I never would have guessed this was the solution to my ME/CFS. Jeff, on Phoenix Rising
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.
Jen Brea’s CCI/ AAI Story
He’s not the only one. Some time last year, Jen Brea began experiencing similar symptoms. Her health had improved on Valcyte, fludrocortisone and pyridostigmine (Mestinon) but a surgery brought back symptoms she’d experienced before – at a new order of magnitude. First an airplane flight triggered a crash, flu-like symptoms and stabbing pains in her head, heart, and gut.
Next came flaccid limbs, numb, painful and weak legs, difficulty speaking and thinking. Then, one night, she stopped breathing – and couldn’t will herself to breathe again. Try as she might, she could not get herself to breathe: fifteen to 45 seconds later, though, her body’s automatic breathing response would kick in. Shifting to sleeping on her side helped.
When she realized that turning her head to her left triggered all these symptoms, something seems to have clicked. As with Jeff, a Philadelphia cervical collar which lifted her head to a better position, immediately ameliorated her symptoms.
After testing demonstrated that her head/neck junction was extraordinarily lax, she was diagnosed with CCI/AAI. The diagnosis made sense of some symptoms she’d never been able to fit into ME/CFS before – why turning her head sometimes left her disoriented and why sudden jolts sometimes hurt so much.
She noticed she was unconsciously hunching her upper back and shoulders in an attempt to balance her head properly on her body. Trying to defeat that by trying to throw her shoulders back and straightening her spine resulted in crippling neurological symptoms. For the first time in her ME/CFS journey, interestingly, she’s in lot of pain. Pain, she reported, “was never really a part” of her illness, before
Jen reported that in December/January she’d three surgeries: a craniocervical fusion and two surgeries in her lumbar spine to remove a hematoma that was compressing her spinal cord (caused by a blood patch) and a tethered cord release, a condition she was probably born with.
The surgeries corrected her breathing problems, the numbness, weakness and pain in her legs. A couple of months later she’s still largely bedridden but is improving.
Because these surgeries can take from 3-12 months to take full effect even in a healthy person, there’s no way to tell yet how much impact they will have on her ME/CFS, POTS, MCAS, etc. Her surgeon warned her, however, that while the surgery could help those conditions, to not expect a resolution of them. Surgery, he emphasized, is a last resort.
The Zebra Network and other websites report three central symptoms of craniocervical Instability (CCI):
- “Heavy” headaches (feeling like the head is too heavy for the neck) and a bobble-head feeling.
- Pressure headaches generated by things like yawning, laughing, crying, coughing, sneezing or straining.
- Symptoms of autonomic nervous system functioning problems such as tachycardia (rapid heartbeat, heat intolerance, problems standing (orthostatic intolerance), gut motility problems, thirst and chronic fatigue.
Other symptoms can include neck pain, central or mixed sleep apnea, facial pain or numbness, balance and coordination problems and vertigo, dizziness, fainting, vision issues, difficulty swallowing, choking, tinnitus, nausea, vomiting, paralysis, downward nystagmus (irregular eye movements).
Note that neck pain and neck issues are not mentioned. Jeff reported that, for most of his ME/CFS, his vague headaches and neck symptoms provided no clues that his head and neck were the cause of his ME/CFS.
The results on the Phoenix Rising Forums (13 people on the Forums diagnosed with CCI/AAI thus far), while anecdotal, suggest that this condition may not be as uncommon as one might think in ME/CFS.
- Check out the Tracking CCI/AAI thread on Phoenix RIsing.
On his Mechanicalbasis website, Jeff provides advice for getting tested and a diagnosis that no-one who potentially has this condition should be without including the specific imaging needed. and how to get your scans into the right hands. Most neurosurgeons aren’t trained to recognize craniocervical instability and finding an imaging facility that does these kind of upright scans can take time.
Upright MRIs with flexion, extension, and rotational views, or supine CT scans with flexion, extension, and rotational views or 3Tesla supine MRI’s. (A patient of Dr. Kaufman’s reports that the extremely strong 3Tesla MRI’s may be the best and are more readily available. Check out the difference between the 3T and 1.5T machines).
Chiaribridges reported that “the ideal tests to diagnose CCI and AAI are an upright MRI with flexion and extension (bending one’s head forward and backward as far as one can) and a 3D CT with rotational views, respectively. Ventral brainstem compression is not always seen in traditional supine MR imaging but it usually very evident on dynamic upright imaging which has the patient flex and extend their neck.
On Phoenix Rising, Jeff reported that the dynamic imaging should also check for Chiari Malformation (which can cause similar issues), Craniocervical Instability, and tethered cord syndrome (which Health Rising will cover later).
He reported that only 3 neurosurgeons in the world can help: Dr. Henderson at the Metropolitan Neurosurgery Group in Maryland; Dr. Bolognese in at the Chiari Neurosurgical Center, New York; and Dr. Gilete in Barcelona, Spain. Dr. Faheem Sandhu in Georgetown, Washington D.C. is quickly developing competence in it. Dr. Patrick Johnson, Director of the Cedars-Sinai Institute for Spinal Disorders in Los Angeles CA, diagnosed his CCI, and knew how to fix it but is not a specialist.
Merck reported that symptom improvement during a procedure called Invasive Cervical Traction (ICT) where one’s head is pulled upward by a pulley system can help diagnose CCI/AAI. Lacking that, a doctor can simply pull the patient’s head up off the spine in the doctor’s office, and see if that helps!
Wearing a Philadelphia Cervical brace helped Jen and Jeff self-diagnose themselves. (As Jeff’s condition worsened, though, the collar didn’t help at all; i.e. it may not help everyone).
- Comorbid Diseases and ME/CFS/FM Mimics – check out the growing list here.
Surgery was the only option for Jeff and Jen but that’s not so for others with this condition. Dr. Bolognese reports that treatment of craniocervical instability typically begins with more conservative medical management, such as neck bracing, activity limitation, physical therapy (including isometrics, sagittal balance, core strengthening and cardio), and pain management.
One of his patients diagnosed with CCI reported on the Phoenix Rising forums that manual traction (having someone pull on one’s head while lying down can help) and prescription interventions have helped quite a bit. She said it’s hard to tell what has made the ultimate difference but has had major improvements. A physical therapist showed Jeff’s family how to do manual traction properly. Some people with CCI also benefit from home neck traction devices.
Sleeping in a bed with the feet elevated and the head down in the Trendelenburg position helped Jeff.
Wayne whose believes the health issues that led to his ME/CFS started with a serious head injury/whiplash as a teenager, and who believes he probably has CCI/AAI and/or cervical stenosis, has found substantial relief from a procedure called AltasPROfilax and specialized chiropractic care. The Perrin technique is another possibility for those with neck/head issues. Find out more about Wayne’s and other’s approaches to possible CCI/AAI and other neck issues here.
Some people with ligament laxity have improved using the Cusack Protocol. Regenex is another procedure mentioned. In it, bone marrow concentrate (BMC) that contains a stem cell fraction (same-day stem cell procedure) is injected directly into the lax ligaments.
If you have EDS check out how to find the right physical therapist to help with ligament laxity.
Surgery is considered the last option. Few neurosurgeons are competent in this surgery, travel will likely be required, and wait times to get the surgery done can be long. During craniocervical fusion the skull is pulled upward and placed into the correct position, and then the occipital bone of the skull is fused to the upper cervical vertebrae to hold the corrected position. A halo or cervical brace is worn while the bones completely fuse together.
“If you are a surgical candidate, I highly encourage you to go slow, research all the options, seek second opinions, talk to other patients, understand the risks, and to think not just about the surgery but about pain management, your personal reaction to anesthesia and medications, and your post-surgical care plan, which may require additional care-taking at home as well as a plan for physical therapy.”
Other Brainstem or Spine Compressing Conditions
Doctors regularly rule out thyroid issues, sleep apnea, etc. before diagnosing ME/CFS/SEID. They need to start ruling out these neurosurgical issues as part of their first line of investigation. Jeff
Hip, on the Phoenix Rising Forums, pointed to a number of conditions which can cause brain stem or spinal cord compression and cause symptoms similar to those found in ME/CFS/FM:
- cervical spinal stenosis — spinal canal becomes too narrow, which can put pressure on the nerves
- syringomyelia — fluid-filled cyst in the spinal cord which compresses the spinal nerves
- Chiari malformation — where brain tissue is pushed into the spinal canal due to a skull which is too small
- tethered cord — where spinal cord is “stuck” to a structure within the spine such as scar tissue
- craniocervical instability — instability of head & neck bones compressing the brain stem or upper spinal cord.
Other spinal conditions that may cause or contribute to ME/CFS/FM/POTS include cerebral spinal fluid leak and intracranial hypertension.
The growing list of potential ME/CFS/FM mimics provides more clarity but also adds complexity to an already complicated disease. Future blogs will attempt to bring some more diagnostic clarity to the structural issues that may be occurring.
- Read Jeff’s full story and his diagnostic suggestions here
- Read Jen Brea’s “A New Diagnosis to Add to the List”
- Read Jen Brea’s February update
- Read Jen Brea’s May update – her longtime POTS is gone.
- Read the massive “Have you ruled out Chiari or Craniocervical Instability (CCI) as a cause of your CFS” thread begun in Jan 2018 on Phoenix Rising for much more on this subject.
- Check out the Tracking CCI/AAI thread on Phoenix RIsing. Thus far 13 people have tested positive for it.
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