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

One would expect Jen and Jeff's respective surgeries to resolve their new frightening CCI symptoms but not pre-existing ME. As you say, Trish, how can ME symptoms relieved by anti-virals - almost overnight as I recall from Unrest - or by extreme mould avoidance (also unproven beyond anecdote) - suddenly disappear after this surgical intervention. It makes no sense. It really doesn't - that this fusion surgery would treat a neuroimmune illness in its entirety. I sense that ME is being reframed here as 'PEM and POTS', and shoehorned into a very interesting - and happy, of course - outcome. I am unsure why misdiagnosis of ME cannot even be considered in these recovery stories as, at the end of day, in the absence of biomarker, we all have 'presumed' ME, no matter who we were diagnosed by or when we were diagnosed. And since the criteria have become ever more changeable, misdiagnosis is surely happening.

Also, PEM is now what seems to validate the ME diagnosis but in Ramsay's time PEM was not talked about it, it was muscle FATIGABILITY on trivial exertion - and days or weeks to recover. A quick look at social media will show that PEM now seems to have been adopted here, there and everywhere by those with other conditions. I do wonder if PEM experienced by those of us with ME is same as PEM being discussed elsewhere. (EDITED to make comment longer.)

 

Trying to be helpful on this specific point, having tried to clear up my own muddle about misremembering ventral as opposed to the correct dorsal root ganglia inflammation findings for Lynn Gilderdale, Sophia Mirza and Merryn Crofts, for which I apologise... perhaps this quote from Malcolm Hooper and Margaret Williams describes the relevance better than I could.

http://www.investinme.org/Documents/Library/magical-medicine.pdf see p98

My own understanding of this is it might relate to sensory hypersensitivity experienced by many people with ME, because the dorsal root ganglia are sensory ganglia. It doesnt quite explain the whole gamut of sensory hypersensitivity in ME because much of it relates to cranial senses like sensitivity to sound, smell and light. So in the absence of further evidence it is impossible to say if these correspond to cranial inflammation but it raises the question.

I would like to qualify this by saying that I do not believe it would be wise for anyone with an ME diagnosis to try this surgery speculatively as a treatment for ME based on current knowledge. I think that is the nightmarish scenario that some feel a responsibility to warn against and I agree its a horrifying idea and support them in this.

The reasons I feel this way are that I dont think we know what conditions (edit : related to ME) the surgery fixed for those who have been helped by it because we cannot reliably diagnose or characterise ME or any subtype of it yet, so it would not be safe to proceed on the premise that someone else's ME is the same as one's own ME. Which is why its not safe to treat ME as a group identity, even though politically our cause is a shared one, seeking better research so that our respective subtypes can be recognised and investigated properly.

For those of our friends who had expert diagnoses of CCI and kindly and bravely shared their experiences after surgery, I just want to say thankyou for sharing and wish you all good luck. I know from what I have read so far of your blog @JenB that you had little choice in going that route due to life threatening post thyroid op apnea. That this brought about ME remission is wonderful news, long may it continue and considering how valuable the humanity shown in Unrest has been, I am glad for you and Omar both. From a perspective of friendly self-interest I am doubly glad because of the powerful impact on ME awareness and research which Unrest and #MEACTION have had, thanks to all those involved and hope the campaign will continue with your involvement and the world will come to understand why your experience is a good argument for more research into ME, not less.

 

As I have said previously, I would love to have Dr Abhijit Chauhduri's view, who is one of few expert consultant neurologists on ME in UK. He replaced Peter Behan (who dxd me in 1984) in Southern General, Glasgow in 1990s and also he performed autopsy on the late Sophia Mirza. He also co-writes the ME Association clinical guidelines in which Chiari is listed as a differential diagnosis of ME. I know Chiari is not CCI but both cause brainstem compression is my very limited understanding.

 

The term PEM only came into existence in around 2000, it not only describes muscle fatigability but also a range of distressing symptoms that can take days, weeks and months to recover from. Back in 1991 when I was diagnosed by an ME doctor the term PEM was not mentioned at all, in fact I didn't experience it until 8 years into the illness when I started exercising again because I was feeling improved.

I've experienced delayed PEM with and without orthostatic intolerance (which developed years later).

 

Is it possible somebody could approach him regarding this?

@Russell Fleming?

 

Interesting, thanks - I was not sure when PEM first came into use - I experience the whole PEM shebang constantly, but of course muscle fatigability is integral to PEM in those of us who have ME, but I fear PEM is now being used indiscriminately on social media, I see people with sleep disorders talking about PEM, for example, in absence of an ME dx. (EDIT: Though I think PENE is much better description for pwME.)

 

Hi @JenB,

How are you doing?

In your tweet, and as Michiel previously mentioned (see page 10), you felt that the fact that ME and CCI have symptoms that are distinct from one another was "simply untrue"

https://twitter.com/user/status/1132704642083590144


I wondered about that as well. Although PEM/PENE is just one of the symptoms. As far as I know PEM/PENE or a kind of (vague) description of it is nowhere to be found in the literature or in any of the symptom lists in articles.

Of course you could argue that the neurosurgeons or scientists involved with CCI have no clue what PEM/PENE is and that's why it has never been mentioned but also Prof. Davenport seem to feel that CCI and ME are not the same. I interpreted that as distinct based on the clinical picture and/or symptoms. He's obviously specialised in objectifying PEM/PENE.

https://twitter.com/user/status/1131517994364891137


Now Jeff also felt it was untrue and responded and talked about fatigue being an overlapping symptom where Trish explained that overlapping is not especially the same as looking at symptoms that separate CCI and ME from each other.

Michiel responded that fatigue is a symptom of many diseases and that of healthy folks. So fatigue is not really a great example to separate ME and CCI.

So I'm still very curious why you felt that the fact that ME and CCI have symptoms that are distinct from one another was "simply untrue"?? Do you feel that ME and CCI have the exact same set of symptoms?

Thank you ♥️

 

@Nasim Marie Jafry I have read in places where people are experiencing PEM with heel spurs

 

I actually think we can know this in my case (that CCI was the mechanism causing all my symptoms) for many reasons and no, I don’t think we need a study to prove that. Or at least, we are not at a point in our understanding of this disease where we can even say that what we find in one study has validity for any given external case any more than we can say that my case as any bearing on anyone else’s. The evidence available within my own case, however, I find highly convincing to the point that I am more than willing to make the above interpretation. (Others may of course feel differently, even after I have finished telling my story.) What I can’t know is why it happened or how it caused my symptoms. Let me finish writing.

I cannot speak for Jeff and how he presents his story. We are very different in terms of the claims we are willing to make.

As for surgery being a last resort, every surgeon I know of is on the same page about this. This is why I am less concerned that people are going to run out and have unnecessary surgeries. Given the diagnostic process I just went through, I can’t see that happening. I could be wrong and I obviously don’t know every surgeon or every case.

As for the other treatments I have tried yes, those helped, too. Along with Mestinon and Fludrocortisone. I list this all in the “my ME is in remission” piece. I never, ever say in Unrest that mold was causing my symptoms or that a persistent herpesvirus was the cause of my ME (although I was having recurring herpesvirus outbreaks). I have never said this period because I never had any evidence I found compelling enough to support either of these hypotheses. (This should not be that puzzling. We know anecdotally and from clinician statements that many people in our community have MCAS. We also have many people who report developing recurrent shingles or other herpesvirus outbreaks.)

I do find the evidence that in my case, CCI was causing my symptoms and that CCI surgery is responsible for that remission compelling enough to make that statement and no, I have not presented that information in full yet. I do not expect it to be convincing to you but I think most people will find the claim reasonable (i.e., a reasonable person could make that claim based on that evidence, a reasonable person could disagree).

 

Except that I know I no longer have it....

 

I think you make an excellent point. Any diagnosis must go through a process of elimination. Just fitting a diagnosis is just the first step.

One of the biggest challenges we have is that "ME" has become an umbrella term which is unscientific. ME is a distinct patient group which has been defined by Ramsay, CCC and ICC. While they may not be "exactly" the same they are definitely in the same ballpark.

For clarity I think we as a community must start being very clear what we are talking about - ME-Ramsay, ME-ICC, ME-Hyde, ME/CFS-CCC, ME/CFS-SEID or CFS-Fukuda. (I disagree with combining the term ME/CFS but the research using CCC often used that label.)

I prefer the ME-ICC for many reasons, but this situation is a good example of how important it is to have a consensus on how to diagnosis a patient group. Not only does the ICC describe ME, it has a primer that includes testing information that should be performed to rule out other diseases and check for comorbid issues that can be treated.

Getting labeled with a diagnosis is only the first step. I loved your detective analogy and maybe that's why I am keen for us all to focus on following the clues that rule out other diagnosis until we are left with those patients who have "ME" and have had everything else possible ruled out. Of course new science and improved testing will give us more tools, but let's use what we have now to our best advantage.

For many of us time has ruled out a lot of the alternative diagnosis. At 29 years, I'm fairly confident my multiple medical explorations and time passing that did not show another disease have ruled out everything else.

Every diagnosis is tentative until proven otherwise.... so this narrative that "lots of people are misdiagnosed" IMO should be changed to every diagnosis for all diseases are tentative until proven otherwise. Doctors are put in an impossible position by insurance companies. They "must" diagnose based on minimal information in order to move a patient through the system. And patients demand an "answer" to what is wrong with them. So of course doctors tell patients "you have xyz". They should say you have "xyz until proven otherwise".

We as patients can help support our doctors by recognizing they are handicapped in a system that does not provide the tools they need.

In this case one of the tools they need is the IC Primer which needs to be adopted by HHS. Hence the #PwME4ICC petition (at change.org) pushing US Health and Human Services to adopt the ICC could have a positive impact on patient care.

With the primer in their hands they will have better guidance on what testing needs done to rule out other diseases. It includes spinal stenosis... but an updated version should include chiari malformation and CCI.

 

Hi Anil!

The symptom criteria are not “clearly distinct” because the symptoms associated with CCI in the literature, in the concept of cervico-medullary syndrome, and the symptom lists that neurosurgeons use in their practices are not “non-overlapping.” In fact, they overlap with many of the symptoms in the ICC-ME criteria. However, CCI is not a condition primarily defined by symptom criteria. There are no central or hallmark symptoms required for diagnosis. Because of this, it is entirely possible to have CCI and not have PEM (which I imagine is the case for many EDS patients) just as it is possible to have CCI and have PEM.

I don’t think we can know what symptoms separate ME and CCI from each other categorically. No one has studied or thought about this so we just can’t know. What every person can do is self-assess whether they think they have symptoms consistent with CCI such that it makes sense to pursue a diagnosis. This, for example, is the intake form at Dr. Bolognese’s practice: http://www.chiarinsc.com/word/New patient form2xx.doc. The list screens for Chiari, CCI, tethered cord and probably a few other neurosurgical conditions common in EDS (i.e., this is not a CCI symptom list!). I have no idea what he or Henderson or others would consider “distinct” for Chiari v. CCI, let alone ME.

I agree that fatigue is not a great way to separate any condition from another condition! Any discussion of fatigue and CCI in the literature can’t be presumed to refer to PEM because these concepts are different. As such, we can’t know if those patients had symptoms comparable to ours.

To me “clearly distinct” means non-overlapping but if what Michiel meant was “not precisely the same” then no, they are obviously not precisely the same.

If what he meant is that there are symptoms that can clearly distinguish between ME and CCI, that might be true, but I don’t think we know what they are.

 

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.

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.

 

[my bold]
I have the greatest admiration and respect for you @JenB, and immensely pleased for your dramatic improvement. But the bit I've highlighted is what really concerns me, because that is the point many are making here. Time and future research may well show a causal relationship; but it could just as readily show a correlation but with different causation. In the latter case surgery might just be shown to be even higher risk for some pwME, there is no telling at the moment.

 

Thank you @JenB.

Yes, there are many diseases that overlap with each other. So it's really not a matter of if there's an overlap in symptoms there won't be distinct symptoms for each condition. Otherwise we would never be able to separate any disease for that matter.

I definitely mean “not precisely the same”. Again these symptoms make in my opinion a crucial and a distinct difference.

I'm curious if someone like prof. Todd Davenport feels that we can't clearly distinguish between ME and CCI, I was under the impression that we could as CCI has been in the literature for around for a century?

Anyways, thanks for your answer. Good to hear your opinion about this.

 

But all science starts with a story. My story isn’t “science” but that doesn’t mean that it is “unscientific.” I am simply relating what I have seen, experienced, and learned so it can be useful to others, including scientists. I have not made any out of sample (where I am the N=1 sample) claims. The only place that has happened is here. And perhaps Jeff does that, but I am not Jeff.

I’ve always hated recovery stories. People would stand up and proclaim them at screenings of Unrest and to me, they were always red herrings because we have no idea if what they think got them better actually got them better and even if it did, to which patients in what context it would apply. “What we need is science,” I would always say, and try to turn the conversation back to the big picture of building a movement, educating doctors and investing in research. This because the goal, to my mind, has never been any one person’s individual, unrepeatable recovery but building a world where every person with ME (or who could develop ME) has access to a system of diagnosis, treatment, and public health/prevention. I kind of hate that I am a recovery story because I don’t want to be a big red herring, even though I of course am so, so grateful to have recovered. I do think, though, that I need to put this out in the public domain as a) this happened and I don’t want people to think I got better through positive thinking or the power of love or some BS and b) it’s probably going to be more than ten cases before long and so better, I think, to be as specific and transparent as I can possibly be. If in so doing, we can get researchers to quickly explore and help us understand what is happening, all the better. Maybe the outcome is that we understand more about ME in general. Or maybe it’s simply that CCI becomes included in the exclusion criteria. Either way, we should figure this out.

As for not recommending people have surgery, I can neither recommend nor not recommend. Everyone’s case is going to be so different. All people can do is decide whether they want to follow the steps required to pursue a diagnosis and then if they are diagnosed, whether they want to act on it. I hope explaining my own path to diagnosis will be helpful to others as they make their own decision.

Again, I DO NOT recommend anyone have surgery simply because they meet ME criteria. However, I cannot see any surgeon agreeing to perform surgery on that basis!

One aside: I realize it is inherently contradictory to be worried about people getting cervical MRIs if indeed we think cervical stenosis, Chiari and CCI should be a part of the exclusion criteria or differential. If that is the case, then ALL people with these symptoms should be getting cervical MRIs to rule these three conditions out. Every country’s medical system is of course different but at least in the US, this seems like it could be a very sensible practice. Again, I do think establishing a baseline rate of these conditions in our patient population needs to happen first.

 

The reasonable man, the man on the Clapham omnibus, the balance of probabilities, beyond reasonable doubt - these are standards used in a legal context. I'm very surprised to hear them used in a medical / scientific context, when discussing claims / evidence. I have never heard them used in this context before, and am not at all sure that it's appropriate. In fact I'm virtually certain that it isn't.

 

Yeah, there may be symptoms that distinguish ME from CCI and vice versa, but I don’t know what those are and I don’t think that anyone does. Objective testing is the only way to conclusively make a CCI diagnosis.

And to be fair, WE have so many different criteria, none of which are precisely the same. However, I would never describe the CCC and ICC as “clearly distinct.” But now we’re getting into language and semantics...

That isn’t exactly true. We separate diseases with same/similar symptoms all the time not based on subjective symptoms but rather objective testing. This is why breast cancer is now 13 diseases. I don’t think two overlapping clinical entities need necessarily have distinguishing symptoms, at least not in 100% of cases. People rarely present that neatly.

 

Dear Jen,
The important outcome is that a) you are restored, B) you are recounting what happened, but what is now even more important is to get neurologists and ME researchers together asap to evaluate the results that you and Jeff and Mattie and others are living.

This disease is horrific. Your film highlighted it. I watch this here at home non stop: it is unsustainable.

Maybe the surgeries will point to areas of the brain stem that need urgent investigation: I’m not suggestion surgeries necessarily but investigation. Things are moving too slowly for the severe patients and they are having trouble hanging on.

Dr Davis the other day stated that they have not proved the metabolic trap to be incorrect. I don’t know how to add up that and brain stem problems.

Best wishes