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

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.

 

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?

 

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.

 

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

 

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:

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.

 

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.

 

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?

 

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.

 

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.

 

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.

 

sore throats, swollen lymph glands

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.

 

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

 

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

 

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.

 

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

 

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.

 

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.