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

I have not had time to read Jen Brea's story in full . However, I think some of her starting facts may be wrong. Looseness of craniocervical ligaments does not cause the skull to drop as far as I know. The skull is bone and sits on the bone of the first cervical vertebra (atlas). Since there is no gap, loosening of ligaments is not going to make the bones any nearer.

I doubt that craniocervical fusion raises the skull up in any way. It just fixes it to the atlas and axis. During the years that I referred patients for surgery like this nobody ever suggested that the skull was raised up.

 

It is truly bizarre, from where I sit, to witness all this unfold. I was diagnosed in Glasgow in an NHS neurology clinic in early 1984, my case 'classic ME', as documented by Melvin Ramsay and Peter Behan after a Coxsackie outbreak in west of Scotland. I have no idea what Jen Brea has, but I have genuinely thought from Day 1 that we do not have the same illness. Everything Jen has written since her successful fusion surgery - as curative for 'ME'- pretty much confirms that. Just to add, back in 1980s in UK, pre-Wessely, when you were diagnosed with ME, it was not referred to as RamsayME, only ME. RamsayME would have been a tautology, as it was the only ME there was. RamsayME is now a necessary descriptor as there has been such proliferation of definitions of ME since then.

Edit - typos

 

I know little more about this. But I found this reference (Young et al. 2014) which states:

The study you quoted is a bit old (from 1980) and in the full text it explains that cranial settling:

So I assume weakness or disruption of the ligaments is part of the causes of cranial settling (perhaps a necessary but not sufficient cause?, I don't know).

 

One particular ligament, and as far as I know only one, when ruptured, will cause settling of C1 on C2 with the peg of C2 passing up towards the brainstem. This perhaps the key reason for operating on RA cervical spines. But this is a highly specific problem seen in RA related to bone erosion where the ligament is attached. It is the transerve ligament of the body of C1, which completes a ring around the peg of C2.

If this was the problem for those recently having cervical surgery in the context of ME I think we would know about it because the radiographic features are barn door and not discussed in terms of ligamentous instability. The ligament has actually failed completely and that is secondary to the fact that the bone has also dissolved away.

 

Do I summarize your view correct as: rupture of ligaments can be part of the process causing cranial settling, but for that to occur one would see damage to the bone as well?

 

That is the situation in RA. Violent trauma might rupture the ligament I guess but I would expect there to be a fracture with it in most cases. The main point is that the position of the peg of C2 would be clearly abnormal. All the discussions I have seen in relation to CCI in the context of ME or EDS talk about a decreased angle between the anterior wall of the posterior fossa (skull base) and the vertebral column, which is something much more subtle.

 

Furthermore, Jennifer writes in her Medium piece: 'It is, nonetheless, a story of which I am uncertain, that has some significant gaps, and that I will never be able to prove in my specific case, even if the scientific literature one day validates aspects of it in general.'

But then later says:

'What is the mechanism or ongoing damage that prevents the vast majority of us with ME from ever recovering? ... How does that mechanism cause the cluster of symptoms we call ME? I know that in my case, the ongoing, post-viral damage was damage to the ligaments in my neck since that is what caused my craniocervical instability and because treating that instability resolved my post-viral symptoms.'

How does she *know* the ongoing postviral damage was to the ligaments in her neck? How can she be certain?

She is contradicting herself in her article.

 

I don't think Jen ever claimed fusion surgery was necessarily curative for ME. I reckon she has only stated she is currently in remission from the condition she had, which was diagnosed as ME. We don't have a clue about the actual cause or disease process is in ME, which means we cannot rule craniocervical instability or any other structural brain condition out. This is how Ramsay describes a typical onset of ME/CFS in his diagnostic criteria:

This is how Jen Brea describes her 2011 onset in her newly released blog post:

I think it's too easy to simply dismiss this case and claim Jen didn't have ME to begin with now the she recovered. I spoke with my doctor recently and he was well aware of this "CCI meme" going on and was going to discuss it in some upcoming conference. I think we will all be a lot wiser regarding this matter in a year or two.

 

Inter

I agree, yes, we do not understand the mechanism of ME, but I think we can be fairly confident that Ramsay and Behan were not looking at CCI. And I will simply never understand how fusion surgery can resolve RamsayME, nor put it in remission, whatever you want to call it, Jennifer is clear that surgery has reversed all aspects of her illness

Yes, low grade fever is referenced by Ramsay - and also Dr E Dowsett in a table in Ramsay's book describes Coxsackie B4 - which Jennifer thinks was her onset virus - as being associated with low fever, less than 101 - yet Jennifer has frequently described her 'triggering' fever as v high - 104 F. I believe she also attributes her illness to previous exposure to black mould, which she has described elsewhere. Dowsett table below.



Also, in an earlier Medium post, Jen wrote: 'It is now clear that all of my symptoms had a mechanical mechanism: brainstem compression (likely with altered cerebrospinal fluid and cranial blood flow) due to cranial settling and craniocervical instability (CCI), in combination with tethered cord syndrome. Given my remarkable improvements, the centrality of those structural mechanisms is, in my case, undeniable. What remains elusive is the root cause. I know that CCI caused my PEM and other ME symptoms. I can never know why I developed CCI in the first place. (I do have some conjectures!) And I have good reason to think that so long as my fusion holds and my spinal cord does not re-tether, my PEM and other symptoms will never come back.'

I am confused as here she says she can never know why she developed CCI in first place, but in the more recent Medium piece quoted above, she says she knows the postviral damage to ligaments in her neck caused CCI. This is what concerns me, these kind of inconsistencies.

Edit: And just to reiterate, the ME Association in UK has Chiari listed under differential diagnosis of ME. I do know CCI is not same as Chiari, but my very simplistic understanding is they both cause brainstem compression issues.
.

 

Did ur doctor actually say meme haha what a champ

 

Hi, as someone who, having read Jeff and jen’s story, made myself more ill by pursuing this avenue - at great personal distress, which I won’t go into here as I’ve detailed it elsewhere, I wanted to say one other thing.

Dr Bolognese, when you apply to be a patient, asks you to fill in a long questionnaire. This questionnaire has many symptoms and asks you to tick yes or no, presumably to see how many symptoms line up with CCI or chiari symptoms as that’s what he specialises in. As I was filling in it, I noticed I was ticking “yes” to nearly everything, and so my immediate thought was “oh my gosh, I must have CCI and I must have chiari and..” because I was ticking yes to all these symptoms that a neurosurgeon had asked us to fill in. It caused me no end of distress.

But the problem is nearly all the symptoms were non specific. It covered nearly all the M.E/CFS symptoms (except I think the light and noise sensitivity). I think it even mentioned chronic fatigue. It covered depression, anxiety and even autism and aspergers! I even started saying to my mum that she should look into CCI because she’s waiting on an autism diagnosis (I myself have an autism diagnosis).

I think there’s something quite strange about that. What reason would there be for us to fill in this questionnaire in the context of asking to see a neurosurgeon? It reinforces our positive bias that we must have some terrible thing wrong with us, and need surgery, because we have these symptoms?

 

Is the questionnaire similar to this 'Brainstem Disability Index'?

Table 1
Brainstem Disability Index

The following 20 symptoms may be referable to pathology at the level of the brainstem. Please indicate yes or no whether your child has any of the following symptoms on a recurring or chronic basis.

Double vision

Memory loss

Dizziness

Vertigo

Ringing in the ears

Speech difficulties

Difficulty swallowing

Sleep apnea

Snoring or frequent awakening

Choking on food

Hands turn blue in cold weather

Numbness in your arms and shoulders

Numbness in your back and legs

Gets tired very easily

Unsteady walking

More clumsy than you used to be
Urinates more often (every 1-2 hours)

Irritable bowel disease or gastroesophageal reflux disease

Weaker than you would expect in your arms or hand

Weaker in your legs


5% for each positive response, 0%-100%

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/table/T0001/

It's from:
Henderson FC, Wilson WA, Mott S, et al. Deformative stress associated with an abnormal clivo-axial angle: A finite element analysis. Surg Neurol Int. 2010;1:30. Published 2010 Jul 16. doi:10.4103/2152-7806.66461
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2940090/?report=classic

Apparently, the "Brainstem Disability Index" is part of the diagnostic criteria used by Gilete (Barcelona) for CCI. Don't know who invented this index, only found the cited table.

 

No, it’s not like that, it’s on his website.

http://www.chiarinsc.com/word/New patient form2xx.doc

It’s got some of these things but a lot more.
Some of these I can understand being on the questionnaire. Others i really cant.

And in most of the categories, there are usually always one or two M.E related symptoms, which I end up ticking. eg unsteady on feet (dizziness). Or palpitations. Or klutzy (ok well this is due to my dyspraxia). Concentration. Pain, feeling hot, feeling cold, autonomic issues (can inc urinary changes), then there’s all the autoimmune diseases & allergies and asthma which I don’t see why it’s relevant to CCI, then there’s MCAS and EDS...and like I said autism and aspergers. And ocd and depression. And suidocal thoughts.

 

Did you get a reply Trish?

Personally I am happy if someone is studying this. I just want to know how they’re studying it and on what basis. Like a proper study. Because we do need more answers from proper research, and not theories. Until then, theories are taken as fact by some people, and that’s what worries me.

 

That’s surprising. It’s been a month since then.

 

Is there a link where you posted it? I would like to read it if that's possible. Thank you in advance.