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

@Jonathan Edwards Thank you for taking the time to respond. I have a couple other questions I was hoping you could answer. Would you agree/disagree that when CCI occurs without blunt force trauma most neurosurgeons will not test for it or treat it? Is a connection between EDS and CCI an accepted or contested idea among doctors? Which physical signs would you consider most important in diagnosing CCI?

 

I agree its problematic. I believe I am not alone in taking the view that this kind of fatigue is probably part of an immune response which is common to many diseases and conditions because it is part of us and how we respond to immune challenges and becomes chronically active in any subtype of ME. If we are classifying cases with a Venn diagram I think that would be the big circle labelled ME, with subtype circles all around the edge half in and half out, linked to various causes.

From that perspective a hypothetical fatigue response would be a common factor which we need to understand before we can discern different contributory factors and symptoms leading to its chronic activation.

Which is why IMHO it might be informative if we can find a molecular signature for the measurable cell stress response that Ron and three other teams have detected, conveniently blogged by Simon McGrath.
https://mecfsresearchreview.me/2019/04/25/something-in-the-blood/

Ron Davis said at the Hampshire College panel that his work on the nanoneedle results is on a back burner due to limited resources and too many avenues to pursue. You cannot blame him for deciding to prioritise particular avenues because we need to understand them all and we all know his concern is sincere. Even so I do hope there might be others out there who can take the investigation of the blood factor forward as I personally believe there is a chance it could be the key to characterising any hypothetical signalling system which would have to exist to coordinate any hypothetical fatigue response and thereby could be a clue to help understand the hypothetical response itself, hypothetically speaking !

That panel is an interesting watch, thanks whoever linked it, here it is again. Ron discusses diagnosis as an obstacle, which IMHO is a key issue (timecode soon after 1:28:08 -ish).
https://forums.phoenixrising.me/thr...r-unrest-at-hampshire-college-feb-2019.76446/

 

I would be interested to know if POTS is the common factor in these cases.

 

Absolutely disagree. All the cases of CCI I dealt with were non-traumatic. Neurosurgeons are not usually the people to make the diagnosis. Usually a physician is involved first. Non-traumatic presentation of CCI is common and routinely diagnosed.

I am not sure about that but I would expect a small proportion of people with true EDS (the 1 person in 5000 who has a dominant gene defect in collagen or tenascin) will sometimes get cervical problems. I would expect these people to have obvious signs of laxity elsewhere. Graham noted that about six operations for CCI are done annually in the UK for EDS (out of hundreds) so my rough estimate is that about 1% or people with true EDS need neck surgery at some time.

Evidence of loss of sensation in the limbs in a long tract pattern - hands particularly - and evidence of pyramidal tract damage in terms of unduly brisk reflexes, clonus, Hoffman's sign and extensor plantar response. Thos would be the simple ones. But a neurological problem is always diagnosed on the basis of presence and the absence of all signs in the neurological examination taken together in a specific pattern for that condition. Bulbar dysarthria would be another suggestive sign.

 

@Jonathan Edwards above mentions symptoms (brisk reflexes, positive Hoffman's etc) that would lead (hopefully) to further testing for patients.
During the development of the NIH Common Data Elements for ME/CFS (CDEs) I pressed hard for Hoffman reflex testing to be included in Baseline testing. It's a straightforward, no cost test that takes perhaps a minute to do both hands and could help better identify patients with neurological issues.
The group decided against including it.
However, the CDEs are to be revised periodically and I hope that this test will be included soon. (Note - I do not know who will be doing the revisions.)

 

I would be against an isolated Hoffman. It is positive randomly in a small proportion of normal people. A Hoffman test should be done as part of a complete neurological examination and for me is never worth doing unless the major reflexes are equivocal. So I would agree with the NIH group there.

But of course someone presenting with symptoms of ME should have a full neurological examination.

 

What would that look like? Wondering if I've ever had one.

 

To clarify - I am not saying that the Hoffman test is diagnostic --- but a positive Hoffman could be an indication that further neurological testing should be done - beginning with looking at briskness of reflexes and proceeding from there.

 

It is generally agreed that it should be the other way around. If the reflexes are brisk then an additional positive Hoffman may indicate that the briskness is pathological. A Hoffman is an extremely poor screening test because it is very often negative when there is a pyramidal lesion. In other words it has low sensitivity.

 

It starts with an assessment of mental state. That can often be worked in to initial conversation but tends to include asking questions testing memory and orientation. That is followed by an assessment of cranial nerves, including vision, smell, eye movements, facial sensation and movements and gag reflex. Following that the limbs and trunk are tested for power, reflexes, sensation, and co-ordination and balance is tested with Romberg's sign. (in brief.) A full examination need not take more than about two minutes if nothing is found on primary screening tests.

 

Rowe, who tests for it and other reflexes at each appointment was also in favor of Hoffman testing being part of the Baseline.

ETA I think his experience with this and(and paper on) patients with cervical spine stenosis may be worth taking into account.

 

Thanks - that's very interesting. I don't think I've had all of that done.

 

Just dropping this here...

https://twitter.com/user/status/1135220523057782784

 

But everyone with concerns is just overreacting.......

It’s all deeply worrying for me.

 

As I said before; no way I will let them cut there.

 

Thanks for mentioning this. I had wanted to bring it up myself but was worried it would sound like I am dismissing Jen's ME/CFS diagnosis. That's not my intention, I'm just curious if it is possible that her thyroid cancer was causing a paraneoplastic syndrome presenting with similar symptoms to ME/CFS?

From my very limited understanding, the symptoms caused by paraneoplastic syndromes can resolve when the cancer is successfully treated, but how long would it take for the symptoms to disappear?

It sounds like paraneoplastic syndromes are rare with papillary thyroid cancer, but I've only done a very very brief google.

This case report is probably irrelevant, and obviously it's just one case, and is very speculative, (and I read that Jen has been diagnosed with Hereditable Disorder of Connective Tissue, not Mixed Connective Tissue Disease) but I thought it was interesting.

Mixed Connective Tissue Disease and Papillary Thyroid Cancer: A Case Report

CONCLUSIONS: We theorize that: 1) MCTD may have been a primary diagnosis complicated by PTC, or 2) MCTD may have been an initial presentation of paraneoplastic syndrome of silent PTC, because her symptoms of MCTD significantly improved after total thyroidectomy. To the best of our knowledge, this is the first case report to associate MCTD with PTC. It highlights the importance of maintaining a high index of suspicion for thyroid malignancy in MCTD patients.

https://www.amjcaserep.com/abstract/index/idArt/894176

Is a PNS in Jen's case in anyway plausible?

 

I just want to say, before I say anymore, that I have the upmost respect and admiration for @JenB, at the way she has fought to get awareness and understanding of what it’s like to suffer with ME, despite being really poorly herself. I also understand how she would want to let as many people know about her recovery and that she thinks her surgery is what healed her.

However, whilst I agree CCI needs to be looked at further to find out how and why Jen and @Jeff_w recovered, I worry about the dangers of such drastic measures of unnecessary surgery on desperate vulnerable people, who will stop and nothing to find a cure. One thing I haven’t seen mentioned on this thread, and forgive me if I missed it, is parents seeking cures for their children. It’s very risky and should be left well alone until more research is done. You can’t just go by personal accounts, it’s needs to be done on a much bigger scale in a trial.

I’m quite a sceptical person, and like figures, numbers and scientific proof from studies. But there are so many people out there that believe everything they read without the evidence and that’s the worrying part.

 

I spent a bit of time looking into the CCI/AAI diagnostic procedure used by the CCI/AAI neurosurgeons, which a patient would have to satisfy before they consider surgery.

 

You are right to a point. I wasn’t saying that CCI wouldn’t be present just that I feel that not all cases would need the surgery as there are different severities. My concern is that just because it’s present doesn’t mean that risky surgery is the answer, when patients would risk it thinking It would cure there ME symptoms.

 

Of course there’s nothing wrong with spreading this message as long as you have a disclaimer