Craniocervical Instability, ME/CFS, and Associated Conditions:
Understanding the Connections and Challenges
by @forgetmenot7777777
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What is CCI
Craniocervical Instability (CCI) is increasingly recognized as a significant structural disease characterized by an abnormality in the alignment and movement between the skull and the upper cervical spine (the cervical vertebrae.)
It can occur due to ligament laxity, weak connective tissue or structural issues in the craniovertebreal junction.
What causes CCI?
CCI can arise for various reasons, including genetic predispositions like hereditary connective tissue disorders le.g., Ehlers-Danlos Syndrome), intections, or as a consequence of tick-borne diseases (or, of course, traumatic physical events.) These factors contribute to the weakening or laxity of ligaments in the craniovertebral junction, leading to instability.
How do Infections weaken our connective tissue?
It can happen through various mechanisms. Inflammation triggered by infections can lead to the breakdown of collagen.
Collagen is everywhere in our bodies: our bones, skin, muscles, ligaments, organs, blood vessels, and intestinal linings.
Collagen, therefore, acts as a glue to keep our bodies together.
The loss of collagen and other connective tissue components can lead to tissue degeneration, especially if the inflammation is chronic.
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How can tick-borne illnesses trigger CCI?
In simple terms, when someone has an infection like Lyme disease, their body produces a substance called Interleukin-1.
This substance then creates two things: collagenase and prostaglandins. Collagenase breaks down collagen, leading to joint damage seen in severe Lyme disease cases.
Bartonella, another tick-borne disease, thrives in connective tissue, and yet again, it targets collagen. It can mess with our body's inflammation process, leading to more inflammation than usual. This extra inflammation can damage our connective tissues. Bartonella can stick to and affect various proteins that is are part of our connective tissues (like laminin, fibronectin, and integrins, all of which are crucial components of the extracellular matrix.) ECM acts as a network of proteins and other molecules that surround the cells in the tissue and provides structural support in the body. It plays an essential part in the regulating process, like wound healing and tissue repair.
Having said all this, Bartonella can result in more inflammation and breaking down parts of the tissue. This cycle of inflammation and damage can continue, making the infection worse. - * These are only two examples of tick-borne diseases.
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How does CCI trigger ME/ CFS? PT.1
Impaired brainstem function due to CCI can lead to the disruptions and normal functioning of various systems in our body. The brainstem controls many autonomic functions, including heart rate regulation, sleep, and blood pressure.
Brainstem compression, stretching, or deformation can also lead to something called "Ischemia".
Ischemia, in short, means decreased blood flow land thus reduced oxygen); to the brain, for instance, which many ME/CFs and Dysautonomia patients are reporting.
The lack of blood flow will then lead to oxygen deprivation. When there isn't enough oxygen in our bloodstream, our bodies automatically switch to anaerobic metabolism (which includes glycolysis), producing energy without oxygen. This process also creates lactic acid.
If the theory is correct that ME/CFS sufferers chronically have too little oxygen in their blood going to their brain, the direct consequence is lactic acid buildup. Lactic Acid build-up can lead to muscle pain, fatigue, weakness, and cell death.
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How does CCI trigger ME/ CFS? PT.2
Glycolysis: when the body solely operates on anaerobic metabolism and glycolysis for energy, the ATP (think of it as a battery that powers almost everything a cell does) that is produced is less efficient, leading to quicker fatigue and less sustainable energy supply for cellular activities (meaning generating energy - ATP -, creating proteins, reproducing and growing to replace old or damaged cells, responding to signals from other cells or the environment or moving nutrients, oxygen and waste products in and out of the cells.)
In conclusion, insufficient ATP production can be triggered by oxygen shortage, mitochondrial and chronic diseases like ME/CFS, bringing us back to how ME/CFS is triggered.
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How does CCI trigger ME/ CFS? PT.3
Going back to the brainstem that CCI is causing to stretch or bend or compress: Our body is meant to stretch and bend, etc.; if we've already got a problem with our vertebrae that are stretching or compressing, stressing, the brainstem and spinal cord, even easy tasks like walking, bending our legs, lifting our arms will put a strain on our body and we will therefore be more easily exhausted.
Numerous nerves and blood vessels surround the spine and the brainstem; they are essential for movement, sensory perception, and regulating many bodily functions.
In conclusion, doing any physical activity, as little as it might be, will lead to more stress on the already irritated brainstem and spinal cord, on the nervous system, and on several other systems, which can lead to various symptoms, which could, hypothetically, also be the cause of PEM in cases of CCI and other structural diseases that led to ME/CFS.
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POTS. MCAS and the Vagus Nerve
Most CCI patients also report suffering from Dysautonomia and MCAS. Why? The Brainstem is crucial for regulating autonomic functions, as stated previously, such as heart rate, blood pressure, dizziness, and blood vessels.
CCI can impact the vagus nerve, which is one of the cranial nerves that extends from the brain down the neck, chest, and abdomen. It's responsible for the parasympathetic nervous system, which helps control involuntarily bodily functions such as heart rate and digestion, for example. If the vagus nerve is compromised, it could affect our body's ability to maintain a steady heart rate, blood pressure, and blood flow distribution, especially when going from a lying to a standing position.
How can it cause MCAS?
One possible explanation is that the mechanical stress and strain caused by CCI might activate mast cells and make them more prone to reacting to various triggers.
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The symptoms and Diagnostic Challenges
CCI presents with a range of symptoms, from neck pain and headaches to more severe neurological manifestations such as seizures, ataxia, paralysis, dystonia, facial numbness, and severe vertigo, to name just a few. Many, if not all, patients also report a symptom that describes having a "wobble head," a heavy head, the feeling that their neck can not support the head's weight, or feeling like their head is sliding off their neck.
Diagnosing CCI poses significant challenges
Accurate diagnosis often requires a comprehensive approach, including detailed medical history, physical examinations, and specialized imaging studies.
Most medical practitioners lack the specialized training and skills required to diagnose Craniocervical Instability and the comprehensive diagnostic process. Additionally, only a select number of doctors possess the necessary advanced equipment like Cone Beam Computed Tomography (CBCT) and other specific diagnostic tools, making accurate identification and assessment of CCI a challenging effort.
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Treatment Approaches and Surgery
Treatment varies based on severity. Non-surgical interventions include physical therapy, pain management, and the use of cervical collars.
However, in severe cases, especially in cases of very severe ME/CFS, particularly where neurological symptoms are pronounced, surgical intervention like occipital cervical fusion (OCF) may be necessary.
Fusion Surgery:
Fusion surgery, such as OF, is considered for patients with severe CCI, especially when accompanied by complications like jugular vein, and other type of blood vessel compression. Studies have shown significant improvement in patients with disabling CCI, particularly in the context of connective tissue disorders like EDS.
The hurdle is that these handfuls of neurosurgeons are scattered around the world, and most, if not all, patients and relatives have to pay out of pocket for these surgeries M which makes them almost inaccessible and entirely out of
1 reach for some.
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Conclusion
Understanding CCI as a legitimate structural disease is crucial in the effective management of associated conditions like ME/CFS. With its complex presentation and significant impact on patient’s quality of life, accurate diagnosis and appropriate treatment of CCI are paramount. This includes considering fusion surgery as a possible option for severely affected patients.
I am in no way saying that every ME patient has CCI or any other structural condition. I’m also not saying that surgery, for those who have CCI, is a cure-all kind of thing. Since there hasn’t been much research on the correlation between CCI and ME/CFS, these explanations are mostly hypotheses, which are plausible to me nonetheless. I do think, from personal experience, that if you have the chance, the energy, and the money, you should explore the possibility.
My neurosurgeon told me in April 2023, before my fusion , that up until then, he had operated on 300 ME/CFS patients , most of them severe to very severe. He told me that 80% get better, 10-15% stay the same. I have come across many people, thousands if you will, in groups, on Instagram, etc., and almost-all of the ones that had surgery for their CCI had a good outcome.
I know people who went into remission; I know many people who got so much better they can lead an almost ordinary life now. I only see a handful of people who either got only a little bit better, didn’t get better, or got worse. But for the most part, I can say that the majority of people with CCI and ME/CFS who underwent surgery performed by one of the few renowned neurosurgeons in the world no longer struggle to make it through the day.
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Layla @forgetmenot7777777 lives with severe ME and CCI and has created this wonderful infographic about the overlap of these conditions. This is a repost of her work and her caption follows below...
"The whole deal about CCI 👤🧠
There's so much info and theories and so much to write about, but I tried to cover the parts that are most prevalent to me at the moment. There's also a whole different issue in connection to CCI that I might make a new post about in the future. I also plan on sharing my CCI story soon, as I haven't done that yet. 🌻
I also want to say, while these are mostly hypotheses, I've spent years and years in groups, read stories from people suffering from CCI+MECFS/LYME, all of this knowledge is in my brain and can't be sourced. And the evidence for that is mostly anecdotal. I still found it important to speak about it, because anecdotal doesn't mean not true if it happens again and again and again and again.
This is my first info post, so please be aware that I've never done any "graphic design." I hope I got the message across and clarified CCI to some people since it's more common in our community than many might think!
And It's a nuanced landscape-while not all ME/CFS patients may have CCI, for those who do, understanding this connection is very important. Treatment is highly individualized, with some finding significant relief from surgical interventions, though it's definitely not a universal solution.
Where to go if you suspect CCI and want to learn more? "ME/CFS+ Brain and Spine" Facebook group. Originally opened by Jen Brea.
Here's a list of renowned neurosurgeons, that are specialized in this field. (I know, they are very few)
US:
Dr. Patel (South Carolina)
Dr. Henderson (Maryland)
Dr. Bolognese (New York)
Europe:
Dr. Gilete (Barcelona, Spain)
Dr. Oliver (Barcelona Spain)
Australia:
Dr Rao (Sydney)
