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

I found another paper, this one by Bolognese and Milhorat , that addresses cranial settling in the context of connective tissue disorders, and talks about upright imaging. It has some good diagrams as well.

The title of the paper is
Syndrome of occipitoatlantoaxial hypermobility, cranial
settling, and Chiari malformation Type I in patients with
hereditary disorders of connective tissue,
And if you search for it on Google scholar there's a free PDF available.
I'm attaching some images that I think are relevant.

 

Thr paper also has a description of the process of intraoperative traction and a diagram. Attached here.

 

To respond to @Jonathan Edwards ' response to me in another thread. I apologize for misrepresenting your position by saying you said traction could not alleviate brainstem compression at all. I did not mean to misrepresent you. I inferred that you thought this because you said traction wouldn't be a useful test.

You recently said: "Traction will relieve brainstem compression by reducing forward-back displacement. The comment I made previously was that I though ti very unlikely that it had anything to do with raising the skull up in relation to the cervical vertebrae (certain not in relation to the head as JenB had originally said)."

And at another point said: "Symptoms of CCI by and large do not change with traction. Even with surgery they mostly do not improve but stabilise. I see no reason to think that traction is a useful guide to diagnosis. "

So I am confused by the contradiction. Are you saying that you think traction could indicate something about brainstem kyphosis in the context of horizontal but not vertical instability? Or just that the effects can be salutory for CCI but not specific enough to CCI to indicate something about the pathology ?

 

Sorry that is a spelling error. It should read "dysphagia," which is difficulty swallowing. Thanks for catching that!

 

Yep! And that change in BDI is I believe what is correcting the cranial settling part, in patients who have it.

Note the change in BDI between supine and sitting.

 

I noticed that. I also noticed that they explore the possible pathology of what is going wrong with the ligaments in that paper. Perhaps the problem is with elastin not collagen ?
Also I still don't understand the category of "functional cranial settling" vs just regular cranial settling but I notice they use this, as well as Henderson.

 

I actually think functional cranial settling was my diagnosis. "Functional" in medicine just means that something doesn't work properly but there is no lesion.

Tethered cord syndrome, for example, is considered a functional disorder b/c there is no actual damage to the cord, it's just pulled tight: https://rarediseases.org/rare-diseases/tethered-cord-syndrome/

I think it does not always need to have a psychological connotation.

At least this is what I have surmised. But this is probably one for Dr. Edwards.

 

It looks from the picture here that they are adjusting the head in three different planes of motion. I wonder if that is true for all patients...I am sure it depends on the measurements and what exactly is required to get to “normal.”

At a minimum, it is definitely not just fixing the head in place.

 

One question i have is why Henderson would only use "slight traction" , like 5-10 pounds, intraoperatively, if often it takes 20-30 pounds in an ict test until patients respond. I guess this could be because od gravity and ict being done upright ?

 

This sounds right. Although the head is about 10 pounds.

 

Another possibility: Henderson fuses with only 5-10 pounds of traction because he doesn't first do an ICT test. If he did use the ICT first, he might choose to fuse with more traction.

Under how many pounds of traction does Dr. B. fuse? This could be a great question to ask Dr. B. and could shed light on Henderson's method, different intraoperative techniques, etc.

 

Puts me in mind of the 'snakeoil' salesmen that we have come into contact with over the past decades. Several of whom, when questioned, would say, 'Oh yes. We plan on a proper clinical trial.' It looked good on their websites but they never did them of course. It would eat into the profit margins. Still, I do hope this team in Spain - especially as it does affect people who are raising funds from the UK - are FORCED to comply with whatever health agency there might be over there, and publish something useful BEFORE performing any more operations on people with M.E.

Edit to add: If all this talk about ME, CCI and surgery continues, perhaps a statement should be sought from the neurologists or their agencies here in the UK. Maybe they can shed some light on why such surgeries are not being recommended at home? Perhaps, as @Jonathan Edwards says above, we don't need a clinical trial at this point, maybe it would be sufficient if neurologists could all have access to the scans and determine if the diagnosis was correct and surgery was warranted in these individuals?

 

This is part of what is hard. Pages of explanation then, “feels like snake oil.”

People have been doing this surgery since the 1960s and in EDS, for twenty years.

I understand that most of the people commenting on this thread are in the UK or Europe and the idea of a private doctor is hard, much less a surgery that is this expensive and not covered under a national health service. I will repeat (because someone on this thread told me they had forgotten) that these surgeries in the US are covered by health insurance, including government-funded Medicare.

I will also remind everyone that Jeff and I both had tethered cord syndrome and had surgery for this condition, too. It is very difficult because you need to first a) have a hunch what might be wrong with you and b) know who to try to get to, but people with EDS have had tethered cord surgery under the NHS and in Germany, under their national health system.

People are also having surgery for Chiari malformation, cervical stenosis, Eagle’s Syndrome, and chronic spinal fluid leaks. There the pattern is the same as with tethered cord syndrome. They have to fight hard to even get the imaging but once imaged, they meet widely held criteria for surgery.

None of the treatment that has helped me is generally available in the UK for people with ME (or EDS). For better or worse, we have a different healthcare system. Please recognize that this is some of the gap.

At least some of this is about fundamental diagnostic confusion. A decent chunk of our community has EDS. I know the hEDS diagnosis has also been doubted here, but I also know at least three people with cEDS (which has a gene) who have been diagnosed since May, including two patients very involved in ME research. They know this disease well but didn’t know about EDS and never imagined they had it. Once you realize someone has a connective tissue disorder, then the clustering of these conditions starts to make sense. We do not know how many people in our community have connective tissue disorders but from what I see with my eyeballs (and what David Kaufman, Ron Davis, and Byron Hyde have seen with theirs) it’s MUCH more than you would expect at random. Hopefully there will be systematic study.

While I don’t meet the criteria for any form of EDS, tethered cord does run in my family. I’ve seen other people recognize this, too.

Anyone who says this is a cure or treatment for ME is indeed selling snake oil. NO ONE in my country has ever remotely said that. They were not even aware of our community of patients until a few months ago!

I do agree, though, that this article is very frustrating and fundamentally irresponsible. The only way for patients to improve from this surgery is if they have CCI/AAI. This is not a generic treatment for ME/CFS, but rather for a specific pathology that some people can have.

 

PS—neurologists in general are very bad at diagnosing these conditions. I do not know why. I think they tend to treat conditions that can be managed with medications, and so are much more focused on MS and Parkinson’s. From what I’ve seen, you need to work hard with your neurologist or skip them entirely and go to a spine neurosurgeon or interventional radiologist, depending on the diagnosis. That may sound “fishy” but this can be true even of some fairly standard (if “rare”) diagnoses. For one, most neurologists only order brain MRIs and if they can’t find anything wrong, they are done with you. That has been my universal experience. They are not skilled in diagnosing many of these conditions, either by ordering the right imaging or even doing the right physical exam.

 

Because this is not snake oil, even if it “feels” like it is. I am not offended—was just hoping for some substantive engagement with the recent posts.

Edit: When I was here in May, so much substantive engagement was ignored or summarily dismissed, so I am sorry if I had a hair trigger reaction. The frustration lingers...for all of us. Will try to forget what happened then and since and keep an open mind!

 

My experience with a neurologist back in 1992 was positive. He ordered a lot of blood work to r/o other illnesses and sent to the hospital for various tests for vertigo. I was never sent to a spine specialist/surgeon, thank goodness.

 

Could you update this with the information from the largest metastudy @Michiel Tack?

This one, 2274 surgeries, zero deaths:

https://academic.oup.com/neurosurgery/article-abstract/67/5/1396/2563905?redirectedFrom=fulltext



You might also note in the n=779 study you quote that 81.58% of patients had improvement in neurological outcomes. This is a little more concrete than the Goel study, about which you remark that outcomes are “generally good.”

Many surgeries carry some risk of death but the n=2274 study had a 0% death rate and the n=779 study, 0.06%. I think this is more helpful than saying “someone died” because of how easy it is to see a single death but not the N.

It is also worth noting that the complication rate in EDS patients is likely much higher due to the connective tissue disorder but that it is unknown what it is in people with ME who do not also have EDS.

I have no idea why Mattie was under anesthesia for 14 hours. Jeff and I were both under for 8. That seems to be the typical time.

Also, you can choose to use your own rib but I think cadaver bone is the standard of care, at least among US surgeons. I don’t personally know anyone who had a rib harvest.

It’s also worth noting that “informed consent” prior to surgery (at least in the US, cannot speak to Barcelona) goes much deeper than all this. If you actually have surgery, you’ll learn and be informed of all this and more.

And re: “costs a lot of money” it might be worth nothing (if this is an international board) that it is generally free or at minimal cost if you live in the US and have Medicare or private insurance.

Finally, I am not sure that “distinct from ME/CFS” is exactly right. Some symptoms overlap, as is clear from the Venn Diagrams. I had neurogenic bladder due to tethered cord syndrome. The symptoms of neurogenic bladder are mentioned in the ICC, for example. As are gait abnormalities, lack of coordination and dizziness. Still other CCI symptoms like tinnitus and parasthesia are mentioned by Ramsay.

I absolutely had symptoms of nerve compression, like numbness, that should have been investigated more deeply. However, there is enough overlap in symptoms that it should not be surprising how I could be diagnosed with ME by seven different US specialists over the years.

Thanks!

 

That’s so fortunate. Mine diagnosed me with conversion disorder when I actually had craniocervical instability and tethered cord syndrome. So I walked home, collapsed and was bedridden for the next several months.

Obviously, if you did have a spine condition you would want to be referred to a spine specialist. I assume this was properly ruled out. You are fortunate to not have problems with your spine—they can be quite debilitating!

 

@JenB I think the big difference was that I was referred to the neurologist by an ME specialist who had 20 years experience and highly recommended him.