07/05/2025
Wow! I guess there’s a decent amount of people with ME/CFS who are basically being cured by treatments for rare conditions like Craniocervical Instability and Tethered Cord Syndrome! This is a great find!
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Could Craniocervical Instability Be Causing ME/CFS, Fibromyalgia & POTS? Pt I – The Spinal Series
by Cort Johnson | Feb 27, 2019
Jeff had a typical ME/CFS onset: he was a young, healthy and active individual before being felled by a viral infection and a high temperature. The infection left him with headaches, dizziness, muscle weakness and pain, sound and light sensitivity, and a general sense of being worn down that was exacerbated by exercise – which he soon had to stop altogether. Socializing was the next activity to go as he buckled down to focus on getting through graduate school.
The best doctors, endocrinologists and other specialists could do was point him to a diagnosis of anxiety or depression (which he rejected). He eventually became bedbound where his research led him to a diagnosis of myalgic encephalomyelitis (ME) (myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS).
Confronting the fact that he now had a possibly lifelong case of a chronic illness was not a happy experience, but it did enable him to find an ME/CFS expert, Dr. David Kaufman, in the San Francisco Bay area.
Besides ME/CFS, Kaufman diagnosed him with postural orthostatic intolerance syndrome (POTS), a disorder characterized by a rapid rise in heart rate upon standing and mast cell activation syndrome (MCAS) – an immune condition.
Next, Jeff joined the Phoenix Rising Forums where he found many people with a similar story. Jeff’s health, though, continued to spiral downwards. Bedridden, with severe PEM, his ability to tolerate exertion essentially disappeared. Combining a shower with a 15 minute talk on the phone could leave him crashed for days.
The crashes would propel him into a world the medical community has little awareness of. Lacking the energy to chew food at times he resorted to drinking protein shakes through a straw and lost weight, becoming emaciated. He had to be wheeled into the bathroom to take a shower. He turned to earplugs for noise sensitivity and low lights for light sensitivity. At his worst, speaking or writing were beyond him.
The only way out of a crash was to stop all inputs – lie perfectly still in the dark – and hope that his body would start to revive. When it did, he poured over medical journals.
Then a breakthrough occurred – in the form of neck pain, a headache that seemed centered at the base of his skull, and a heavy-feeling head that seemed to wobble like a bobble doll when he walked.
Turning his head to the right could cause him to nearly lose consciousness. Strange and alarming symptoms – all associated with autonomic nervous system dysfunction – popped up. His dsyautonomia, formerly moderate, now spun out of control, causing his heart rate to drop as low as 30 bpm at night, causing him to stop breathing and waking him up, disoriented, gasping for air.
Losing ground, fearful he would become too debilitated to research, he redoubled his efforts, scouring medical journals for clues until one day the light bulb went on.
The condition was called craniocervical instability / atlantoaxial instability (CCI/AAI) – and it fit. (It is also called cranial-cervical syndrome, hypermobility of the cranial-cervical junction, atlanto-axial instability, atlanto-occipital instability, craniocervical instability, craniocervical injury, upper cervical instability, others.)
The strange headaches, the heavy head, the problems turning his head in one direction, the autonomic nervous system issues – they could all be explained by loosened or lax ligaments at the junction between his skull and his vertebrae which kept his head properly situated atop his body. (The atlantoaxial junction (AAI) is the most mobile joint in the body.)
With his head destabilized, his spinal column was contacting and compressing his brainstem – throwing his autonomic nervous and sensory systems out of whack. His ANS had become so disturbed that even during sleep when it theoretically should have been mostly at rest – it was oscillating up and down causing bizarre heart rates.
There was no denying the validity of an CCI/AAI diagnosis – that diagnosis is well established in the medical literature. All Jeff – an immobolized patient with one suspect diagnosis (ME/CFS) and another unusual diagnosis (to most doctors – POTS) – had to do was to convince doctors that instead of a mood disorder – anxiety – he had a real (and admittedly rather unusual) neck issue.
That turned out not to be an easy task. Although CCI/AAI has been reported to occur with rheumatoid arthritis, Ehlers Danlos Syndrome (EDS), Downs Syndrome and other inflammatory conditions, unless it was the result of some trauma such as an auto accident, it is almost always dismissed as a possibility. Dr. Kaufman was a believe,r but his diagnosis wasn’t enough. A top doctor at a well-known medical clinic sneered at his self-made diagnosis – rejecting even POTS, let alone CCI/AAI as a possibility.
Jeff came to his own rescue again. After finding that pulling up his head relieved his symptoms he got a cervical collar which helped – giving him more time to, as he put it – to “find competent medical help”. Encouraged that a potential fix (fusing the top two vertebrae in his spinal column to his skull) was possible, he soldiered on.
Joining an online CCI/AAI group provided another realization: most CCI/AAI patients were in the same boat as ME/CFS patients – often waiting years for a diagnosis. Plus, there was another connection – most had been diagnosed with POTS, MCAS and Ehlers Danlos Syndrome (EDS) – three common comorbid diagnoses in ME/CFS. (About one in fifteen of people with EDS get CCI).
The EDS diagnosis was particularly intriguing. EDS occurs when the joints become hypermobile and is often diagnosed using the Beighton hypermobility self-assessment questionnaire. Like ME/CFS, MCAS and POTS, many people with EDS remain undiagnosed.
Jeff, like Jen Brea (see below), however, passed the Beighton hypermobility test . The only thing hypermobile about him, it seemed, was the junction between his head and neck plus mild hyperextensibility of his elbows and knees.
Noticing that when he pulled his head up his symptoms lessened, he bought a Philadelphia cervical collar which helped until he took it off one day, put a less rigid one on and collapsed, suffering from partial paralysis, racing heart, dizziness, etc. even after putting the stronger cervical collar on again.
The ensuing ambulance ride to the hospital began a five-month hell-on-earth odyssey in the hospital that Jeff describes in riveting fashion in his blog. It ended when the Director of the Spine Center visited, ordered the correct imaging tests (dynamic CT scan w/ flexion and extension views), diagnosed him with CCI/AAI) and fitted him with a halo – a metal cage screwed into his skull to keep his head upright.
His dizziness, POTS and cognitive issues disappeared. He couldn’t believe how well he felt. He could talk fluently on the phone for hours without a relapse. He could read books again.
A spinal fusion by one of the world’s few CCI/AAI-literate neurosurgeons eliminated his symptoms, and they have remained gone. He’s now able to work out at the gym and is healthy. He also has good range of motion (after Dr. Bolognese used a new technique which maintains more range of motion.)
Despite the fact that Jeff didn’t have any overt neck symptoms until well into his illness, it appears that all the time he had craniocervical instability / atlantoaxial instability (CCI/AAI). He also had ME/CFS/MCAS/POTS, but all that was triggered by his CCI/AAI, and disappeared when his neck instability was fixed.
Another pathway to “ME/CFS” or rather ME/CFS-like symptoms has been identified.
https://www.healthrising.org/blog/2019/02/27/brainstem-compression-chronic-fatigue-syndrome-me-cfs-fibromyalgia-pots-craniocervical-instability/
