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- The GIST can be found near the bottom of the blog
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One sign that things are improving is simply an increase in activity, and that’s what we are seeing on all fronts. The battle for the $10 billion long COVID (and post-infectious diseases) Moonshot is underway, and one to create a Center for post-infectious diseases at the NIH is about to begin.
We recently had the 3-day RECOVER Initiative Clinical Trials webinar, a 3-day conference on mast cells just finished up (blog coming up), Solve M.E. talked with Rob Wust about his exciting muscle work, and Peter Rowe about his new book, and now the Renegades – yes, the Renegade Research team from Remission Biome – has been putting on its own webinars. It’s latest is “The Mechanical Basis Model/Brainstem – Keys to Understanding ME/CFS”
The brainstem: a complicated piece of architecture…(Image – Nadezdha from Wikimedia_Commons)
The Brainstem
For years we heard little about the brainstem. Because it lies at the base of the brain and most MRI focus on the upper, more recently developed parts of the brain, most scans don’t do a good job of examining it. The brainstem, though, is definitely in the ME/CFS, FM and long-COVID researchers’ sights now.
A Walk Around the Brainstem
Because his talk describes the brainstem, let’s start off with the second talk – “A Walk Around the Brainstem” by Michael VanElzakker of PolyBio.
The brainstem is old! (Formed hundreds of millions of years ago it predates humans. Mammals, birds, reptiles, and amphibians all have brainstems that regulate core functions.)
It regulates essential processes like wakefulness, sensory processing, pain, sleep, and the autonomic nervous system, and brainstem problems have been found in ME/CFS, fibromyalgia, and long COVID.
It’s a piece of complicated and delicate architecture that’s surprisingly fragile for such an important organ. In an Unraveled podcast, Dr. Kaufman evocatively described it. Humans, he noted have a very heavy brain sitting on a little neck, which just happens to be the most mobile joint in the body. That mobility is enabled by no less than 20 ligaments twisting in and out of the CCI. Heavy head, easily damaged ligaments, tender spinal cord, intermixed with lots of bones – it is amazing things don’t go wrong more often.
The structures through which the CSF flows are thin enough that even small kinks in them could impair flow. VanElzakker highlighted the fact that the vagus nerve – a kind of inflammation monitor of the body – empties into the brainstem. It’s through the vagus nerve that the brain learns when to initiate “sickness behavior”; i.e. produce the flu-like symptoms that we associate with colds – and which are very similar to those found in ME/CFS and long COVID.
Indeed, a potentially pivotal Nature paper demonstrated how the specific vagal neurons in the brainstem send inflammatory or anti-inflammatory messages to the brain. The authors were clearly jazzed by their findings:
“We suggest that pharmacologically targeting this circuit may provide exciting new strategies to modulate and manage immune disorders, including autoimmune diseases (for example, rheumatoid arthritis), cytokine storm, toxic shock and other hyperactive immune states, such as those promoted by new immunotherapies.”
Note that the vagus nerve can be very sensitive to small amounts of inflammation – small enough that they wouldn’t be detected in the blood. Indeed, VanElzzaker has proposed that small infections near the vagus nerve may be triggering ME/CFS.
Before Jeff Wood, nobody was seriously looking at the craniocervical junction in these diseases.
Jeff Woods and the Mechanical Basis for ME/CFS
Few people can be said to have started a movement, but Jeff Woods certainly did. Woods self-diagnosis of craniocervical instability (CCI), and his resulting recovery after spinal surgery, opened up an entirely new field of inquiry in chronic fatigue syndrome (ME/CFS).
Woods, who now works on a neurosurgery team with Dr. Bolognese, developed his on hypothesis called, “The Mechanical basis for ME/CFS”. Woods’s hypothesis is more about physics or engineering than anything else. He believes that damaged connective tissues in the brainstem and/or spine may be causing many of the problems in at least some ME/CFS patients.
Woods aptly noted that the spine, and delicate spinal surgery, is not exactly where anyone – patients or clinicians – wanted to end up with this disease – and he hastened to note that spinal surgery is not at all necessarily the endpoint for people with these issues.
Besides craniocervical instability, Woods noted that other spinal issues may be happening:
- In Chiari malformation, a part of the brain called the cerebellum extends into the spinal cord.
- High cerebral spinal fluid pressure (intracranial hypertension) – which may be common in ME/CFS – can put pressure in the brainstem.
- In cervical spinal stenosis, the spinal canal in the neck narrows, putting pressure on the spinal cord.
- In internal jugular vein stenosis, the vein that drains blood from the brain becomes narrowed, thus producing intracranial hypertension.
A large 2020 Swedish study confirmed that connective tissue issues, or problems related to them, were common when it frequently found hypermobility, signs of idiopathic intracranial hypertension, and craniocervical obstructions in ME/CFS patients.
The common element in all of these are connective tissue issues, and here’s where Jeff’s talk added something new, at least to me. It turns out that infections spark an innate immune response called collagenolysis that is designed to attack the connective tissues.
Some pathogens break down connective tissue in order to burrow into our tissues. That triggers an innate immune response which can disrupt the collagen and damage the connective tissues in the brainstem area. (Another possibility – well-known to people with rheumatoid arthritis – is an autoimmune attack on these tissues.)
Woods proposed that people with weakened connective tissues – such as those who exhibit hypermobility – are more at risk of damage during an infection. Indeed, Peter Rowe, in “Living Well with Orthostatic Intolerance” describes several case reports where an infection, in combination with hypermobility, kicked off spinal (and other) issues.
That said, hypermobility is not the culprit, per se. Many people who are hypermobile are perfectly healthy. Something else is turning hypermobility into a pathogenic condition.
Dr. Yellman – Neuroanatomical Considerations in ME/CFS – A Clinician’s Perspective…
Dr. Yellman joined the Bateman Horne Center in 2019 and has been focusing on these diseases since then.
Many people are acquainted with the brainstem and the basics of spinal issues, but it was with Yellman’s presentation that things turned really juicy. We rarely get news from the clinic side on spinal issues and that’s what we got with Yellman who was, as he typically is, superbly organized and clear.
Another report straight from the “bench”, or clinic, in Dr. Ruhoy and Dr. Kaufman’s Unraveled Craniocervical podcast (on Patreon) gives us a chance to compare two approaches from ME/CFS experts.
One thing that is clear – both clinics have seen quite a few ME/CFS, long-COVID, etc., patients with spinal issues, including people with craniocervical / atlantoaxial instability (CCI/AAI). Kaufman called the problem “super-relevant” while Ruhoy noted it’s probably particularly relevant to patients who were house or bedbound and/or had problems with dysautonomia, and/or hypermobility.
Yellman started off by saying that there are more questions than answers but that “incompetent connective tissues” that produce weak or lax ligaments appear to be the culprit. These weak ligaments can result in trapped or even deformed nerves in the neck, spine and even the peripheral nerves.
Yellman noted that hypermobility increases the risk of all the spinal conditions sometimes found in these diseases. The biggies are idiopathic intracranial hypertension (IIH), Chiari 1 malformation, craniocervical instability (CCI), atlantoaxial instability (AAI), and tethered cord syndrome.
Mast Cells, Mast Cells, Mast Cells!
Mast cells seem to be everywhere these days. Tying mast cell activation syndrome (MCAS) and hypermobility closely together, Yellman said that nearly 1/3 of patients with an MCAS diagnosis also meet the criteria for hypermobile syndrome and that one study found that 2/3rds of patients with POTS and/or EDS met the criteria for MCAS.
Activated mast cells can tweak the peripheral nerves – sending pain signals to the brain – and induce an IgE response that attacks connective tissues in the brainstem, which produces lax blood vessels causing orthostatic intolerance, as well as asthma, swallowing problems, etc. It’s no wonder there’s so much interest in mast cells now.
(They do not appear, as yet, to provide the answer to ME/CFS or the connective tissue issues found in CCI/AAI and other spinal issues. While mast cell inhibitors/stabilizers have become an important tool in many ME/CFS experts’ toolboxes, they’re not curative – yet. Either mast cells are not the only game in town (protein proteases may be more important in IBS) or we don’t have good enough treatments for them (or more likely both). One thing is clear – we need more research on them. An overview of the 2024 Mast Cell Masterminds Conference is coming up.
Craniocervical Instability (CCI) (CO-C1) and Atlantoaxial Instability (AAI) (C1-C2)
We heard that CCI/AAI can impede upon the brainstem/upper spinal cord, but Yellman also pointed that the increased instability/mobility of these crucial joints can also impact blood or cerebral spinal fluid flows from the brain.
Headaches
CCI/AAI can produce a wide range of symptoms, but Yellman looks first for headaches – a common symptom in CCI. As Yellman went through the different causes of headaches, I began to feel for doctors! Here are some of the questions Yellman asks himself to determine what may be causing a patient’s headache:
- Does it get worse after getting up or lying down?
- Does it develop as the day goes on?
- Is it relieved by increasing blood volume or using drugs for orthostatic intolerance?
- Is the headache get worse after lying down at night?
- Could the patient be experiencing hypertension while lying down?
- Does the headache get worse with blood volume enhancement?
- What about a cerebral spinal fluid leak? (drippy nose, Valsalva manuever and putting head down makes worse)
- Is it a migraine type of headache? (aura, photophobia, MCAS caused?)
- Is it a tension headache – associated increased neck tension, connective tissue disorder, increased fight/flight; radiating along neck, shoulder; do headaches improve with massage, TENS, muscle relaxants, Botox?
- Could sinus congestion be causing it?
- Sleep disruption – is sleep apnea present?
- Could MCAS response to food/medication/chemical sensitivity be causing it?
- How about hypoxia (low oxygen levels) or hypertensive urgency? Or excessive sensory processing?
- What about medication overuse – could the patient be having rebound headaches?
- Are there are structural TMJ abnormalities?
- Is it a product of PEM?
CCI/AAI Diagnosis
Yellman on how he functionally assesses CC1/AAI instability
Noting that diagnosis is always evolving, Yellman stated that he begins with a functional assessment. Does the use of an over-the-counter inflatable collar or a soft cervical stabilization collar (which raises the head up) reduce symptoms? While this is not a failsafe test and will miss some things – it’s an easy first assessment.
Yellman noted that doing traction in an upright position is a more helpful test and, indeed, Kaufman called traction – whether done at home or in the office – the money test for him. Even people with borderline or normal imaging results can respond quite well to traction at times.
An upright image is superior if you can get a good image; dynamic flexion protocols can induce symptoms but is very helpful. Yellman described a variety of MRI and CT scans that are helpful.
Even most neuroradiologists and neurologists are not familiar with these conditions and don’t know how to correctly position patients for these tests. He said he can’t tell how many times – almost every time – that a neuroimaging test is positive for CCI and neuroradiologists say there’s nothing there. He commonly sees evaluations that miss it.
Yellman didn’t go into the next step – invasive traction accompanied by extensive imaging – but Ruhoy and Kaufman called it a critical test to determine if CCI surgery is suitable, and to help guide the surgery. With invasive traction, they’re looking for really profound changes in one’s symptoms.
The Dilemma
Yellman faces the same dilemma that Ruhoy and Kaufman talked about in their Unraveled episode: when to move forward with surgery. CCI surgery, after all, is a major undertaking for a healthy person, let alone a person with ME/CFS. The surgeons who do this procedure are rare.
The surgeries can result in complications (CSF leak/fistula, artery damage, hardware failure, vertebral instability in the spine below). Recovery times appear to be pretty long and the range of motion can be impaired.
On the other hand, some people’s symptoms can improve dramatically, and waiting too long can be devastating if the CCI or tethered cord progresses – as it probably will over time. Severely ill patients may become too weak to be able to handle the surgery or derive as many positive benefits as they would have otherwise. Nerve damage may occur that can’t be repaired by surgery.
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”.
After doing everything he could to avoid surgery, Dr. Kaufman is now moving more quickly to surgery for certain groups of patients. He is now “convinced” that if a patient is bedbound, that until it’s ruled out, the patient probably has CCI. In these patients, he immediately begins investigating whether they have CCI, checking out traction, looking for physical therapy – because he’s pretty sure that’s where they’re headed.
- Patients who have some “unstableness” and may have postural orthostatic tachycardia syndrome (POTS), dysautonomia, and hypermobility, but are still functioning and can hold down a job, his job is to keep them functioning, reduce inflammation, and tighten up their ligaments.
- People who are not bedbound, often not able to work, have more severe POTS, have more severe mast cell activation, are doing sort of OK – may be headed towards surgery, but he tries to avoid it.
- The profoundly disabled, mostly bedbound, group, for which nothing has worked, are probably headed for surgery.
Before CCI/AAI is considered, Yellman has his patients pace effectively, attacks MCAS, works on dysautonomia and orthostatic intolerance, tries to calm the sympathetic nervous system, reduces sensory stimulation, and improves metabolic deficiencies.
He only goes after CCI/AAI when his more severe patients hit a wall and just can’t improve. Then he tries traction and physical therapy and tries to manage intracranial hypertension using drugs like acetazolamide, topiramate, Factor Xa inhibitors, and pentoxyphylline.
He openly wondered, though, if by trying to attack the problem in other ways first, some of his patients who needed the surgery were getting worse. It’s a tough call.
More Conservative Approaches
Yellman, Kaufman, Ruhoy, and Woods all agree that other options than surgery exist for the right patient. The great goal, of course, is to find a way to strengthen and repair the connective tissues (ligaments). Yellman said that, many times, conservative treatment is all that is necessary for some patients.
Deepwater traction using a flotation device in the deep end of the pool can help some people who don’t benefit from the collar outside of the pool. Yellman noted that these benefits by stretching the head upward that home traction devices provide can produce negative results as well, particularly in those with tethered cord syndrome.
Yellman often has the patient wear the collar for 3-4 hours/day while upright, while doing physical therapy at the same time, to strengthen the muscles.
Yellman reported on a patient who was able to spend only an hour upright a day but after doing manual cervical traction for an hour a day, plus regular physical therapy, has been able to work 8-hour days for over 2 years.
Sometimes treating a tethered cord can improve stability in the craniocervical joint (Yellman)
By removing the tension present in the spinal cord, tethered cord release surgery is a more conservative approach than CCI/AAI surgery, which is sometimes able to reduce the CCI/AAI instability enough so that the physical therapy may be enough. Yellman said he’s had many patients with CCI who’ve experienced huge improvements in their functional capacity by having tethered cord surgery.
Yellman, Ruhoy, and Kaufman also use mast cell and immune therapies in an attempt to turn off the immune response that may be damaging the connective tissues. It turns out that the connective tissues are loaded with innate (inflammatory) immune cells, including mast cells. Simply too much stretching from abnormally loosey goosey connective tissues can result in inflammation.
Better mast cell/immune treatments would surely help. With regard to more exotic ways to strengthen the connective tissues, Yellman has not yet seen success from ME/CFS patients who have tried prolotherapy, platelet-rich plasma, and stem cell therapy but needed more data. Ruhoy and Kaufman agreed about injections (which can cost tens of thousands of dollars), but Ruhoy has seen patients benefit from peptides and stem cell therapies, and she and Kaufman think they may be the future.
Surgery Likely Not a Complete Cure
Yellman, Ruhoy, and Kaufman also appeared to agree that CCI/AAI surgery is not a complete cure; this is not generally a get-the-surgery-done-and-walk-away-healthy-for-the -rest-of-your life treatment.
If the patient has more than CCI – as many patients do – the mast cell problems, the POTS, the autoimmune issues, etc., will still need to be addressed.
If the patient gets hit by another infection, or they have problems below the surgery, or get another hit – the PEM, the dysautonomia, the mast cell problems – can come back.
CCI, then, can fix the structural problem, but whatever caused it may still be lurking in the background. (Note that ME/CFS CCI patients are different from other CCI patients who don’t display all the ME/CFS symptoms.)
A Lyme/Mold doctor noted that after surgery for tethered cord, some patients need more surgery, and she’s looking into IVIG to produce better outcomes. A patient who’d had CCI surgery found that his PEM went away and then came back but knows of another person whose PEM went away entirely.
Other Spinal Issues
Acquired/Occult Tethered Cord Syndrome
Sometimes tethered cord surgery can improve the stability in the craniocervical joint.
Dr. Yellman touched on another major spinal issue that can show up with CCI/AAI or by itself – tethered cord syndrome (TCS). TCS is a broad term that refers to a malformation of an elastic band called the filum connecting the bottom of the spinal cord to the tailbone. The spinal cord should be able to move up and down easily, but in TCS it is held taut.
TCS can result in neurogenic bladder, lower back pain, migratory pain in legs, urinary urgency, frequent UTIs, lower extremity weakness, pulling sensation on the upper spine, sensory loss, inability to walk on the heels, etc. One early test for TCS has people walk on their heels to see if that provokes symptoms.
Surgery to release the “tethered cord” relieves tension in the spinal cord – and can help with CCI/AAI. Yellman said he’d had many patients with tethered cord release and CCI/AAI for whom tethered cord release had produced huge improvements in functionality.
On the other hand, Kaufman pointed out that tethered cord surgery can make some patients’ CCI/AAI worse – and be diagnostic in that way. He noted, though, that if both tethered cord and CCI/AAI are present, that Dr. Bolognesse – the go-to guy for CCI/AAI surgery in the U.S. – now does the tethered cord surgery first.
Yellman uses urodynamic testing to see if neurogenic bladder is present and 3T Prone/supine L-MRIs can tell if the cord is moving properly.
Venous Congestion / Outflow Obstruction Syndromes
Then it was onto a fascinating topic – venous congestion or outflow syndromes; syndromes that impair the blood flows in the veins. Arteries provide blood flows to the brain and the veins carry up the used-up blood, which is now full of waste products, out of the brain. Cerebral spinal fluid flows out of the brain do the same thing.
THE GIST
- Renegade Research’s recent webinar “The Mechanical Basis Model/Brainstem – Keys to Understanding ME/CFS” asked if spinal issues in the brainstem held the key to understanding ME/CFS.
- The brainstem is a very old piece of nervous system real estate that regulates essential processes like wakefulness, sensory processing, pain, sleep, and the autonomic nervous system.
- It’s a piece of complicated architecture that’s surprisingly fragile. Dr. Kaufman evocatively described it. Humans, he noted have a very heavy brain sitting on a little neck, which just happens to be the most mobile joint in the body. The structures through which the CSF flows are thin enough that even small kinks in them could impair flow.
- VanElzakker highlighted the fact that the vagus nerve – a kind of inflammation monitor of the body – empties into the brainstem. A recent Nature study that found a master immune switch there proposed that the finding opened the door to drugs that could treat a variety of immune ailments.
- Jeff Woods’s ME/CFS recovery story following craniocervical instability (CCI) surgery essentially jumpstarted interest in the craniocervical/atlantoxial joint supporting the head. He described other spinal issues (Chiari malformation, high cerebral spinal fluid pressure (intracranial hypertension), cervical spinal stenosis, jugular vein stenosis)
- ‘The common theme in all of these is damage to the connective tissues that results in trapped or even deformed nerves in the neck and spine, damage to the brainstem, and impaired blood and cerebral spinal fluid flows.
- People who are hypermobile are more at risk from these problems. Mast cells that break down the connective tissues appear to play a major role. Jeff described a process called collagenolysis which occurs when the body breaks down the connective tissues in an effort to get at pathogens.
- Dr. Yellman from the Bateman Horne Center in 2019 brought a much-needed clinical approach to these complex issues. Insights from an Unraveled podcast by Dr. Ruhoy and Kaufman on craniocervical instability (CCI) were included in the blog as well. Both clinics have seen many of these patients with CCI.
- Yellman begins by determining whether an over-the-counter inflatable collar or a soft cervical stabilization collar (which raises the head up) reduce symptoms? While this is not a failsafe test and will miss some things – it’s an easy first assessment.
- Doing traction in an upright position is a more helpful test and, indeed, Dr. Kaufman called traction – whether done at home or in the office – the money test for him.
- Imaging is next. An upright image is superior if you can get a good image; dynamic flexion protocols can induce symptoms but is very helpful. Most neuroradiologists, though, don’t know how to properly assess these scans.
- After that comes invasive traction accompanied by extensive imaging at a specialty clinic to determine if CCI/AAI surgery is warranted. The surgeon will be looking for profoundly positive symptom improvement.
- All three doctors face a dilemma when to move forward with a major surgery that can produce serious complications, has a long recovery time, and has produced differing degrees of success – including some remarkable increases in functionality.
- Kaufman described three groups of patients: patients with some unstableness who he tries to keep functioning and tighten up their ligaments using mast cell, immune therapies, and physical therapy; more severe patients who are not able to work who may be heading for surgery, and the profoundly disabled, mostly bedbound, group, for which nothing has worked, who he believes are probably headed for surgery.
- Yellman has his patients pace effectively, attacks MCAS, works on dysautonomia and orthostatic intolerance, tries to calm the sympathetic nervous system, reduces sensory stimulation, and improves metabolic deficiencies.
- He only goes after CCI/AAI when his more severe patients hit a wall and just can’t improve. At that point, he tries things like physical therapy, at-home traction, as well as drugs to treat things like intracranial hypertension. He said in many cases conservative treatments are enough and cited the case of a formerly bedbound patient who after regularly doing traction and physical therapy was able to return to a full-time job.
- Sometimes tethered cord surgery which releases the cord at the base of the spine – thus relieving tension at the top of the spine – can help significantly with CCI/AAI issues.
- Yellman, Ruhoy, and Kaufman appeared to agree that CCI/AAI surgery is not a complete cure; this is not generally a get-the-surgery-done-and-walk-away-healthy-for-the -rest-of-your life find of treatment.
- While some remarkable improvements have been made, if the patient has more than CCI – as many patients do – the mast cell problems, the POTS, the autoimmune issues, etc., will still need to be addressed. If the patient gets hit by another infection, or they have problems below the surgery, or get another hit – the PEM, the dysautonomia, the mast cell problems – can come back.
- Venous congestion or outflow syndromes that impair the blood flows in the veins and cerebral spinal fluid flows may also be contributing to these problems. With venous blood flow problems showing up in the abdomen, pelvis, legs and cranial area one wonders if they play a major role in these diseases.
- Caroline Christian in her Frozen in Amber blog told her story of tethered cord syndrome surgery. After she was diagnosed with a neurogenic bladder she had two options: surgery or a progressive decline in functioning.
- The invasive traction test which lifted her skull off her brainstem produced some remarkable results. She wrote “I felt incredible joy having a clear brain and boundless energy! Even my vision improved, and I felt better than I had in 15 yrs – a reminder that I am still inside this broken body. I was euphoric, and I felt a deep sense of equanimity, trusting that everything would be OK.”
- Her tethered cord syndrome surgery improved her physical functioning dramatically (from 20-50%) and she went from being bedbound to being able to walk 20-60 minutes 3-4 times weekly. On the downside, her bladder dysfunction continues to decline, her cognition is still low, and her stress intolerance is still very high.
- Julie Rehmeyer, who has had CCI surgery which appeared at first to go really well but who is not doing well at present, spoke to the complexities that can be present. In some cases, the problems go way beyond the anatomical issues found with CCI/AAI and tethered cord. For complex patients like her, there is no clear path.
- Check out the Spinal Treatment Poll at the end of the blog.
He proposed that people with ME/CFS and/or hypermobility may have a different form of idiopathic intracranial hypertension, and described a course where the pressure builds and builds, causing a cerebral spinal fluid leak.
One wonders how far the vein obstruction issues will go. Yellman catalogued a variety of ways the veins can become obstructed (venous stenosis (narrowing)), anatomical problems that impinge on them (CCI/AAI, Eagle syndrome, spinal stenosis, and disc herniations. He proposed that even venous congestion syndromes found elsewhere in the body (May-Thurner, Nutcracker’s (Compression of the renal vein)) could be contributing to the venous pressure and intracranial hypertension found in the skull.
Drs. Ruhoy and Kaufman started off a recent Unraveled podcast focusing on compression syndromes that can interrupt blood flows, saying “we are sure we are on the cusp of something”. A blog is coming up.
Peter Rowe would probably agree. He recently showed that venous obstructions in the pelvic area were causing orthostatic intolerance and ME/CFS symptoms in people with interstitial cystitis. Another possible example of venous issues involves blood pooling in the abdomen and legs in POTS.
Caroline’s Story
Caroline told her tethered cord story on her “Frozen in Amber” blog.
Caroline Christian didn’t have the CCI/AAI surgery but she did have tethered cord surgery, and provided a rare complete overview of it in her “Frozen in Amber” blog.
In the “Reluctant Patient“, Caroline made it clear that she really did not want to go down the anatomical route. As someone with hypermobile Ehlers-Danlos Syndrome (hEDS), she knew she was at increased risk for these kinds of problems. Still, who wants to want to have spinal surgery? In a response to a comment, she wrote:
“For the past two years, I have had my hands over my ears, my eyes closed while saying lalalalala to block it all out. I want it to all go away. I wish we could find a comfy ledge along the steep cliff face called ME/CFS and live our best days. Sadly, progressive neurological conditions do not usually comply.”
After she was diagnosed with a neurogenic bladder, one of her doctors (who’d diagnosed her spinal fluid leak some years earlier) sent her off to get a lumbar MRI and flexion and extension x-rays, which she sent off to an expert. Her urodynamics tests indicated that nerve damage had left her unable to effectively void her urine.
He quickly diagnosed her with “occult (hidden) tethered cord syndrome”. While she was missing some common symptoms, her positive urodynamics test (pee test) trumped all. (One test involves walking on your heels and seeing if your symptoms get worse.)
She received a rather stark prognosis: get the surgery done or watch things get worse. The longer she waited to “de-tether”, the more likely that the nerve damage will become permanent and immune to further surgeries.
She was skeptical, but eventually agreed to get an invasive cervical traction (ICT) (to assess craniocervical instability – no sutures needed, by the way) and an intracranial pressure (ICP) bolt test (to assess intracranial pressure) before getting the tethered cord surgery. Bolognese uses the invasive cervical traction test to assess whether or not to go forward with the CCI surgery. He’s looking for a “clear and positive symptomatic response”. He got one with Caroline.
In “I needed that like a hole in the head: what my invasive cervical traction and ICP bolt tests revealed“, she reported that her brain fog and orthostatic intolerance disappeared! She wrote:
“I felt incredible joy having a clear brain and boundless energy! Even my vision improved, and I felt better than I had in 15 yrs – a reminder that I am still inside this broken body. I was euphoric, and I felt a deep sense of equanimity, trusting that everything would be OK.”
Caroline regularly reported on the results of her surgery. In Sept. 2022, she posted her “One-year tethered cord surgery update“.
There was no doubt the surgery had been successful – and no doubt that she was still quite impaired. She went from moderate-to-severe ME/CFS (bedbound for about 18-20 hrs a day) (only in ME/CFS might something that keeps one bedbound for most of the day be called “moderate”) to moderate ME/CFS. Her physical function gradually improved from about 20% to 50%. A physical therapist had been of immense help.
For the first couple of months after the surgery, her nervous system struggled to adjust, leaving her at times with dramatic and sudden crashes that she hadn’t experienced before.
Noting that “many people see gains after the ‘structural’ surgeries (e.g., tethered cord release, cranial cervical fusion, Chiari decompression, and other related surgeries), only to see them evaporate after several months”, at 6 months, she crashed to find that her debilitating dysautonomia, weakness, and exertion intolerance was back.
Three months later though, her vitality started to return. Somehow, she managed to sell and buy a new house during this period.
From being mostly bedbound before the surgery, she can now walk 20-60 minutes 3-4 times weekly. On the downside, her bladder dysfunction continues to decline, her cognition is still low, and her stress intolerance is still very high.
So, in end, while Caroline was still quite limited functionally, she was quite a bit more functional than before. She was also still facing many health challenges; i.e. the surgery was a help but not a cure-all.
Julie Rehmeyer, who has had CCI surgery which appeared at first to go really well but is not doing well at present, spoke to the complexities that can be present. In some cases, the problems go way beyond the anatomical issues found with CCI/AAI and tethered cord.
She noted that abdominal and jugular vein compressions and cerebral vascular issues may need to be addressed for some people to get real benefit—and there’s no central place to go where all these conditions are taken into account. Right now, there’s no clear pathway for people for whom neurosurgery did not produce good results.
Conclusion
VanElzakker believes that many roads probably lead to ME/CFS
In the end, it all seems very individual. As VanElzakker asserted – regretfully – during the webinar, there appear to be many paths to ME/CFS – and spinal issues (CCI/AAI, tethered cords, spinal stenosis, cerebral spinal fluid leaks, intracranial hypertension) have emerged as a significant one.
Woods’s “Mechanical Basis” hypothesis gets all the more intriguing when problems with venous blood flows – in the legs, gut, pelvic area – as well as cerebral fluid and lymphatic flows – get added in. One wonders how many different “mechanical obstructions” may be in play in these diseases.
While CCI/AAI surgery is a bear in every way (diagnosis, finding a surgeon, expense), it can produce dramatic increases in functioning. It seemed pretty clear, though, that fixing the anatomical problem – as important as that can be – is not always the end of the story. Nor does the surgery always produce the benefits one might have hoped for.
It was good, therefore, to see ME/CFS experts like Yellman, Ruhoy and Kaufman keeping a close eye on the spine and trying to strengthen the connective tissues before surgery is needed.
Check out the Spinal Treatment Poll
I definitely have neck issues that are tied to my ME/CFS and Fibromyalgia, and they are recurring. But one of the things I found quite helpful was local application of 880nm Infrared Light and 660nm Red Light into my neck. This seemed to allow the gentle nightly neck mobility exercises I do nighty under hot water in the shower to work much better, more reliably strengthening my neck rather than putting it out worse, which would often happen prior to using my red light pad daily.
I have worked in the field of birth defect research for 40 years and believe articles like this one do not serve the ME/CFS or Long Covid populations.
Chiari malformation is a birth defect: https://birthdefects.org/chiari-malformation/
Tethered spinal cord syndrome is a birth defect: https://birthdefects.org/tethered-cord-syndrome/
Ehlers-Danlos syndrome is a group of inherited disorders that affect your connective tissues — primarily your skin, joints and blood vessel walls.
None of these conditions fits the primary criteria for ME/CFS or Long Covid although there may be some similar symptoms.
There are three important papers on recent studies in ME/CFS. One a blood test that is 91% accurate at predicating ME/CFS.
https://www.medrxiv.org/content/10.1101/2023.03.18.23286575v2
The second, new work by one of your supporters, Dr. Nancy Klimas, who has successfully treated patients with Long Covid or post-vaccine symptoms with a compounded nebulized treatment for multiple infections:
https://www.sciencedirect.com/science/article/pii/S2666354623001345
And three, a study funded by the ME Association on Asia, a acronym for symptoms in ME/CFS and some suggested treatment protocols.
https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2024.1422940/full
Any one of these studies is more germane to the subject of ME/CFS or Long Covid. And all of these studies deserve aggressive advocacy to see if they can be replicated.
I would love to see more research in these areas in the future because a type of Chiari can develop due to intracranial pressure and because illness can cause connective tissue damage. I was not born with either but I did have brain sag (which can be mistaken for Chiari) and connective tissue issues while I was ill. And the fact is, most of us aren’t getting really thorough spine imaging and/or it’s not being read by an expert in these and related conditions. And Long Covid is vascular—some connections haven’t been explored yet.
Hi Betty!
The doctors believe that these cases are caused by inflammation not an inherited birth defect. Interestingly, people with CCI/AAI do not have ME/CFS symptoms – the ME/CFS group is different and they can be relieved by these treatments. Other things, though, are clearly going on. For most people with ME/CFS and these conditions it appears that the surgery is not a complete fix.
Thanks for the links! I’m pretty sure we did the Morten paper – looking forward to the others 🙂
Hi Cort, Please accept my apologies. You did cover the blood test in a Sept. 2023 blog. This is so important, however, that it deserves follow-up to see if these researchers are involved in continued study. No one can really know if they are being diagnosed and treated correctly without a reliable diagnostic test. What if you had some other illness with similar symptoms and you could be treated right now? The body only has so many ways to react to illness and the likelihood that several diseases could have over lapping symptoms is very high.
I was most interested in the CD8 t cell studies by Klimas et al. I have Long Covid with A-fib, high blood pressure and now problems with gait that are impairing my balance and ability to walk. The two patients referenced in this study also had the gait impairments. The nebulized drug they are using is Inspiritol, a compounded medicine, and my concern is the FDA will not approve it because no company will be able to get a patent.
Cort, I know you are a blogger and not an advocate, but I think you have the clout to make your voice heard to these researchers and perhaps regulatory agencies like the FDA.
What if the blood study and the nebulized treatment are all we need to be diagnosed and get well? Even if it requires lifelong treatment, I would take it. Not a billion dollar Moonshot, but honest replication of these studies.
As I understand it, you know Dr. Klima personally. How about getting in touch with her and asking where we stand with replicating her study and having Inspiritol available to ME/CFS and Long Covid patients.
If it works, the government should cover it just like they do vaccines and Paxlovid.
agree!
Personally I have a now ‘mild’ form of CFS. I can work full time, with pacing, but I still can’t do anything more than very mild exercise.
But what pains me most is seeing my 16 year old daughter come down with long covid/cfs.
It gets a bit soul destroying getting hopes up on these sorts of things only for there to be no follow up. This has happened a lot with Nancy Klimas over many years.
I echo Betty’s request of getting some follow up from Nancy, please. The work is published, it showed some success, so what’s not to talk about and promote?
Sorry to hear about your daughter! A Klimas blog is coming
Great, thanks Cort!
Agree. The matters in the article are an unnecessary distraction, other than the link to the research on the vague nerve which is interesting and potentially very relevant
The tissue is the issue. There’s an underlying weakness in the tissue. For some reason this is activated in some people with sub clinical eds and a degeneration occurs
All I can say, Matthias, is that you’re bucking the experience of some very experienced ME/CFS doctors! Hopefully, though, these issues are not determinative in most people. How about this? Immune activation and connective tissue damage affects different tissues in different people? In many people the blood vessels, in some both the blood vessels and the spine, in others it causes the neuromuscular restrictions Rowe has found.
All you may need to do tweak the nerves is to have “incompetent” connective tissues that moves them out of place a bit. (?)
Fair enough Cort. I must curb my cynicism
I’m completely open in whatever the answer to this riddle is.
But , in my experience, I think sub clinical EDS is ride. I also think mcas, which is part of tissue abnormality and eds can bring these problems on in later life.
This is a spectrum disease. What you describe as birth defects are just accelerated, more pronounced forms of the disease.
I have a friend with eds. Exactly the same symptoms except he has an aneurysm and chiari malformation. Everything else is identical.
I believe this is at least a subtype of m.e. CFS.
I’ve become very good at spotting fellow sufferers just in their pallor and certain features, even down to the kindve hair we have.
I believe this is a phenotype illness and m.e
Is a sub clinical ( because the science is so vague) version of eds.
There are likely a multitude of things going on but I’ve never met anyone who doesn’t have these issues in the neck so far.
I’m not saying your wrong tho. I’m just saying I think subtleties and old classifications and distinctions are seeing different diseases when they are actually the same disease with different levels of severity and expression
I too believe ME/CFS is a type of EDS. So far 14 sub-types of EDS have been identified.
And EDS does not only affect connective tissues. A French EDS specialist, Dr Hamonet, thinks that it is more a neurological illness.
It’s the building blocks of our lives isn’t it the connective tissue and tissue type.
We are not born equal. Some have resilience written into their DNA
Others have a more inflammatory, weird set of Cascades that can come from that.
I think any part of the body can be affected. Definitely the brain can be part of that.
Eds can also be brought on in later life. Acquired eds as doctor adding points out. I see that personally as just a subclinical weakness reading it’s ugly head after certain insults to the body.
I personally think tissue type in humans will be a very interesting avenue of enquiry. To me it’s the ground zero from which everything else flows. Regenerative medicine will be the ultimate treai
The first paper from Oxford was a good one. Metabolism, lipids and amino acids seem to be coming up all the time, clearly there’s something there. But no one has nailed the causative link.
I was especially interested in their findings and comments on tryptophan.
Regular use of ldn fixes this, every 2 hrs, I use 7mls, and caffiene too, although I can’t use it atm as I don’t have a good source, but the low dose naltrexone does the trick, calcium ion channels are very prevalent in the brain stem, google it. The main thing is you have to keep up the dosage.
Congratulations, Vanessa – Just checking – have you been diagnosed with any of these spinal conditions?
Hi Cort, no only CFS/ME from ebv and Fibro too, I’m very Thankful for LDN and the life it gives me, without it I cannot function, saves my heart and brain stem every time I take it, every 2 hrs.
Glad to hear Vanessa! Since LDN can reduce mast cell activation which could be attacking the connective tissues that makes sense to me. 🙂
When you are using 7ml of LDN every two hours which doseage do you end with in mg over the day? This sounds like a lot.
Hi N, it’s not really alot as it’s action for cfs only lasts for around 2 hrs, I can go longer at night, 3 to 4 to 5 hrs, but if I want to function throughout the day, 2 hrly is best, so around 50 to 80 mg a day, it’s still low dose it’s just more regular, a little caffiene is often helpful too, and can be used I find when having this much, it also helps calcium influx, I obtain this much by pretending to be a alcoholic, well you know what I mean. I also do yogic breathing and chi gong and pacing too, but I have zero pain, pem, fatigue and pots from this application. I believe one day it will be the normal treatment. I even presented my abstract at the last international ME conference in Aus on Poster board.
80mg is quite a lot, it’s 30mg above the usual tablet. So you can’t call this micro dosing anymore. Normally people start with 2.5mg and never exceed 25mg a day.
Taking naltrexone in such a high dosage and on long term bears quite a few risks because it has not been developed for long term use. It could really create a lot of health issues in the long term.
Good luck with the treatment I hope it will continue working for you.
Caffiene also increase Cerebral Spinal Fluid.
I was bedbound and severe with every symptom from a spinal CSF leak, with intracranial hypertension and venous compression likely contributing to the leak in the first place.
My connective tissue worsened with the severity of the leak and recovered immediately after the leaks were patched. The damage was a result of the condition, not the other way around. I believe there are many paths in and I would highly recommend evaluation with a CSF *specialist* before surgery.
Mast cells are vasoactive. Heart rate and breathing also affect intracranial pressures and flow. The body may be simply trying to protect the brain.
For me, medications masked those symptoms and made my condition worse overall. Now that I know what’s happening and the leak is repaired, I am able to manage the intracranial hypertension that has worsened due to my structural issues going untreated for so long. I think it’s so important to stress the possibility of intracranial pressure and spine issues and treat our bodies as if there’s an actual injury—even if the initial insult was illness.
I also think it’s important to note that, like normal MRIs, normal opening pressures may not be an indicator of actual pressures. Not the best analogy, but if you’re making balloon animals and twist the neck, the head has more pressure than the tail. Pressures fluctuate with position, medication, diet, and other factors, and the estimates of intracranial pressure issues may be underestimated.
I met a man who religiously used an inversion chair several times a day. Eventually after a period of a couple years recovered from ME/CFS. From memory he had ME/CFS for 7 years
Note: He also took glucocorticoid steroids during bad periods, so could be that. But he was adamant it was the inversion chair that helped him
Check out Liz Parrish court and perhaps do an interview.
She has regrown muscle and ligaments on herself and countless others through regenerative medicine techniques.
Surely this should be tried to stabilize and strengthen tissue before invasive procedures are considered
Thanks! I had never heard of her.
She used herself as patient zero in order to try and cure her son of type 1 diabetes.
One of the things she wanted to do as part of that was regrow muscle and ligaments. This was all measured by MRI. She was just not quite 50 and said she could feel her muscles weakening slightly.
She now has the muscle mass of a twenty five year old female and the metabolic health.
It seems to me that her therapies, which are quite well known in the regenerative field, are having great success with patients.
If we’re losing connective tissue and ligament etc, surely replacing these tissues would at least be an avenue to go by.please cort, I’d love it if you could liase with her.
I think there’s so much room for investigation.
Safer and more effective than surgery or exercise
I’m completely open in whatever the answer to this riddle is.
But , in my experience, I think sub clinical EDS is ride. I also think mcas, which is part of tissue abnormality and eds can bring these problems on in later life.
This is a spectrum disease. What you describe as birth defects are just accelerated, more pronounced forms of the disease.
I have a friend with eds. Exactly the same symptoms except he has an aneurysm and chiari malformation. Everything else is identical.
I believe this is at least a subtype of m.e. CFS.
I’ve become very good at spotting fellow sufferers just in their pallor and certain features, even down to the kindve hair we have.
I believe this is a phenotype illness and m.e
Is a sub clinical ( because the science is so vague) version of eds.
There are likely a multitude of things going on but I’ve never met anyone who doesn’t have these issues in the neck so far.
I’m not saying your wrong tho. I’m just saying I think subtleties and old classifications and distinctions are seeing different diseases when they are actually the same disease with different levels of severity and expression
Yes…my hair right at my neck is very kinky looking but only on that area of my neck although the rest of my hair is very fine and now thinner sice covid 19.
My wires hair,that was once very thick ,began falling out since covid 19 and has never come back to the thickness her hair once was
☝️correction… “wifes”
can’t seem to find the “edit” on this forum
I am trying prolotherapy for my cranial instability to try and recover the ligaments and hopefully take the heat off the Vegas nerve. I have had 4 sessions and the last session gave me 3 full weeks of good sleep less pain and no headaches. Then things collapsed and it was time for my next treatment. Doc says the three weeks should increase to longer periods each time until it stays for a year or so. I am hoping to work on some neck exercises to help the instability after several more sessions of prolotherapy. I learned to try this from some video’s I found on you tube called caring medical, prolotherapy and Vegas nerve. I am hoping this non invasive but expensive technique will help.
Great piece, as always, and the first piece that really spoke to my experience. (Not that they need to!)
I have a hx of TMJ and TOS (that nobody but me has connected to my current situation), Eagle Syndrome/IJVS which led to IIH which led to a presumed csf venous fistula which has led to SIH. So, I vacillate between low and high pressure. And what helps low pressure is hell for high pressure and vice versa. (I also have OH and MCAS.) Same with OH. If you treat the OH then your ICP goes sky high. All of this conspires to create a sort of orthostatic dementia, at least for me. Profound.
As you said, it’s really hard to find one-stop shopping for these issues or even someone who WANTS to fathom them. (It all makes perfect sense to me – a rotten sequela of issues.)
And don’t get me started on trying to get insurance to consider covering all this… 😉
(Btw, on some webinar Eric Gordon mentioned that he’s seen some help with using the peptide BPC TOPICALLY, sometimes mixed with GHK or sometimes by itself to help with ligament laxity which, in turn, has helped with dysautonomia. Sometimes a little help, sometimes a lot. I haven’t tried it myself. My funds are limited, as are peptides!)
The spine can be so complex! Definitely going to look into peptides.
And just to be clear for others reading, Gordon was talking about ligament laxity in CCI. (Some of that he sees as a mast cell/inflammation issue.)
My initial diagnosis was fibromyalgia in the mid 1990s, then CFS January 3, 1997. My brainstem was tender and extremely sensitive in the mid 1990s when I saw the doctor at Duke University who diagnosed me with fibromyalgia. I complained to him that just the pressure of a pillow on the back of my neck if I lay on my back in bed was too painful and I had to roll onto my side. The length of my spine was not a problem in the beginning days of diagnosis and early treatment but eventually it became the central issue as symptoms progressed with terrible tenderness, burning and tingling symptoms. I saw a neurologist in 2021 and was diagnosed with arthritis in my spine at that point that also caused a weakening of function in my right hand. I had an injection in my spine and after an initial worsening of symptoms for ten days, I had two months with no spine problems. I have not had another injection but I use the guaifenesin protocol for fibromyalgia and it also helps my spine issues, which today are minimal thanks to 17 years of treatment on the guaifenesin protocol. It is not a cure but the guaifenesin protocol is an effective treatment when done properly and given the necessary time to reverse symptoms. I will always need to use this treatment for the rest of my life and gladly do so because life is good and fulfilling even in my elderly years.
Guaifenesen! Is that’ something. Thanks for sharing your story.
The spine can be so complex! Definitely going to look into peptides.