Tracking CCI / AAI MRI & Treatment outcomes

[Scotty81]

My daughter has had the necessary cervical imaging and we've sent that to Dr. Gilete. I'll post her anonymized report later on as we have recently received it. Because there are no reference ranges in the report, we've been trying to understand 1) how her imaging measurements compare to the pathological ranges and 2) how they compare to others who have also graciously shared their results. This may be a fool's errand since I've read on this post that one can have only mild deviations from normal ranges and yet be greatly affected with symptoms.

Nevertheless, I looked through the pages in this thread and have put everyone's measurements in one Excel file. Since it is easier to interpret trends from pictures rather than from tabular data, I've also color coded the results so once can glance at the table to see what it is telling you. There's a snippet below.

Because this forum does not allow one to post an Excel file, I simply renamed the file with a .txt extension. If you are interested in viewing the file, download it to your PC, and rename the file's extension from .txt to .xlsx. If everything works like it is supposed to, then you should be able to successfully open the file.

I also list the reference ranges that JenB noted on the mepedia article. If additional people get measurement data from Dr. Gilete or Bolognese, feel free to add your data to this file. Alternatively, I can add your results to the file if you post them. I'm certainly not listing anything that others haven't already publicly posted. Right now, the printed file fits on 2 pages on my printer, but as more people post, that will grow.

If anyone would care to comment on what this data is showing, that would certainly be helpful. I'm tagging the following since it is their data that I've posted. Also tagging jeff and JenB so they are aware of these results all being in one place. Note: I only included people who have posted any portion of their measurement data in this thread. Apologies in advance if I've missed anyone.

@AnkeC @bombsh3ll @brando @debored13 @User891 @HansG @Joly @LJS @Mânca @mattie @moonell @Silencio @Yuno

@jeff_w @JenB

 

[JenB]

Hi @Scotty81 this is really interesting!

Note that there are some established references ranges based on consensus for some of these measurements. Also, some of these measurements have been well-studied, so we know what the population average is. The dynamic measurements have NOT been well-studied. We also do not know what the reference range should be in flexion or extension, either.

I’ll have a look when I’m on my computer. Thanks for compiling.

For reference:
https://www.me-pedia.org/wiki/Craniocervical_instability#Measurements
https://www.me-pedia.org/wiki/Clivo-axial_angle
https://www.me-pedia.org/wiki/Grabb-Oakes_measurement

 

[frozenborderline]

Because there are no reference ranges in the report, we've been trying to understand 1) how her imaging measurements compare to the pathological ranges and 2) how they compare to others who have also graciously shared their results. This may be a fool's errand since I've read on this post that one can have only mild deviations from normal ranges and yet be greatly affected with symptoms.

Can you simply post what her measurements are in all positions, i have brain fog and can't make heads or tails of the document, which is the reference range,which is her measurements etc.

I know about cxa mostly but one can Google all the other measurements ranges. Translational Bai is important and can be calculated but response to traction and dynamic bdi can be positive even if other imaging is not

 

[Scotty81]

@debored13

I was planning to post my daughter's imaging report from Dr. Gilete anyway. So, here are the results:

The tip of the odontoid peg lies below level of Chamberlain's line.
No significative cerebellar tonsils ectopia.

Craniocervical Junction Measurements:
CXA Neutral 143 / Flexion 138 / Extension 150
BAI Neutral 4.8 / Flexion 5.8 / Extension 4.3
Grabb Neutral 6.8 / Flexion 7.3 / Extension 5.4
Translational BAI: 1.5 mm
BDI Neutral 1.5 / Flexion 3 / Extension 4.2

C1-C2 neutral misalignment.
Rotary AAI looking left and right.

Miss. X meets clinical and neuroimaging international criteria for diagnosis of craniocervical instability (CCI)* and atlantoaxial subluxation(AAI).

Cervical Subaxial:
Signs of subaxial instability at:
Mild C3-C4 & C4-C5.
Cervical cinerradiology or DMX will be recommended to finally assess subaxial instability.

Impression
I think that it would be convenient to rule out a Mast Cell Activation Disorder as this syndrome could coexist with craniocervical instability & cervical instability and related clinical pictures.

After reviewing Miss. X's case, though further investigation is needed (see below) and although surgery is the last option to be considered, we think that she could be candidate for:

Occipitocervical posterior fusion stabilization with intraoperative reduction (traction).Number of levels: depending on DMX or cineradiology once subaxial instability evaluated.

Further testing recommended

• Cervical cinerradiology or DMX
• To rule out a Mast Cell Activation Disorder
• MRI Venogram, in order to see all intracerebral venous system and cervical (neck) venography in all internal jugular vein trajectory from lateral sinus to subclavian vein.
• Ophtalmologic consultation

Diagnoses
Principal diagnoses related to current situation:
1-Craniocervical Instability (CCI)
2-Atlantoaxial Instability (AAI)
3-Subaxial Instability probably at C3-C4 & C4-C5 (to be confirmed by cinerradiology)

Recommendation (after further testing and face to face consultation)
Occipitocervical posterior fusion stabilization with intraoperative reduction (traction).
Number of levels: depending on DMX or cineradiology once subaxial instability evaluated.

 

[frozenborderline]

@debored13

I was planning to post my daughter's imaging report from Dr. Gilete anyway. So, here are the results:

The tip of the odontoid peg lies below level of Chamberlain's line.
No significative cerebellar tonsils ectopia.

Craniocervical Junction Measurements:
CXA Neutral 143 / Flexion 138 / Extension 150
BAI Neutral 4.8 / Flexion 5.8 / Extension 4.3
Grabb Neutral 6.8 / Flexion 7.3 / Extension 5.4
Translational BAI: 1.5 mm
BDI Neutral 1.5 / Flexion 3 / Extension 4.2

C1-C2 neutral misalignment.
Rotary AAI looking left and right.

Miss. X meets clinical and neuroimaging international criteria for diagnosis of craniocervical instability (CCI)* and atlantoaxial subluxation(AAI).

Cervical Subaxial:
Signs of subaxial instability at:
Mild C3-C4 & C4-C5.
Cervical cinerradiology or DMX will be recommended to finally assess subaxial instability.

Impression
I think that it would be convenient to rule out a Mast Cell Activation Disorder as this syndrome could coexist with craniocervical instability & cervical instability and related clinical pictures.

After reviewing Miss. X's case, though further investigation is needed (see below) and although surgery is the last option to be considered, we think that she could be candidate for:

Occipitocervical posterior fusion stabilization with intraoperative reduction (traction).Number of levels: depending on DMX or cineradiology once subaxial instability evaluated.

Further testing recommended

• Cervical cinerradiology or DMX
• To rule out a Mast Cell Activation Disorder
• MRI Venogram, in order to see all intracerebral venous system and cervical (neck) venography in all internal jugular vein trajectory from lateral sinus to subclavian vein.
• Ophtalmologic consultation

Diagnoses
Principal diagnoses related to current situation:
1-Craniocervical Instability (CCI)
2-Atlantoaxial Instability (AAI)
3-Subaxial Instability probably at C3-C4 & C4-C5 (to be confirmed by cinerradiology)

Recommendation (after further testing and face to face consultation)
Occipitocervical posterior fusion stabilization with intraoperative reduction (traction).
Number of levels: depending on DMX or cineradiology once subaxial instability evaluated.

That cxa is not terribly abnormal but it's also not totally normal.
I forget what the normal range for translational BAI and the other measurements is.

I feel that Gilete can be a little too eager with duagnosis compared to the other surgeons. How does your daughter respond to traction? That can be an important test.

 

[Diwi9]

I have not been keeping up with these threads on CCI, but wanted to give an update.

I was an early screen with Dr. B and informed that I have CCI. Dr. Chheda had me do three courses of different kinds of traction prior to my official consultation with Dr. B.

I had quality response from traction in terms of pain and intense muscle spasms, both manual and weighted over-the-door traction. I did not feel a miracle in terms of my symptoms. However, my symptoms have been improved by interventions with Dr. Chheda.

At this time, I can do more than most with ME/CFS, but much less than a healthy person. I currently struggle most with cognitive and sensory issues on a chronic basis, while my fatigue waxes and wanes. My POTS is also variable, but mostly controlled through medication.

Dr. B said that I was not a candidate for surgery at this time. He DX'd my CCI as mild and that my cerebellar tonsils were in the "gray zone" (basically dropped, but not to a point there is a definite indication for intervention). He said I may have some level of intracranial hypotension, but this would require further diagnostics.

His advice to me was to avoid trauma to my neck, continue traction on a palliative basis, continue core strengthening exercises (I was lucky to find an excellent PT who has already assisted me with this),

He also explained that my bilateral hand and arm parasthesia is from bulging and herniated discs at lower levels, so not CCI, however the CCI and the bulges and herniations are all likely a result of my hypermobility and prior athleticism.

I felt the consult with him was worth the time, energy, and cost for the peace of mind. He was familiar with my records, which were extensive, and he was friendly.

 

[PNWMom]

My daughter had a consult with Dr B this week.

Seven years ago, she was diagnosed with ME/CFS by an ME expert MD. Since then, she also has been diagnosed with POTS, seronegative Sjogren’s, gastroparesis, MCAS, EDS and other stuff. She has been seen at the Mayo Clinic and had a full genetic exome map.

Starting in July, she had had some astonishing results in PT when her therapist stabilized and put just a tiny bit of pressure on her C2. This manual traction calmed a lot of her overt neuro symptoms. The symptoms returned as soon as the PT released her. This particular PT is something of a dysauonomia/EDS specialist and has sent patients to Dr B (and Dr B’s patients have gone to him as well). His reports of working with Dr B mostly reassured us that Dr B doesn’t recommend surgery to everyone.

Reluctantly, her neuroimmunologist ordered T3 flexion/extension MRI imaging. The resulting report dismissed any abnormalities but we expected that from radiology. The neuro told us that many of her patients who have done surgical fusion for CCI have expected miracle results and haven’t had them so to be cautious.

Dr B diagnosed my daughter with complex Chiari type 1 and CCI. He said she is definitely a surgical candidate but he told her to be fully educated before she made the decision. She would have two surgeries two months apart. The Chiari would be first. Depending how that went, the CCI surgery would follow. At this point, she is planning on the Chiari surgery for sure.

 

[nokmax76]

Does anyone know how Jeff from MEchanical basis and Jennifer Brea are doing as of now? Are they still in remission/cured?

 

[JenB]

My daughter had a consult with Dr B this week. She was diagnosed with ME/CFS by an ME expert MD. She also has been diagnosed with POTS, seronegative Sjogren’s, gastroparesis, MCAS, EDS and other stuff.

Starting in July, she had had some astonishing results in PT when her therapist stabilized and put just a tiny bit of pressure on her C2. This manual traction calmed a lot of her overt neuro symptoms. The symptoms returned as soon as the PT released her. This particular PT is something of a dysauonomia/EDS specialist and has sent patients to Dr B (and Dr B’s patients have gone to him as well). His reports of working with Dr B mostly reassured us that Dr B doesn’t recommend surgery to everyone.

Reluctantly, her neuroimmunologist ordered T3 flexion/extension MRI imaging. The resulting report dismissed any abnormalities but we expected that from radiology. The neuro told us that many of her patients who have done surgical fusion for CCI have expected miracle results and haven’t had them so to be cautious.

Dr B diagnosed my daughter with complex Chiari type 1 and CCI. He said she is definitely a surgical candidate but he told her to be fully educated before she made the decision. She would have two surgeries two months apart. The Chiari would be first. Depending how that went, the CCI surgery would follow. At this point, she is planning on the Chiari surgery for sure.

I am surprised that this ME doctor has had many patients who have had CCI surgery. Do you have any sense if this is recent?

Best of luck with all this!

 

[JenB]

Does anyone know how Jeff from MEchanical basis and Jennifer Brea are doing as of now? Are they still in remission/cured?

it has not been a panacea (I still have MCAS), but no PEM, no pots, no neuro or gastro symptoms, no dysautonomia. I expect that to remain true unless I injure myself or eventually develop adjacent segment disease/instability.

 

[nokmax76]

it has not been a panacea (I still have MCAS), but no PEM, no pots, no neuro or gastro symptoms, no dysautonomia. I expect that to remain true unless I injure myself or eventually develop adjacent segment disease/instability.

Oh, hello. I'm not on the site much and only did a quick scroll through the thread, so didn't realise you were the actual JenB On behalf of myself and all the others you've helped with you advocacy, thanks for all you've done and continue to do.

While your surgery may not have been a total cure, it is a remarkable, courageous and inspiring transformation you and Jeff have been through and I truly hope your expectations are met as regards the improvements being permanent.

I have only just stumbled on this fascinating development in ME treatment and I need to read the thread properly, which of course here in Fogland, means very slowly, and do further research. I'll probably be back in the New Year with more questions and my own story.

 

[nokmax76]

Actually, sod it. I'm going to tell my story now. I haven't mentioned it anywhere here before but I have Chiari type 1 malformation with syringomyelia. I first developed the classic symptoms of both conditions (nerve numbness, nystagmus, instability, swallowing problems, muscle weakness and headaches when sneezing or coughing) over thirty years ago. I then had three operations in under two years to try to correct the conditions. As far as the symptoms of Chiari go, the operations were successful. The swallowing and headache problems disappeared while the other symptoms have declined at a much less rapid rate than before the surgeries. Scans at the time of my last operation showed my syringomyelia cyst was like a dark balloon in my spinal canal now it's more like a magic marker line.

However, about one year after the last operation I noticed new health problems that I now know to be symptoms of CFS. I'll get on to this in a bit; but it's important here to note I did not have ANY of these symptoms prior to my diagnosis of Chiari or at any point until way AFTER the last operation. It took around two years of tests for the doctors to find out that I had Chiari and during that time NOT ONE of the twenty plus symptoms I can name now affected me then. NOT ONE. I want to make this clear because while, on preliminary reading, CCI may be a viable mechanical basis for ME, I am really not sure, given my own experience, that Chiari type 1 is. Therefore, I urge caution before going ahead with such a serious clinical procedure like decompression surgery for Chiari on the basis that it will alleviate ME issues. If anything, for me, I have narrowed my health issues down to either: the stress of the major operations in a short space of time triggering a possible genetic pre-disposition to ME or the delicate surgeries themselves permanently upsetting my neurology and triggering my ME. Now, I am not wedded to either conclusion - and maybe more research will change my mind - but I am close to walking one of them down the aisle.

As you can imagine, I have searched like a fiend to find the cause of my particular symptoms. I had a tremendous, twenty-year battle with my consultants to get them to take my post-surgery health problems seriously. You all know the issue with arrogant, lofty or just plain stumped doctors being frustrated or dismissive of CFS patient testimony especially when they can't spot anything on standard tests. They flat-out denied that Chiari could be the cause of my newly developed, profound fatigue, PEM, chronic pain, brain fog and other exotic symptoms like night sweats and shortness of breath. And due to lack of mainstream evidence to support my complaints they thought all the symptoms were in my head anyway. Their remedy, of course, was the usual referrals to pyschologists, pyschiactrists and anti-depressant prescriptions. Eventually I shook off their ignorance and found my way to alternative medicine and self-help through sites like this.

Part of me will never forgive the doctors for the way they treated me back then. Especially as a lot of it was pre-internet so I was devoid of connection to others in the same boat. Much more importantly though, I suspect that they might be right about Chiari not being the direct cause of my symptoms. Added to what I've already said, I will explain more once I have looked at the new information and gone over my old notes. But again, I urge caution. In the cause of doing research about ten or fifteen years ago, I recall a Dr in a hospital in the US (the name escapes me) that carried out Chiari-related decompressions as a "miracle cure" for CFS. This Dr was sued something like 30 times by patients whose conditions worsened after the surgery. Please be careful people. I don't want to rain on anyone's parade but there is a world of difference between hope and hopium. The latter can be very, very dangerous.

 

[JenB]

Actually, sod it. I'm going to tell my story now. I haven't mentioned it anywhere here before but I have Chiari type 1 malformation with syringomyelia. I first developed the classic symptoms of both conditions (nerve numbness, nystagmus, instability, swallowing problems, muscle weakness and headaches when sneezing or coughing) over thirty years ago. I then had three operations in under two years to try to correct the conditions. As far as the symptoms of Chiari go, the operations were successful. The swallowing and headache problems disappeared while the other symptoms have declined at a much less rapid rate than before the surgeries. Scans at the time of my last operation showed my syringomyelia cyst was like a dark balloon in my spinal canal now it's more like a magic marker line.

However, about one year after the last operation I noticed new health problems that I now know to be symptoms of CFS. I'll get on to this in a bit; but it's important here to note I did not have ANY of these symptoms prior to my diagnosis of Chiari or at any point until way AFTER the last operation. It took around two years of tests for the doctors to find out that I had Chiari and during that time NOT ONE of the twenty plus symptoms I can name now affected me then. NOT ONE. I want to make this clear because while, on preliminary reading, CCI may be a viable mechanical basis for ME, I am really not sure, given my own experience, that Chiari type 1 is. Therefore, I urge caution before going ahead with such a serious clinical procedure like decompression surgery for Chiari on the basis that it will alleviate ME issues. If anything, for me, I have narrowed my health issues down to either: the stress of the major operations in a short space of time triggering a possible genetic pre-disposition to ME or the delicate surgeries themselves permanently upsetting my neurology and triggering my ME. Now, I am not wedded to either conclusion - and maybe more research will change my mind - but I am close to walking one of them down the aisle.

As you can imagine, I have searched like a fiend to find the cause of my particular symptoms. I had a tremendous, twenty-year battle with my consultants to get them to take my post-surgery health problems seriously. You all know the issue with arrogant, lofty or just plain stumped doctors being frustrated or dismissive of CFS patient testimony especially when they can't spot anything on standard tests. They flat-out denied that Chiari could be the cause of my newly developed, profound fatigue, PEM, chronic pain, brain fog and other exotic symptoms like night sweats and shortness of breath. And due to lack of mainstream evidence to support my complaints they thought all the symptoms were in my head anyway. Their remedy, of course, was the usual referrals to pyschologists, pyschiactrists and anti-depressant prescriptions. Eventually I shook off their ignorance and found my way to alternative medicine and self-help through sites like this.

Part of me will never forgive the doctors for the way they treated me back then. Especially as a lot of it was pre-internet so I was devoid of connection to others in the same boat. Much more importantly though, I suspect that they might be right about Chiari not being the direct cause of my symptoms. Added to what I've already said, I will explain more once I have looked at the new information and gone over my old notes. But again, I urge caution. In the cause of doing research about ten or fifteen years ago, I recall a Dr in a hospital in the US (the name escapes me) that carried out Chiari-related decompressions as a "miracle cure" for CFS. This Dr was sued something like 30 times by patients whose conditions worsened after the surgery. Please be careful people. I don't want to rain on anyone's parade but there is a world of difference between hope and hopium. The latter can be very, very dangerous.

Can I ask, did you have your Chiari surgery with an EDS-literate neurosurgeon, one aware of “complex Chiari?” that is, Chiari + CCI? Or tethered cord syndrome?

I say this only because in my limited experience, getting to maximum healing requires that ALL your issues, structural or otherwise, are accurately diagnosed. It is very common to have more than one neurosurgical problem. It is also common to have MCAS.

Chiari decompression surgeries often fail b/c co-morbid CCI or subaxial instability isn’t attended to. Craniocervical fusion often result in worsening symptoms, much later, because of tethered cord syndrome. And then there is the issue of vascular stenosis...so many possibilities.

I am so incredibly sorry for all that you have been through and encourage you to keep digging. It would not surprise me in the least given your history of Chiari and Syringomyelia if you may not have some of these other conditions, too: https://onlinelibrary.wiley.com/doi/full/10.1002/ajmg.c.31549

In my view, any condition that causes transient ischemia of the brain/spinal cord with activity can potentially cause the symptom., PEM.

 

[JenB]

I’d also just mention that there is a long history of Chiari decompressions “failing” because the cases were actually complex Chiari, and the CCI was not addressed. This was something many neurosurgeons missed in the 1990s/00s. @jeff_w

 

[PNWMom]

I am surprised that this ME doctor has had many patients who have had CCI surgery. Do you have any sense if this is recent?

Best of luck with all this!

It wasn’t the ME doc (I probably misstated it). It is her physical therapist (DPT). He sees a lot of patients who are affiliated with the University of Utah team (Melissa Cortez, neurology; Laura Pace, neuro-gastroenterology). I don’t know how he came to be so well known in the dysautonomia community. I assume he has also seen patients of Dr Bateman simply because of geography. I do know that people fly in from outside Utah to see this PT. He is obsessed with the connection between the nervous system/neurological disorders and body mechanics—it is something he has been doing for at least 5-7 years. He uses the most updated criteria for EDS and if he perceives that someone meets them, he will often refer them to an MD who can officially diagnose them,

 

[winterschlaf]

Diagnosed with CCI and "Likely AAI" by Dr Gilete, Barcelona.

Also: loss of cervical lordosis, protruding discs at C5-6 and C6-7, degenerative disc at L5-S1.

Upright imaging done at Medserena Manchester, UK. Was read negative / all clear by Prof F Smith.

Fundraising for trip to Barcelona for further testing to confirm AAI, conclusively rule out subaxial instability, and to investigate symptoms of raised intracranial pressure and suspected vascular involvement.

I have begun experiencing many clusters of focal seizures, daily and nightly, and respiratory distress which matches accounts of central apnoea.

If I can crowdfund successfully, and it is offered following a full face to face evaluation, I will have fusion surgery in Barcelona asap.

 

[lagarto]

Diagnosed with CCI and AAI by dr Gilete, in person. All scans done in Barcelona through dr Gilete. I am considering surgery.

 

[Mendo Zebra]

I had a video consult with Dr Bolognese, who says I’m a gray zone mystery. My morphometrics are basically negative for craniocervical instability/CCI. Possible vertical instability but my s/s don’t fit that either. My during traction reaction wasn’t positive enough. I had an intense hour long seizure like rebound after traction, which he thought was a bit of a mystery. I have epilepsy and because of my risk of seizures and traction rebound reactions he doesn’t think I should have any more traction or invasive diagnostics at this time. He thinks I should get better seizure control med management and my mast cell activation better controlled and then try traction again very carefully putting a cervical collar on as traction is released. And if I have a very positive reaction to the traction get in touch with him.
Basically not enough s/s or diagnostics for CCI but enough that he can’t rule it out either.
Same goes for tethered cord, although he did say I should have an X-ray of my lumbar region.
When I asked about progression he didn’t really have a definitive answer.
I just was hoping Dr B would have a little more insight in my situation. And Yes he says other patients are like me and they just don’t know enough yet...

I did not remember to ask what my measurements are, hopefully, I will get those in copies of his notes.

* He also said if not for the seizure risk he would reco trying more traction “but I don’t want to give your PT a heart attack.”

I really want to know what happened to me when I had the intense traction rebound neuro crash. I wonder if there’s something going on with my odontoid? This wasn’t addressed. Nor was the fact that Chiari hasn’t been 100% ruled out.

And I wanted to share a few details about Dr Bolognese’s initial consultation.

Questions he asked:
“When do you feel the worst?”
“When do you feel the best, most comfortable?”
“What has helped you the most?”

I had written up a detailed list for him ahead of my appointment but these questions caught me off guard a bit and I am still not sure I represented myself accurately.

 

[lagarto]

Diagnosed with CCI and AAI by dr Gilete, in person. All scans done in Barcelona through dr Gilete. I am considering surgery.

Decided against surgery, for the time being at least. They would need to fuse down to T1, which wasn't clear from the beginning.

 

[Bidding lady]

My daughter, Manon, Diagnosed with ME - POTS - EDS five years ago has had diagnosis of CCI and AAI. MRI done last summer in July. Scans sent to Gilete who confirmed diagnosis. We are waiting to hear from them for visit for her to undergo traction.