI case (I imagine CCI looks slightly different in EDS v. Down's Syndrome v. RA v. TB). Yet I still had CCI and it was patently causing all of my symptoms. At least, I think that much is clear. You guys can be the judge when I finish describing my case. I'd be curious to see what other alternative explanations you might see. I don't find blood transfusion (didn't have one) or anesthesia (why didn't it work the first, second, third, fourth, sixth or seventh time?) or "spontaneous remission" to be particularly compelling, but again, more soon.

Ha--I really need to stop writing here and get those Medium pieces written!

 

[anniekim]

Can I ask you, in a friendly way, if a tiny wee part of you thinks you may have been misdiagnosed? I

@JenB, i too would be interested in your answer to this question? Thank you.

 

[rvallee]

I can't follow much of this thread because of its sheer size but speaking as someone who generously directs a lot of anger and targeted contempt towards those who have made our lives considerably worse that we should be nicer to one another as we are all trying to figure something out without having the proper tools for it.

It just does not achieve anything, too much energy is spent on accusations here. More problem-solving and less blame-assigning, back to basics. We don't know. They don't know. Nobody knows. Let's just figure it out nice and calm with the shared reality that diagnosis is as difficult as pinpointing the exact nature of this disease.

 

[Trish]

Where does all this leave me jen? A mum in the UK with no way of finding out if I have some messed up spine?? A gp who still proposes I have 'functional' issues... I feel lost.

Hi @Hopeful1976. I am in the UK too. I sympathise with you over the GP's lack of understanding. It's so hard to find ones who understand ME is a physical illness. I found giving them a copy of the ME Association 'Purple book' helped a bit. I'm not sure they read much of it, but at least it shows doctors it's an illness to be taken seriously. You can ask the MEA to send a copy to your GP practice.

As to the spinal stuff. So far there is no scientific evidence that CCI/AAI have any relevance to ME. Unless the ME patient also has specific neurological symptoms or signs that aren't part of the normal ME picture, then of course that should be checked out.

I am not rushing to try to get my neck checked. Even if I were able to travel to a testing centre, which I'm not, I don't have any of the specific signs that indicate CCI/AAI as Jen and Jeff did.

 

[dreampop]

It is usefulness to ask whether, in general, persons with ME/CFS have symptoms consistent, in general, with people with CCI. And the pathological reasons behind asking that question. I think those are valid disclaimers.

In my opinion, if (the generic) you were to see improvement afterwards but still have symptoms of ME, as is the case with myself, then logic says you had ME as well as the other condition, and if your ME symptoms disappear then, logically, it is likely that you had something that looked like ME but actually wasn't (and congratulations, you've won the health lottery, you got your life back).

I do not understand the tendency to assume misdiagnosis of an indivdual who clearly has met all the criteria for ME/CFS, but has recovered. It seems that Jen has been asked to do the impossible - say she never had ME/CFS, something untrue to her and her ME/CFS experts, if the syndromes are different and produce clearly different syndromes. Or say CCI creates ME/CFS, or was "something that looked like" it, a feat she as an individual can neither prove or disprove (even a clear case report would not prove this).

If she met the criteria, and all these experts think she had it, that's the best we have for the time being. Without a clear test, it's kind of frustrating to keep this they never had "real me" going and impossible for someone to asnwer.

 

[Guest 102]

No I am not. Many people on this thread have argued that cervical stenosis (which can cause the symptoms of severe ME, at least according to Peter Rowe) and Chiari (which can also cause similar symptoms) need to be ruled out. Perhaps CCI does as well. However, most people who have ICC-ME (that is, meet the clinical/symptom criteria) do not have access to the imaging that could rule out these conditions. In order to ensure that people who meet the ICC-ME clinical criteria do have access to this imaging, we'd first need to establish how frequently these conditions occur in our population in order to justify recommending that all patients or patients with certain additional symptoms be referred for cervical imaging. Otherwise "rule out Chiari, cervical stenosis and CCI" is abstract and will almost never happen in practice.

This does not change my assertion that I had ME for all of the reasons of onset, symptoms, response to drug treatment and lab findings I have previously described.

Chiari is already ruled out in UK according to Abhijit Chaudhuri. I did suggest in another post you contact him to get his input.

 

[Guest 102]

Hi @Hopeful1976. I am in the UK too. I sympathise with you over the GP's lack of understanding. It's so hard to find ones who understand ME is a physical illness. I found giving them a copy of the ME Association 'Purple book' helped a bit. I'm not sure they read much of it, but at least it shows doctors it's an illness to be taken seriously. You can ask the MEA to send a copy to your GP practice.

As to the spinal stuff. So far there is no scientific evidence that CCI/AAI have any relevance to ME. Unless the ME patient also has specific neurological symptoms or signs that aren't part of the normal ME picture, then of course that should be checked out.

I am not rushing to try to get my neck checked. Even if I were able to travel to a testing centre, which I'm not, I don't have any of the specific signs that indicate CCI/AAI as Jen and Jeff did.

If I were to have the specific MRI tomorrow and any abnormalities were found I still would not have that surgery unless I had the kind of symptoms Jen and Jeff have had, there is no evidence or reason for me to do so.

 

[anniekim]

I can't follow much of this thread because of its sheer size but speaking as someone who generously directs a lot of anger and targeted contempt towards those who have made our lives considerably worse that we should be nicer to one another as we are all trying to figure something out without having the proper tools for it.

It just does not achieve anything, too much energy is spent on accusations here. More problem-solving and less blame-assigning, back to basics. We don't know. They don't know. Nobody knows. Let's just figure it out nice and calm with the shared reality that diagnosis is as difficult as pinpointing the exact nature of this disease.

I am sorry I don’t see any anger on this thread? I just see people politely asking questions and discussing Jen’s remarkable story of remission of symptoms after surgery.

 

[dreampop]

If I were to have the specific MRI tomorrow and any abnormalities were found I still would not have that surgery unless I had the kind of symptoms Jen and Jeff have had, there is no evidence or reason for me to do so.

But, if you had Chiari, would you? Just a thought experiment. Because one could make the same argument Chiari and ME/CFS don't look a lot alike.

In all seriousness, I have great reservations about this kinds of surgery and agree we need more data, and someone smarter than me really needs to figure out how malleable the objective measurements are. I still think the only way to get this done in a remotely timely fashion would be to squeeze it into the NIH study although it's probably too late for that.

 

[Guest 102]

I still don't understand the nature of the ignorance you say I am revealing. I did not understand what Michiel meant by distinct. You can say I don't have a firm grasp on the English language, which is fine, but that is an honest misunderstanding. It stems in part from the fact that I don't think you can definitively distinguish between all cases of CCI and ME based on symptoms. Good thing we don't have to.



Well, then clearly I have come across many unskilled neurologists. (I can't disagree with that! )

I'm sorry, one skilled neurologist endorse which analysis? That I had CCI and tethered cord? Those are my diagnoses, not my personal analysis.

What I *think* you are saying is that the textbook CCI case is clear and the textbook ME case is clear based on symptoms alone (but really? where? when? and what doctors have been trained adequately to diagnose ME? isn't that what we are all fighting for?). My experience is that textbooks are often wrong and do not encapsulate the range of symptoms patients actually experience and that clinically, there can be a lot of variation in symptoms.

I still don't think I can say that CCI is "distinct" because I cannot distinguish between the symptoms I had prior to my thyroidectomy and ICC-ME or Ramsay ME or another flavor of ME. Neither could Donna Felsenstein, Nancy Klimas, Derek Enlander, John Chia, Jose Montoya, Dan Peterson or David Kaufman. (Yes, I know it is ridiculous that I've been able to see that many doctors. It's part of why I am putting all this out there--I know how unfair it is.) Neither could any of the many neurologists I have seen over the years.

What I will say is that the neurosurgeon that operated on me was skeptical CCI was causing my symptoms to the point of being almost hostile. He did not recognize my symptom picture as consistent with CCI and I think thought I was wasting his time. It was only when he ran me through the gauntlet of testing that he surprised himself and saw that objectively, yes, I did meet the criteria. For him I was an alien, a highly unusual, once or twice in a career case. Among his patients, I was an outlier. Among ME patients, I am not. I was a dog in cat's clothing, as it were.

So in the end, I can't really blame the neurologists although as a lot, I wish they were a little more curious and a little less prone to diagnosing people with conversion disorder.

Again, so much more to write...

Sorry, I am really bad at absorbing detailed threads, you have way more stamina than me! So your CCI presentation was rare, yet your ME was typical, so does that not mean that it would be rare for a pwME to have obvious CCI signs - and most of us probably don’t. I am lost!

 

[Guest 102]

But, if you had Chiari, would you? Just a though experiment. Because one could make the same argument Chiari and ME/CFS don't look a lot alike.

In all seriousness, I have great reservations about this kinds of surgery and agree we need more data, and someone smarter than me really needs to figure out how malleable the objective measurements are. I still think the only way to get this done in a remotely timely fashion would be to squeeze it into the NIH study although it's probably too late for that.

I don’t have Chiari, my diagnosis has not changed in 36 years.

 

[dreampop]

I don’t have Chiari, my diagnosis has not changed in 36 years.

I was just asking hypothetical, a question that I asked myself as I was reading this thread.

 

[JenB]

@JenB, i too would be interested in your answer to this question? Thank you.

Hi! Thanks for the friendliness Here is my long answer to that question: https://link.medium.com/ul5iukw35W

The short answer is no. I met the clinical criteria in spades and for now, ME is a clinical diagnosis. I also responded to commonly prescribed treatments and had numerous lab findings consistent with this diagnosis. To say that you have ME if you don’t know what is causing your symptoms but not if you have additional testing almost no one has had and uncover a mechanism underlying those symptoms is to render ME a diagnosis of exclusion. It would take more research and an updating of the criteria for me to rethink my understanding of this. We would also need to explain all of my “looks like ME but hardly definitive” (because that does not exist) lab findings and onset history.

I find the “you got better, therefore didn’t have ME” or the “you found your mechanism (not to be clear, cause), therefore you never had ME” reasoning to be circular.

Better to ask if/how/why cranial settling can cause PEM and all of the other symptoms I no longer have.

 

[JenB]

Sorry, I am really bad at absorbing detailed threads, you have way more stamina than me! So your CCI presentation was rare, yet your ME was typical, so does that not mean that it would be rare for a pwME to have obvious CCI signs - and most of us probably don’t. I am lost!

Sorry about that!

I don’t know. We would need research to figure that out. Maybe I am a unicorn (I don’t know a lot about how the other six cases presented or what their surgeons thought of their symptoms). Or maybe we present differently (those of us with CCI who meet the criteria for ME) such that CCI criteria/descriptions need to be updated. Or maybe a lot of people who meet ME criteria have CCI. I think it is too early to be able to draw any firm conclusions.

 

[JenB]

If I were to have the specific MRI tomorrow and any abnormalities were found I still would not have that surgery unless I had the kind of symptoms Jen and Jeff have had, there is no evidence or reason for me to do so.

I think this is totally reasonable. I also would not have had this surgery on the basis of my pre-thyroidectomy symptoms, even with positive imaging. I would still like to find out the prevalence of CCI, though, among pwme. I wish I had known I had it going into my thyroid surgery. It would be helpful to know whether there is a need for additional precautions during surgery (or not) and for whom.