Two chronic fatigue syndrome (ME/CFS) experts; Dr. Kaufman – an internist, and Dr. Ruhoy – a neurologist – began a more or less weekly Patreon channel called Unraveled: Understanding Complex Illness in December of last year. The Patreon broadcast provides the rare chance to see two experts who are deeply immersed in these diseases publicly dig into their many different aspects.
In an email, Dr. Kaufman spoke about their goal in doing the Patreon series – which is occurring at least once a week.
“Our goal, our reason for doing this, is to get the word out there and not just to patients but to their families and especially to other physicians. I am quite passionate about the desperate need to recruit more physicians to this work both for ME/CFS and the exploding epidemic of Long Covid. In addition to pulling/luring physicians into this work–which is incredibly challenging and rewarding–I want to help educate primary care physicians since they are–or should be–the backbone and foundation of healthcare in general and particularly for this patient population.”
Health Rising last covered Episode #3 – The “Septad” – a Treatment Roadmap for ME/CFS, POTS, Long COVID.
Please note that the following is attached to the beginning and end of every podcast.
Now we’re onto Episode #4.
Episode #4: Craniocervical Instability
What an episode this is! It’s quite possible there would be no discussion of craniocervical instability (CCI) in ME/CFS and related diseases without Dr. Kaufman. Jeff Wood – in the most remarkable display of self-diagnosis I’ve ever seen – started the CCI ME/CFS conversation off about five years ago.
Somehow Jeff – who was very severely ill at the time – came up with the idea that he had a condition that no one had connected with ME/CFS before. Not only that, but it was a rare condition afflicting the upper spine which (in his case) would require major spinal surgery to fix. Jeff, not surprisingly, struck out in his attempts to have doctors give his hypothesis a shot – until he met up with Dr. Kaufman.
Dr. Kaufman gave him the referral and the rest is history. Jeff had the surgery, fully recovered, and spread the news and now CCI, tethered cord syndrome, and other spinal issues (cerebral spinal fluid leaks, idiopathic intracranial hypertension, cerebellar tonsils) are part of the diagnostic algorithm for many ME/CFS experts.
Doctors have come a long way since Jeff and Jen Brea startled the ME/CFS communities with their remarkable recoveries from their severe ME/CFS. Please note, though, that while there have been some remarkable recoveries, some people have not done well at all with surgery, and some people have had much more moderate results. This is a work in progress.
Given that Dr. Kaufman and Dr. Ruhoy, a neurologist, have probably seen more CCI ME/CFS patients than any other doctor, this episode was a great opportunity to get it straight from the horse’s mouth. What more, really, could you want?
“Super Relevant” Problem
The episode started off with Dr. Ruhoy stating that they had both seen “many” of these patients, with Dr. Kaufman stating the condition was “super-relevant” and Dr. Ruhoy, a neurologist, noted that it was probably particularly relevant to patients who were house or bedbound and/or had problems with dysautonomia, and/or hypermobility.
CCI all revolves around the CO-CI or atlantooccipital joint. It’s a joint at the top of the spine which separates the skull from the vertebrae. It’s not just a separator, though, it’s actually part of the brainstem, the blood vessels that feed the brain, compartments of cerebral spinal fluid, and can be associated with low-lying cerebellar tonsils or Chiari malformation.
It’s clearly a densely packed piece of real estate in the body.
The Atlantooccipital Joint
Dr. Kaufman offered up a picturesque view of the atlantooccipital joint. Humans, he noted have a very heavy brain sitting on a little neck. The brain is held in place by the most mobile joint in the body which allows us to turn our heads in all sorts of directions. (Jen Brea – another CCI patient – later recognized that her inability to turn her head to one side without sometimes severe symptoms was an indication that she had CCI). That great mobility arises out of 20 different ligaments twisting in and out of the CCI.
Information from the brain flows into the brainstem and then makes it into the spinal cord, and passes through an opening called the foramen magnum in the skull. Heavy head, easily damaged ligaments, tender spinal cord, intermixed with lots of bones – it is amazing things don’t go wrong more often.
The problem involves ligament laxity; i.e. the 20 ligaments in the area can no longer hold the skull in its proper place. Connective tissue problems, trauma (such as whiplash, from concussions), and infections can all degrade the ligaments and cause instability.
It was good to hear Dr. Ruhoy state that having CCI was “not necessarily a problem” at least so far as needing to have surgery, but then stated that since it’s an anatomical problem, the surgery is really the only way to correct the anatomy.
That’s not the total picture though. Reducing the inflammation that is attacking the ligaments to tighten them up can help. Still, CCI tends to be a progressive condition. Dr. Kaufman agreed that the challenge for them as physicians is “finding and diagnosing those patients before they have instability or CCI”, because although there are things they can “hopefully do to tighten the ligaments”, once anatomical instability is present, he “wasn’t sure that anything else will fix that”.
The whole subject of spinal surgery – which Dr. Ruhoy noted that not too many people were crazy about – was part of a “huge conversation” that Dr. Kaufman said he had with his patients. You can’t “lift the head (back) up” to where it should be simply with exercise or therapy”.
Symptoms include things like “my head feels too heavy”; and symptoms that flare when the head is in a certain position, some people may hold their head differently on their neck. Dr. Ruhoy said she’d looked for a straight line across their ears. An off-kilter line doesn’t mean they have CCI, but in the presence of symptoms, it is suggestive. (More on how to diagnose CCI in the next episode.)
One thing that happens in CCI is that the low end of the brainstem – which contains neural pathways that can affect just about any function in the body – gets impacted by the skull.
The Knotty Question – to Have Surgery or Not – and If So, When?
The surgery is a fusion between C0 and C2 – not something that Dr. Ruhoy would, as a neurologist, have as her endgame, but it’s the most effective means of correcting CCI right now.
Dr. Kaufman noted that there is a spectrum. Some patients have some “unstableness”. They may have postural orthostatic tachycardia syndrome (POTS), dysautonomia, and hypermobility but they are still functioning and can hold down a job. His job is to keep them functioning, reduce inflammation, and tighten up their ligaments.
Others are not so functional but not bedbound, often not able to work, have more severe POTS, have more severe mast cell activation, they are doing sort of OK – and may be heading towards surgery, but they try to avoid it.
Then there’s the profoundly disabled, mostly bedbound, group. Dr. Kaufman noted all the drugs in the world, all the titrations, all the treatments for mast cell, bacterial overgrowth, their autoimmune problems – which can otherwise work in their case – do not get them better – because he cannot fix a damaged brainstem.
Given that it’s much harder to get these very ill patients to have this surgery done, the question is: do they have patients who are not bedbound do the surgery before they get so ill that they have more trouble getting the surgery done, recovering from it, etc.?
Dr. Ruhoy said that she was seeing a good response from injection therapies, but since they don’t correct the underlying connective tissue situation, she didn’t know that it would obviate the need for surgery at some point.
Stating “It’s a huge challenge”, Dr. Kaufman stated for the past 40 years he’s done anything possible to have his patients avoid surgery, but in this instance, he’s done a complete 180-degree turn on this. “Professionally and personally, it’s very challenging” for him since he knows how major this surgery is. But the fewer the comorbidities, the less bedbound, the better their muscles work, and the better their prognosis post-surgery.
With regard to his bedbound patients – Dr. Kaufman said he has changed his tune. He is now “convinced” that if a patient is bedbound, they have CCI – until is ruled out – it is that common in bedbound patients. In these patients, instead of going for the low-hanging fruit (mast cell activation, infection, POTS) first, and then looking at CCI, he’s immediately investigating whether they have CCI, checking out traction, looking for physical therapy – because he’s pretty sure that’s where they’re headed.
Dr. Ruhoy agreed. At Mt. Sinai, as with Dr. Kaufman, they go after the mast cell, POTS, etc. because that helps prepare for the surgery and recovery.
First Step – Manual Traction
If the imaging indicates it’s safe, Dr. Ruhoy and Dr. Kaufman try to do manual traction at home. Not only is getting a cervical MRI for his bedbound patients difficult, but is almost always normal in the typical radiology reports. (Special kinds of imaging are needed to distinguish CCI and allied issues).
Plus, the imaging results at these levels are not always in sync with the symptoms. (This is actually a well-known issue with imaging of all kinds.) Ruhoy has seen a “remarkable” clinical response to traction at home in people with either normal or borderline cervical issues.
(As an aside, Ruhoy noted that in almost all the MRIs, she sees a loss of lordosis (curvature of the spine) and degenerative issues (cervical disc disease) – even in relatively young people).
Dr. Kaufman agreed that regardless of the imaging results, the “money” for him regarding diagnosis is in the “powerfully revealing” response to traction. He prefers hands-on traction but noted it can be difficult to do. Dr. Ruhoy actually prefers home traction as you can control the weight.
Second Step – Invasive Cervical Traction
If the manual traction proves helpful, the next step is to do invasive cervical traction (ICT) – something that only Dr. Paolo Bolognese – clearly their go-to surgeon – does in the U.S. It’s a vital step because it also helps with prognosis; a good result from the invasive cervical traction means patient is a good candidate for the surgery – and is an important part of pre-surgery planning.
Since Dr. Bolognese is doing imaging at the same time the traction is underway, it also gives him guidance regarding the surgery. At every increment of change in the traction, Bolognese takes pictures and asks about symptoms.
The ICT simulates the effects of the fusion and Dr. Bolognese uses the results of the fusion to find the sweet spot for the surgery. Dr. Ruhoy said she wouldn’t do a CCI surgery without it.
- Dr. Ruhoy and Dr. Kaufman’s Patreon series continues with Episode 4: Craniocervical Instability (CCI) – the inability of the joint at the top of the spine – the most mobile and complicated joint in the body – to keep the skull in place – allowing it to settle on the brainstem and potentially causing virtually every symptom in the body.
- It’s no surprise that they covered this topic so early. Both consider it “super-relevant” to ME/CFS and related diseases, and Dr. Kaufman was the first ME/CFS doctor to consider it and ultimately uncover it.
- After Jeff Woods and Jen Brea’s successful surgeries for CCI, tethered cord syndrome, and other spinal issues (cerebral spinal fluid leaks, idiopathic intracranial hypertension, cerebellar tonsils), it became part of the diagnostic algorithm for many ME/CFS experts.
- The problem involves ligament laxity; i.e. the ligaments are no longer able to hold the skull in its proper place. Connective tissue problems, trauma (such as whiplash, from concussions), and infections can all degrade the ligaments and cause instability.
- Since CCI tends to be a progressive condition, the challenge for doctors is finding and diagnosing those patients before they have instability or CCI because although there are things they can “hopefully do to tighten the ligaments”, once anatomical instability is present, nothing but surgery will work.
- The subject of spinal surgery is part of a “huge conversation” that Dr. Kaufman said he had with his patients. Unfortunately, you can’t “lift the head (back) up” to where it should be simply with exercise or therapy.
- There is a spectrum. His job with patients with some “unstableness” who may have postural orthostatic instability syndrome (POTS), dysautonomia, and hypermobility, but are still functioning and can hold down a job, is to keep them functioning, reduce inflammation, and try and tighten up their ligaments.
- Others who are not so functional but not bedbound, often not able to work, have more severe POTS, have more severe mast cell activation, are doing sort of OK – may be heading towards surgery.
- Regarding the profoundly disabled, mostly bedbound, group, Dr. Kaufman noted all the drugs in the world, all the titrations, all the treatments for mast cell, bacterial overgrowth, their autoimmune problems – which can work in their case – do not get them better – because he cannot fix a damaged brainstem. Unless he is shown evidence to the contrary, he assumes that all bedbound patients have CCI, tethered cord syndrome, or both. With these patients, he immediately investigates whether they have CCI.
- Given that it’s much harder to get these very ill patients to have this surgery done, the question is: do they have patients who are not bedbound do the surgery before they get so ill that they have more trouble getting the surgery done, recovering from it, etc.?
- Using at-home traction or manual traction to begin to assess whether CCI is present is much more revealing than doing standard imaging (which is mostly useless). Reducing symptoms via these methods is a powerful indicator that CCI may be present.
- The next step is invasive cervical traction – done in the U.S. only by Dr. Bolognese. Not only can it be extraordinarily revealing but it also helps Dr. Bolognese plan the surgery, if necessary.
- Calling it a “miracle”, Dr. Ruhoy said that when she’s been in the room during an ICT, the response almost brings tears to her eyes.
- Dr. Kaufman noted that if you have more than CCI – as many patients do – you need to take a global approach and fix the mast cell problems, the POTS, the autoimmune issues, etc. to get well. Failed CCI stories may have occurred because these other things were not taken into account.
- Both Kaufman and Ruhoy agreed that alternative methods such as injections can only temporarily help and are extraordinarily expensive.
- Kaufman said he’s “almost at the point” where if he has a patient with POTS, MCAS, and the other parts of the septad, he’s convinced that if he doesn’t aggressively do things to strengthen their connective tissue – that patient is going to need CCI surgery at some point.
- Some treatments may be coming. Dr. Ruhoy said she has seen patients respond to peptide and stem cell therapies (Kaufman agreed).
- Next up – diagnosing CCI.
Dr. Bolognese is looking for resounding symptomatic changes as the traction – which lifts the head off the brainstem – occurs – as well as structural changes. Ruhoy and Kaufman have seen significant structural changes as a result of the ICT – but not significant clinical changes – and vice versa. In those cases, Bolognese usually repeats the test in 3-6 months.
Dr. Kaufman said to physicians who are skeptical of invasive cervical traction (ICT) – just watch a few videos – it is a “miracle”. Dr. Ruhoy said that when she’s been in the room during an ICT, the response almost brings tears to her eyes. People’s faces change, she said.
Kaufman said that during ICT, he’s seen patients state that they didn’t know they had trouble with their vision but all of a sudden they could see clearly, or their hearing clears up – they can hear clearly now, or think clearly.
In the Septad (the seven main interacting features of these diseases), everything interacts with everything. Kaufman, noting the negative reports regarding CCI that sometimes circulate on social media, asserted that you can’t expect CCI to fix everything. If you have more than CCI – as many patients do – you need to take a global approach and fix the mast cell problems, the POTS, the autoimmune issues, etc. as well.
Almost everyone he’s sent for a fusion has also ended up having a “tethered cord”. He’s also seen things like “venous compression syndrome” and Dr. Ruhoy noted that “eagle syndrome” can also be found.
Since these are different surgeries and both sometimes need to be done, the question arises what to fix first? Dr. Ruhoy said that doing the CCI first can make the tethered cord worse and vice versa. (Releasing the tethered cord can create a pull on the CCI and fixing the CCI can impact the tethered cord). The treatment approach is clearly evolving. Dr. Kaufman said that at first, Dr. Bolognese always did the more difficult CCI surgery first. Now he’s doing the tethered cord first.
Dr. Kaufman asked Dr. Ruhoy an important question: have you seen any patients that you were sure needed CCI receive clear benefits from alternative alternatives such as injections? Dr. Ruhoy said yes, but the benefits were short-lived. This is because the injections can provoke a mast cell reaction that needs to be managed.
In the end, Dr. Kaufman and Dr. Ruhoy came down rather hard on injections – stating that they can cause tens of thousands of out-of-pocket dollars – and at the end of the day, while they can temporarily help, they aren’t fixing the problem. Dr. Kaufman compared the situation to having a flat tire. If you’re driving your car and you have a flat tire, you cannot fix that tire unless you jack up the tire. Injections, in other words, don’t jack up the tire; i.e. tighten up the ligaments.
Again it was back to: when is the optimal time for surgery? Ruhoy said that for young patients who are still functional, she tells them they’re a good candidate. She strongly recommends it. Dr. Kaufman asked: how do we identify patients who are at risk for surgery at some point. He outlined three groups of patients:
- Patients with connective tissue disorders such as hypermobility.
- Patients who have chronic inflammation (mast cell activation syndrome, POTS, leaky guts, infection) who do not have connective tissue disorder.
- Patients with both – Kaufman said he’s “almost at the point” where if he has a patient with POTS, MCAS, and the other parts of the septad, if he doesn’t aggressively do things to strengthen their connective tissue – that patient is going to need CCI surgery at some point.
Some treatments may be coming. Dr. Ruhoy said she has seen patients respond to peptide and stem cell therapies (Kaufman agreed), and she thinks the answer may ultimately be there.
Dr. Kaufman reiterated that his goal is to find ways to avoid the surgery. The problem is that most alternatives are often experimental, not FDA-approved, never covered by insurance, etc.
More on Craniocervical Instability
- Craniocervical Instability, Neurosurgery and M.E.: Just the Facts, Please – An Editorial by Jeff Wood
- Jeff Woods Mechanical Basis website
- Jen Brea’s Amazing ME/CFS Recovery
- Health Rising’s Craniocervical Instability and Spinal Issues Poll
Next Up – Episode V of the Patreon – Diagnosing CCI and other spinal issues.
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