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

[JenB]

No absolutely not. That is what I am begging those involved to do - get some imaging and publish it. What I consider unethical is referring for surgery.

And I don't really buy the idea that nobody is being referred for surgery on the neck to cure ME. Somebody is referring for surgery. Apparently the surgeons are too busy to know what is going on. So presumably the physician thinks that PWME might benefit from surgery, without having documented the imaging evidence that would suggest that this is a sensible path to follow. If radiologists have fancy machines to do standing up MRI to show CCI they are quite likely to report CCI, just like the path labs that like to report strange autoantibodies. Maybe the quality control is good but reading Henderson';s review suggests to me that it is all pretty vague. He says that the imaging is not in itself diagnostic without signs. What were the signs?

I don’t think we can have it both ways—that on the one hand, I never had ME to begin with but rather CCI, and therefore my case is of no relevance to anyone else AND that ME patients who have CCI shouldn’t be referred for CCI surgery if they have imaging that shows CCI because they have ME.

In the latter case, surgery is always a complex choice because there is so little research on the effects of anesthesia and surgery on ME patients (this is why I delayed my thyroidectomy for five years!) but that is a choice every ME patient who is given surgery as an option must face, no matter for what diagnosis.

 

[ME/CFS Skeptic]

There is some overlap between this symptom cluster and ME (how great an overlap depends on which of these neurosurgeons you talk to, which I acknowledge is far from encouraging).

Thanks for sharing your story Stewart.

In just want to pick into this because you keep saying that there is an overlap between ME and these symptoms, but I haven't seen this in any publication or even video. The cervical medullary syndrome consensus definition from 2014 that Henderson uses in his publication seems quite different from ME. And since it's a consensus definition one would assume this is the most common description. The video you gave me of Ulrich Balzdorf's talk, "Symptoms and signs of cervical medullar syndrome in Chiari patients", lists the following symptoms: "Headache, visual symptoms such as blurred vision and double vision, hearing-related symptoms such as tinnitus and hearing loss and balance difficulties."



That doesn't seem like ME/CFS either. Perhaps you interpreted this differently because Balzdorf said that symptoms such as headache were "exertional". That reminds us of ME/CFS but given the list of symptoms above Balzdorf most likely meant changes in posture - not the post-exertional malaise and relapses that are typical of ME/CFS. Could you give me another reference that does describe cervical medullary syndrome as something that looks like ME/CFS?

 

[ME/CFS Skeptic]

That brings me to another point.

@Jeff_w and @JenB you both said you cannot in good consciousness agree with the following statement "Patients with CCI/AAI suffer from symptoms that are clearly distinct from the symptoms of ME/CFS" because you believe to be inaccurate or simply untrue. Yet when I ask about a reference that describes CCI as something looks like ME/CFS I get no response. According to Jeff "Known symptoms of CCI/AAI as described in the scientific literature include POTS, profound fatigue, sensory overload, weakness." If that is true that it would be quite easy to show me not one but several publications on CCI where this is the case.

I saw that the MEpedia page lists some ME/CFS symptoms (chronic fatigue, orthostatic intolerance, heat intolerance etc.) as typical of CCI. The symptoms that are characteristic of CCI in the scientific literature such as severe neck pain and neurological complications such as numbness, balance problems, paralysis, vision problems etc. are only mentioned as "other symptoms" in the MEpedia article. As source MEpedia doesn't refer to a scientific publication but to a webpage of the zebranetwork who in their turn took the info from a blog post of the Pain Relief Foundation. I've contacted the Pain Relief Foundation. They thought CCI is almost exclusively diagnosed in EDS patients - which is in contrast to Hendersons 2016 review which said that researchers have only recently begun to look at CCI in this patient population. The info they provided focuses on CCI in patients with EDS. I've asked where they got the list of CCI symptoms from and they kindly responded and said it was a mixture of two sources (which can be found if you click on "refrences" on the blog post)

The first is the influential Milhorat et al. 2007 paper which I already mentioned at the beginning of this thread and an accompanying video. Yet the patients in this study had not only CCI but also Chiari Malformation and EDS. So it's very likely that symptoms of chronic fatigue, POTS etc. were due to these comorbid conditions because these are known to cause symptoms that resemble ME/CFS.

The other source is s a video of a presentation by Henderson back in 2012 where he does list those symptoms under CCI.


Yet in his actual publications (which are also more recent) Henderson describes CCI quite differently. In a 2018 publication, he describes ten adult subjects with ventral brainstem compression who were treated with fusion and occipito-cervical stabilization. "Common symptoms included headache or neck pain, memory loss, hypoesthesias or paresthesias, clumsiness with frequent falls, imbalanced gait, and weakness in the upper or lower extremities." In a 2017 review, Henderson describes the symptoms of CCI an associated brainstem compression as follows: "weakness of the limbs hyperreflexia and pathological reflexes (e.g., Babinski, Hoffman’s sign, absence of the abdominal reflex), paresthesias, and a plethora of other symptoms—including sphincter problems, headache, neck pain, dizziness, vertigo, dyspnea, dysphonia, altered vision, and hearing, syncope, emesis, altered sexual function, altered menses, and gait changes."

It should be noted that even Gilete, the doctor from Spain, describes CCI on his website in terms that do not resemble ME/CFS: "Headaches, Neck pain, Double vision, Memory loss, Dizziness, Vertigo, Ringing in the ears, Speech difficulties, Difficulty swallowing, Sleep apnea, Snoring or frequent awakening, Choking on food, Numbness in arms or legs, Unsteady walking, Clumsyness, Weakness in arms, hands or legs." In a video presentation on craniocervical instability, that is available online, Gilete describes one of his patients with a spectacular recovery. The 45-year-old women had constant breathing problems, pain the neck and behind the eyes, swallowing and speaking problems and facial paresis and hemiparesis. (minute 3.50).



So I would kindly want to ask whether Jeff or Jen could direct me to the scientific articles that do describe CCI as something that closely resembles ME/CFS.
If those papers do not exist, then it might be a good idea to include a description of a "typical CCI case" on your blog posts or website. That would help readers to consider whether they might have CCI or not and prevent unnecessary worrying.

 

[Kelly M.]

I think you might have misread that: on the PR poll as it stands, 18 have tested positive for CCI/AAI, and 11 tested negative. Total of 29 people.

A typo, doesn’t really make any difference though.

 

[Jeff_w]

Hello @Michiel Tack -

You have repeatedly stated on multiple social media platforms that there are no scientific articles that describe CCI as resembling ME/CFS.

You wrote:

So I would kindly ask whether Jeff or Jen could direct me to the scientific articles that do describe CCI as something that closely resembles ME/CFS.

If those papers do not exist, then it might be a good idea to include a description of a "typical CCI case" on your blog posts or website. That would help readers to consider whether they might have CCI or not and prevent unnecessary worrying.

Here are some articles that demonstrate CCI symptoms and ME/CFS symptoms overlapping.

In Henderson’s most recent article from 2018, he lists the symptoms of his CCI patients. 100% of those pre-operative CCI patients had fatigue.

In addition to the article mentioned above, here's a second article: This case series examines CCI patients' pre-operative symptoms, and how they resolved after fusion surgery:

• 37 year old female: Fatigue prior to fusion.
  • After fusion surgery: Resolution of all symptoms.
• 55 year old female: Fatigue prior to fusion.
  • After fusion surgery: Resolution of all symptoms.
• 65 year old male: Fatigue prior to fusion.
  • After fusion surgery: Resolution of all symptoms except for preexisting left-sided weakness.
• 58 year old male: Fatigue prior to fusion.
  • After fusion surgery: Resolution of all symptoms.

In yet a third article, pre-operative CCI patients answered questions on a "Brainstem Disability Index." Symptoms of brainstem compression include: "Gets tired very easily," "memory loss," and "speech issues." Those symptoms are consistent with fatigue, brain fog, and word finding difficulties -- in short, compatible with ME/CFS

I know you have read each of these 3 articles above. You’ve cited each one of them, many times. Yet, you have continued to argue that there's no evidence that CCI symptoms and ME/CFS symptoms overlap. However, the evidence states the exact opposite. How did you miss this critical information?

Furthermore, research abounds that in addition to fatigue, POTS and brain fog are central to CCI. This is entirely consistent with ME/CFS.

To further bolster your position that CCI and ME/CFS don't overlap, you mention Dr. Batzdorf’s presentation titled, "Symptoms and signs of cervical medullary syndrome in Chiari patients.” You stated that this presentation lists symptoms that aren’t characteristic of ME/CFS. You then concluded that this research presentation fails to support that CCI is at all consistent with ME/CFS.

The problem here is that you apparently didn’t notice the topic of the presentation. The entire presentation was about Chiari patients, not CCI patients. While these two groups can overlap, these are distinct patient populations.

Also: You helpfully attached a slide from one of Henderson’s presentations, in which he displays a list CCI symptoms. This list of CCI symptoms includes: Fatigue, POTS, cognitive changes, and memory loss. All of which are consistent with ME/CFS.

Here is that slide:




Please consider this, Michiel: You are repeatedly asking me, Jen, and Mattie to place disclaimers on our own work. You are even going so far as dictating what these disclaimers should say. Even more troubling, the disclaimers you’re demanding are inaccurate.

You've said that adding these disclaimers would “prevent unnecessary worrying,” and that Jen and Mattie and I are scaring people. However, I’d respectfully suggest that the reverse may actually be true. In my opinion, it is you who is causing people to worry unnecessarily.

You are stoking people's fears by repeatedly saying there’s no overlap between ME symptoms and CCI symptoms. This is inaccurate.

In 2017, after 3 years of intense research, I figured out the connection between CCI and ME/CFS. I approached Dr. Kaufman (my ME specialist), introduced him to the concept of CCI with research articles in hand, and informed him of my thoughts about this. He studied the articles with interest. Thank goodness for those few doctors who are intellectually curious. I then introduced Dr. Kaufman to my expert neurosurgeon.

Having done a risk-benefit analysis, I chose to undergo fusion surgery. My ME/CFS is now completely gone. I’m getting my life back.

I posted my fusion journey on Phoenix Rising. Jen and Mattie read of my journey and soon followed suit. They have both improved after their fusions. Jen, who is further along post-surgery than Mattie, has publicly stated that she is in complete remission from ME/CFS.

I am Patient Zero. Jen, Mattie, and I are the vanguard of ME patients who have undergone fusions.

Besides the 3 of us, there are now 4 additional ME patients who have had fusions -- a total of 7 so far. We have all improved. The additional 4 have all agreed to be interviewed for my website.

One of those 4 found me on this very board! She was ill with ME/CFS for decades. She has told me she's now in complete remission after her fusion. I will be interviewing her after June 1st, as I interviewed Mattie. More to come.

 

[roller*]

excerpt from matties interview.
would have thought, that especially tinnitus should have resolved ?

Well all in all, as of this early period post-op, I would say I have improved from severe ME to moderate ME:

• I still have exercise intolerance, though less so.
  
• I am still limited in what I can and cannot do, though I can do more than before.
  
• I still feel sick and weak, though this has improved.
  
• I still have to pace myself carefully throughout the day, but the payback when overdoing it is much less severe.
  
• I still feel like $*** upon waking up in the morning. Even though my sleep architecture has improved, I still feel like my batteries are not charged.
  
• I still have terrible tinnitus.

https://www.mechanicalbasis.org/interviews.html

Dr. Gilete was definitely not pushing me to have surgery. He told me:

“You have CCI and AAI. I really think you could benefit from surgery so I will offer you this option. I cannot guarantee any results but I think there is a high probability of improvement. You as the patient have to weigh the possibility of improvement of symptoms and quality of life over the risks of surgery and the debilitating nature of a cervical fusion. It is ultimately your decision.”

Like you and Jen, I had a posterior fusion stabilization from C0-C2: Skull - Atlas – Axis.

 

[Alvin]

excerpt from matties interview.
would have thought, that especially tinnitus should have resolved ?

I am reminded that someone (Dr Klimas, someone else?) had once said in a video or interview that ME and severe ME are almost two different conditions. What if for example you have ME but when it leads to enough muscle loss that you end up with CCI hence become severe?
Extending this logic once you treat the cause of the escalation you end up back at moderate.
Supposition of course.

 

[obeat]

If a signalling problem in the brainstem/ brain contributes to ME , how do we investigate it and who's going to do it? Surely this is the realm of neurology?

 

[Trish]

What if for example you have ME but when it leads to enough muscle loss that you end up with CCI hence become severe?

As I understand it, CCI results from problems with the ligaments that prevent too much movement between skull and atlas (nodding) and in AAI rotation between atlas and axis (turning the head from side to side). It is laxity or damage of some sort to these ligaments that allows too great a range of movement that creates pressure on the brainstem and the specific symptoms of CCI and/or AAI, not anything to do with muscles.

Though I wonder whether someone with mild ligament laxity or damage and with strong muscles supporting the joints means less symptoms because they can use their muscles to help prevent too great a range of movement, and when muscles weaken with long illness or age, the symptoms of CCI/AAI might become more problematic. This paragraph is speculation.

 

[Jeff_w]

Re symptoms.
one for ME that does not appear to be on any of the CCI lists is sore throats, swollen lymph glands

I had those above symptoms. They resolved with my fusion surgery.

When one's autonomic nervous system (CCI / brainstem compression) isn't functioning properly, the immune system can also malfunction.

I had a post-viral onset of ME/CFS, as did Jen.

 

[Alvin]

As I understand it, CCI results from problems with the ligaments that prevent too much movement between skull and atlas (nodding) and in AAI rotation between atlas and axis (turning the head from side to side). It is laxity or damage of some sort to these ligaments that allows too great a range of movement that creates pressure on the brainstem and the specific symptoms of CCI and/or AAI, not anything to do with muscles.

Though I wonder whether someone with mild ligament laxity or damage and with strong muscles supporting the joints means less symptoms because they can use their muscles to help prevent too great a range of movement, and when muscles weaken with long illness or age, the symptoms of CCI/AAI might become more problematic. This paragraph is speculation.

I don't know, maybe its ligaments instead of muscles.
I do know i am slowly seizing up, my chiropractor can only do so much but as the ME gets worse i am likely going to be in much more pain.

 

[Barry]

As I understand it, CCI results from problems with the ligaments that prevent too much movement between skull and atlas (nodding) and in AAI rotation between atlas and axis (turning the head from side to side). It is laxity or damage of some sort to these ligaments that allows too great a range of movement that creates pressure on the brainstem and the specific symptoms of CCI and/or AAI, not anything to do with muscles.

Pure speculation on my part here, but worth airing. I wonder if someone sleeps with very poor "head posture" could cause some issues over time. If, for example, they sleep with very unsupportive pillows, or otherwise putting undue strain on their neck.

 

[Sly Saint]

sore throats, swollen lymph glands

I had those above symptoms. They resolved with my fusion surgery.

I had a post-viral onset of ME/CFS, as did Jen.

When one's autonomic nervous system (CCI / brainstem compression) isn't functioning properly, the immune system will also malfunction.

See the above link.

so why, if it is also a symptom of CCI ie immune system disfunction, does it or similar not appear in a CCI symptom list.
(not being agressive here, just trying to understand)

eta: my point being is that if it is common for people with CCI to also have problems with the immune system then surely more of them would present with these symptoms in addition to the ones listed.

 

[Trish]

@Jeff_w thank you for the update.

One thing that puzzles me with all this talk of symptom overlap. Surely there are lots of diseases with symptoms that overlap with ME. That's why diagnosis has to include checking whether we have one of those other diseases, not ME. For example, MS has some overlapping symptoms. But to distinguish whether someone has ME or MS, the key thing is to look for the symptoms that don't overlap.

So also with CCI/AAI. In order to tell whether someone has these, it is the specific things that identify the person as having CCI that are important in diagnosis, not the common things that occur in lots of other conditions, like fatigue.

As I understand it, to be diagnosed with CCI/AAI sufficiently bad to need surgery, not only does there have to be specific measurements on scans, but also neurological signs such as numbness and weakness in specific areas and specific types of headache, some breathing and swallowing problems etc. These are not part of the diagnostic criteria of ME.

I think the point Michiel and others are making with the suggestion of disclaimers, is that people who simply fit the diagnostic ME criteria, but don't have any of these specific neurological signs as well, are unlikely to be candidates for this surgery.

Surely it is reasonable to ask those writing of their experiences with this surgery in the context of ME to point out the need for neurological signs and symptoms for diagnosis. Rather than pointing out the symptoms that overlap, it seems to me important to say that the individual having the surgery had neurological signs that were not part of ME criteria that led to the diagnosis.

This would make it clear to those of us who don't have any such signs or symptoms that this treatment is unlikely to be relevant for us, and nor are responsible neurosurgeons going to offer us the operation. Then we can simply read the articles with interest and good wishes for the people concerned while not having our hopes and/or fears raised that it applies to us too.

 

[Amw66]

I don't know, maybe its ligaments instead of muscles.
I do know i am slowly seizing up, my chiropractor can only do so much but as the ME gets worse i am likely going to be in much more pain.

Same for my daughter. very tight neck. Tendons/ attachments can look / feel like bone at times.

 

[Tilly]

@Graham,

I agree with your caveats about the maths but I think my original point stands. Maybe I was not clear.

The whole business of the link between 'EDS' and ME is shrouded in mystery. The ME research community is snatching around for any tiny clue yet there appears to be an elephant in the room in the form of a link with a genetic connective tissue condition that if real would provide a massively powerful lead for starting research. But as you say, there are no reliable data.

There are three groups of people who might be said to have 'EDS' with symptoms of ME. The first would be people with recognised genetic variants - now identified in most of the extra-articular EDS types. The puzzle is that several genetic screening studies of PWME have now been done and there is no mention of these EDS gene variants coming up. The only gene found recently was a prolyl hydroxylase 4 variant. PH4 is involved in collagen synthesis but it is not an EDS gene so it does not support a link to EDS. We have nearly 2000 members here and my impression is that not more than maybe two or three families are documented with EDS gene variants. Moreover, these variants will be quite a bit commoner than the EDS type itself if they are recessive or partial penetrance.

The second group would be those with clinical signs of extra-articular EDS type - like the woman in the photo with skin folds or someone with cardiac defects. My impression is that these extra-articular types are not particularly associated with ME though. The tales in the literature are of a link to type III (articular) EDS or 'hEDS'. There have never been any threads here on what to do about your skin folds or mitral valve disease. From what I can glean it seems likely that maybe a handful of people here may have extra-articular EDS features but even there I am not sure. So many of these things are common enough in the general population anyway.

The third group are those with hypermobility, who if they have chronic pain and fatigue will be diagnosed as EDS by private physicians with 'an interest in EDS'. The sad thing is that pretty much all private physicians with an interest in EDS have followed the makeshift approach to diagnosis that I decided not to get involved in. What Jen Brea calls the 'EDS space' is almost entirely populated by physicians who do not really know what they are talking about - like the Swedish people we have seen on a recent thread.

I have developed huge respect for PWME, and their intelligence and tenacity in trying to get at the truth. ME is a real illness that destroys lives. I can see no plausible reason why any form of EDS should cause the core symptoms of ME and what I can see of the story of a link between EDS and ME looks more like a phantom than an elephant. There are no data worth taking seriously.

Again I must say that I think it entirely plausible that neck fixation may induce remission in ME but that is not necessarily anything to do with CCI or EDS or anything similar. The current situation worries me greatly but maybe it will lead us to a genuinely useful understanding if we continue to argue it through. After all, there must be a truth in there somewhere.

There is a new tool Kit for GP
https://www.rcgp.org.uk/clinical-an...toolkits/ehlers-danlos-syndromes-toolkit.aspx

I also find this interesting but haven't had time to digest it all yet https://medicalxpress.com/news/2019-05-mast-cells-crucial-osteoarthritis.html

 

[Mij]

I had a post-viral onset of ME/CFS, as did Jen.

Post viral or did you have persistent EBV? I read on your Medium that EBV disappeared after surgery and so did Jen's CoxB virus.

 

[Alvin]

Same for my daughter. very tight neck. Tendons/ attachments can look / feel like bone at times.

Sorry to hear this
My chiro was saying there is only so much she can do, for now things are stable but long term the outlook is not good

 

[Jonathan Edwards]

There is a new [hES] tool Kit for GP

I would be interested to know what @WillowJ thinks of this.

WillowJ and I have been debating the merits of various approaches to hEDS. One of the aspects of the new definition of hEDS that does seem sensible to me is a requirement for multi system involvement and various pointers towards something that might be more homogeneous in the way that the EDS types with genetic markers are.

The downside of the new system is that it is very likely to be interpreted rather loosely by clinicians so that about 100 times too many get the diagnosis.

I would also agree with WillowJ that it makes no sense to relabel just old fashioned hypermobility, which is present in one person in ten, with 'hEDS'. And I fear this is exactly what this toolkit is doing. This is the muddle that Rodney Grahame passed on to rheumatologists for so long. To me it is the worst of all worlds and totally unhelpful but I wonder what WIllowJ thinks.

The say the old hypermobility syndrome is part of the spectrum of hEDS but if anything I would say it is the other way around.

 

[Jonathan Edwards]

The interest in mast cells in arthritis really goes back to a paper by Adrian Crisp around 1994. But nothing has ever indicated that mast cells have a special involvement in human joint disease - it is just that they are present in all connective tissues in bucketloads and are always caught up in inflammation. If you remove them from mice the inflammation is likely to be less but that tells us nothing about human disease.