Have you ruled out Chiari or Craniocervical Instability (CCI) as a cause of your CFS

[StarChild56]

I agree with this, and I’m somewhat concerned about people doing traction without first knowing their anatomy. It’s safer to consult a specialist neurosurgeon and get a baseline MRI before doing this. But I know that’s not always possible.

You just really don’t want to be stretching out those sensitive neural structures incorrectly. There’s a neurosurgical issue called “Tethered Cord,” and traction could worsen that, if you have it. That’s why I think it’s best to see a neurosurgeon, as the traction only picks up on limited issues, when you could potentially have more complexities. I’d just hate for you guys to get worse from experimenting with this.

I am really very late responding to this but I wanted to add because I have just been diagnosed as having CCI/AAI and have the same doctor as @jeff_w and will have the same neurosurgeon...

My specialist ordered PT for me until my surgery but it is not doing any exercises - just the PT who has a doctorate and who spoke with my specialist for specific instructions - performing manual cervical (though my specialist called it cranial) traction while I lay on a PT table. I had my first appointment and eval today.

My specialist wants me to also use a home cervical traction device, but I am not to use it until I bring it in to the PT for her to show me how to use it properly. Sitting in a chair, 20 minutes a day and 20 lbs pressure.

Same thing with the cervical collar - my specialist wants me to wear it 4 hours a day till surgery. I emailed him this weekend asking if I can just go buy one as I know it will help my pain/pressure (he'd lifted my head in a gentle yet firm way in our office visit which instantly reduced the pain/pressure) but he asked that I try to wait until after he gets the report back from the PT about today's eval and session (and my feedback as to how I responded).

So I just wanted to throw out there that we may want to be cautious about cervical traction without being under a doctor's care.

ETA - I just read further and found out more about the MRI requirements...then called my MRI imaging center where I had an open MRI with the flexion/extension and the receptionist said their machine is only a .3 Tesla...I hope she is wrong - But anyway most of my questions I guess have been answered ---
Also, just out of curiosity - @jeff_w - I had a cervical MRI laying down but Dr. K added extension and flexion (which my PCP was happy to include). This is the MRI he showed Dr. Bolognese who says I'm a candidate for both the CCI surgery but also CSF leaks (which Dr. K already suspected, I was referred to Dr. C at Stanford and accepted but they are so booked up they have no idea when I could be seen - and now knowing I need CCI/AAI surgery too, obviously Dr. C at Stanford is not the person for me). But it sounded like from what I've read here that you have to have an MRI standing and I did not. Is this a new advance in diagnostics, to your knowledge? I will ask Dr. K when we have our next consult but that may be a month.

Lastly - @jeff_w - I can not thank you enough for all that you have shared here. I am completely overwhelmed with all the things I need to do for this surgery in addition to my other diagnosis I'm dealing with and it is nice to read your story and inspiring to hear of your recovery.

Thanks also to @Gingergrrl for telling me to read about your story.

 

[StarChild56]

I'm also a patient of Dr. Kaufman's - I just had an appointment with him earlier this week. He ordered a supine MRI for me as well. I was confused by that, because I've read here on the forum to get an upright MRI. I asked him about it, and he said a supine 3T MRI would be better. I don't remember the exact words he used, something about being more precise about little details, since they have to measure things that are very small. Maybe he has new information about imaging and diagnosis? I thought I'd check in with folks here to see what they think.

Okay that is what Dr. K ordered for me, too (and I had to hold my neck down for a long time, which HURT, then hold my head BACK for a long time which was not as bad - so I assume those are the flexion/extension). I did not ask why, but got it and then he showed my MRI to Dr. Bolognese "Review of MRI with Dr. Bolognese suggestive of leak with intracranial hypotension and reactive hypertension as well as probable CCI."

And added this diagnosis:
"Spinal instabilities of occipito-atlanto-axial region [M53.2X1]"

 

[StarChild56]

on the BTM FB group a lady said that feeling better with traction doesn't always mean the issue is definitely CCI or AAI, there are other brain issues that improve with traction she said, in her case some ear/vestibular issues. she was wrongly diagnosed with cci she says and had unnecessary surgery. so she says its important to have imaging show those issues (CCI/AAI) for sure before proceeding with surgery, though occult tethered cord doesn't show on imaging.

I'm so very new to this CCI/AAI thing but what my specialist told me that Dr. Bolognese does - in the OR, but before the actual surgery (and of course only after reviewing my MRI and finding me a good candidate) - is something called "Invasive / Provacative" measures/screenings...puts on the halo, and or bolts, takes measurements (I'm probably messing the specifics up) and only if this last screening proves the necessity - then he operates. So I am happy Dr. Bolognese uses imaging then additional testing before actually performing the surgery.

ETA: also, my response to the manual cervical traction, neck brace and home traction will give a good picture about the need for the surgery. But since we are going ahead with trying to schedule, I am pretty confident they are confident that both surgeries will benefit me. I do understand there are no guarantees & significant risks. Some of which my specialist already went over.

 

[Murph]

I'd like to hear a story of someone who showed their test results to one of the three doctors preferred by Jeff and heard back that they don't have CCI or a related condition.

With such a strong pattern of diagnosis among members of these forums, I'd like to rule out a systematic pattern of (potentially false) positives before increasing my confidence that the CCI/Chiari/AAI explanation that was so obviously useful for Jeff can be applied more widely.

 

[jeff_w]

With such a strong pattern of diagnosis among members of these forums, I'd like to rule out a systematic pattern of (potentially false) positives before increasing my confidence that the CCI/Chiari/AAI explanation that was so obviously useful for Jeff can be applied more widely.

It's a good question. A diagnosis of CCI/AAI/Chiari is based on physically-verifiable measurements. It's an objective diagnosis, rather than subjective one.

It's impossible to fake brainstem distortion/compression (CCI) or cerebellar herniation (Chiari) on an MRI.

The reason I recommend only 4 neurosurgeons is that they are specialists in these very areas.

I'd like to hear a story of someone who showed their test results to one of the three doctors preferred by Jeff and heard back that they don't have CCI or a related condition.

There are plenty of people in EDS forums who submit their imaging to those specific neurosurgeons, and are then told their imaging was negative. However, as of this moment, I haven't seen anyone on Phoenix Rising have that experience.

It's a great question. We'll get a lot more clarity over time.

 

[jeff_w]

I am really very late responding to this but I wanted to add because I have just been diagnosed as having CCI/AAI and have the same doctor as @jeff_w and will have the same neurosurgeon...

Also, just out of curiosity - @jeff_w - I had a cervical MRI laying down but Dr. K added extension and flexion (which my PCP was happy to include). This is the MRI he showed Dr. Bolognese who says I'm a candidate for both the CCI surgery but also CSF leaks (which Dr. K already suspected, I was referred to Dr. C at Stanford and accepted but they are so booked up they have no idea when I could be seen - and now knowing I need CCI/AAI surgery too, obviously Dr. C at Stanford is not the person for me). But it sounded like from what I've read here that you have to have an MRI standing and I did not. Is this a new advance in diagnostics, to your knowledge? I will ask Dr. K when we have our next consult but that may be a month.

It's only within the last 12-15 months that Dr. Bolognese updated his preferred imaging to be a supine 3T MRI. Prior to this, he preferred upright. The other CCI/AAI-literate neuosurgeons still prefer upright.

Lastly - @jeff_w - I can not thank you enough for all that you have shared here. I am completely overwhelmed with all the things I need to do for this surgery in addition to my other diagnosis I'm dealing with and it is nice to read your story and inspiring to hear of your recovery.

Thanks. I think you're in very good hands, and I look forward to reading about your journey with this neurosurgery.

 

[StarChild56]

Thanks. I think you're in very good hands, and I look forward to reading about your journey with this neurosurgery.

thank you ever so much

 

[Murph]

It's a good question. A diagnosis of CCI/AAI/Chiari is based on physically-verifiable measurements. It's an objective diagnosis, rather than subjective one.

There are plenty of people in EDS forums who submit their imaging to those specific neurosurgeons, and are then told their imaging was negative. However, as of this moment, I haven't seen anyone on Phoenix Rising have that experience.

These are good answers and I'm pleased to hear these surgeons are not just outputting positive results! It does seem that their extra level of specialisation gives them a higher diagnosis rate - I'd love to be a fly on the wall to hear a debate between a neurosurgeon who thinks an MRI's measurements rule out CCI and one who thinks it can be ruled in. I bet there exists some interesting complexity that would help me put some context around this whole thing.

EDIT: this sort of thing is obviously among my concerns:

on the BTM FB group a lady said that feeling better with traction doesn't always mean the issue is definitely CCI or AAI, there are other brain issues that improve with traction she said, in her case some ear/vestibular issues. she was wrongly diagnosed with cci she says and had unnecessary surgery. so she says its important to have imaging show those issues (CCI/AAI) for sure before proceeding with surgery, though occult tethered cord doesn't show on imaging.

I'm not saying she went to one of those three doctors. I'd be very interested to know where and with whom she got the diagnosis and neurosurgery.

 

[jeff_w]

These are good answers and I'm pleased to hear these surgeons are not just outputting positive results! It does seem that their extra level of specialisation gives them a higher diagnosis rate - I'd love to be a fly on the wall to hear a debate between a neurosurgeon who thinks an MRI's measurements rule out CCI and one who thinks it can be ruled in. I bet there exists some interesting complexity that would help me put some context around this whole thing.

The main point of contention is that most neurosurgeons believe CCI can only happen after a physical trauma, such as whiplash or a high-impact trauma. CCI is also generally assumed to be a very rare, acute problem that doesn't warrant much attention except in the emergency/trauma context.

In my own case, and in the case of many others, we don't have a high-impact trauma trigger. This makes it nearly impossible to be evaluated for this, because MDs will tell you it's not possible to get CCI without an obvious trauma.

In contrast, the CCI/AAI specialist neurosurgeons all have a strong interest in hypermobility of the craniocervical junction.

 

[Gingergrrl]

My specialist ordered PT for me until my surgery but it is not doing any exercises - just the PT who has a doctorate and who spoke with my specialist for specific instructions - performing manual cervical (though my specialist called it cranial) traction while I lay on a PT table. I had my first appointment and eval today.

I am dying to hear how your first PT appt went this morning @Forçe e Honra (only when you have time of course)! I am also curious from what you just wrote, did your PT consider manual cervical traction and manual cranial traction to basically be the same thing (like my PT does)?

Thanks also to @Gingergrrl for telling me to read about your story.

No problem! After you shared what was going on, it was so incredibly similar to what Jeff went through that I was hoping that you would read his story and be able to ask him some questions.

I'd like to hear a story of someone who showed their test results to one of the three doctors preferred by Jeff and heard back that they don't have CCI or a related condition.

I have the same doctor as @jeff_w and @Forçe e Honra and I currently have significantly worsening neck pain that originally started from a car accident in 2006 when I was hit and my car flipped upside down and I had horrible whiplash. However, after my doctor asked me many questions in our recent phone consult, he does NOT feel that I have CCI (and I agree) vs. that I have worsening cervical stenosis and radiculopathy. He wants me to get a regular MRI of my neck to compare to one that I had in 2010 (so it can help guide treatment in my PT or possibly provide other diagnostic info) but nothing beyond that.

The main point of contention is that most neurosurgeons believe CCI only happens after a physical trauma, such as whiplash or a high-impact trauma. And so, they won't check for CCI if the patient hasn't experienced a recent physical trauma.

That is interesting b/c I had high impact physical trauma and whiplash in 2006 but I had never heard of CCI in my life prior to your case (Jeff). In fact, my neck issues were my absolute lowest priority compared to the autoimmunity that was causing POTS, MCAS, muscle & breathing weakness, etc. I still wonder if there is any connection but I did not become severely ill until after mono/EBV and toxic mold exposure many years later. I also went into remission from autoimmune treatments and my neck did not start to get this bad until I started really using the muscles in PT & Pilates (all part of my rehab program).

 

[jeff_w]

I'd be very interested to know where and with whom she got the diagnosis and neurosurgery.

It's important to learn all you can from other CCI/AAI patients. The best way to do that is to join that BTM group, or others like it. That way, any one person's experience, whether positive or negative, can be placed in a broader context of outcomes.

You've cited one scary anecdote, but that isn't at all typical or representative of the thousands of stories in that group. I'd encourage you, and everyone else interested, to not simply take my word for this. Instead, join that group and start reading! Find out all you can. Talk to thousands of people there. You'll get much more information to consider while formulating your own conclusions. There are about 5,600 members in that group now.

 

[StarChild56]

I am dying to hear how your first PT appt went this morning @Forçe e Honra (only when you have time of course)! I am also curious from what you just wrote, did your PT consider manual cervical traction and manual cranial traction to basically be the same thing (like my PT does)?

I will definitely post tomorrow morning on my own thread - but it was wonderful
Yes, she feels that what our doc said is manual cranial therapy is manual cervical therapy - but again, not to be done to me the way it may done with most patients, has to be done in a very specific way.
Not only do I have the CCI/AAI issues - I have horrific herniations at 3 levels that are indenting the thecal sac significantly, along with bilateral foamanal (sp) stenosis, congenital narrowing, 2 other discs that are very badly bulging in what is called central and broad (all getting close or going into the dura)...and lots of arthritis and bone spurs.
My PCP was horrified - said in all her years and having many patients with neck problems she'd never seen a neck this bad and was really, really happy I have Dr. K to spearhead getting proper treatment. Even before this MRI in 2018, my MRI in 2014 showed a lot of degeneration and caused enough concern that when I had my tonsils removed in 2016, the surgeons had to be very careful and modify how they do things to not hurt my neck. (Don't remember how).

 

[MEPatient345]

It's only within the last 12-15 months that Dr. Bolognese updated his preferred imaging to be a supine 3T MRI. The other CCI/AAI-literate neuosurgeons still prefer upright.

Jeff, could you tell me what supine 3T imaging to request? I want to be checked for CCI and also cervical spine stenosis. I can access supine 3T locally.

 

[jeff_w]

Jeff, could you tell me what supine 3T imaging to request? I want to be checked for CCI and also cervical spine stenosis. I can access supine 3T locally.

Just a regular supine 3T MRI.

If your doctor is willing to write the order for flexion and extension views, that can give you even more information than just a standard static MRI (but is not necessary). Not all MRI facilities will do flexion-extension views though, and not all doctors are willing to write an order for one.

 

[Rlman]

just realized the link I posted earlier (about upright mri finding chiari that was missed by supine mri) didn't work. here it is:

http://newsroom.ucla.edu/stories/ne...stores-teens-vision-ability-to-enjoy-her-life

 

[Rlman]

btw @jeff_w what imaging diagnosed your CCI when you were in the hospital? Just wondering, as since you couldn't be vertical at the time you likely weren't able to do an upright MRI?

 

[Rlman]

This I find strange. Did her surgeon perform surgery based just on symptoms? You generally need to have imaging that confirms the diagnosis, which involves measurement of angles and/or actual distance/movement between certain structures that is abnormal.

And no, traction at home is definitely not diagnostic! We do these things because they help provide clues re: whether it’s worth the hassle of pursuing a diganosis.

Based on what she said on the FB group, it seems that her dr diagnosed based on symptoms and traction results. She said she showed other surgeons her original scans and they said surgery shouldn't have been done. Also she had low lying tonsils diagnosed as chiari, but she said they had a lot of extra room and there were no flow interruptions. That being said maybe the chiari diagnosis might not have been negligence on her surgeon's part since she was having 'horrific pressure headaches' at the time. They only went away after her ear surgery though, the chiari surgery didn't help that. She recommends everyone gets multiple opinions on their scans before going ahead with a surgery from drs who dont know each other. she herself now dealing with a lot of pain and other symptoms from that original surgery. her later ear surgery healed her symptoms like dizziness, nausea she says.

Perhaps if she had gotten 2nd opinions pre surgery they would have said worth trying surgery idk. anyways on the BTM group many people say surgery has helped immensely, and of course it saved Jeff, so not trying to say surgery is bad.

 

[jeff_w]

btw @jeff_w what imaging diagnosed your CCI when you were in the hospital? Just wondering, as since you couldn't be vertical at the time you likely weren't able to do an upright MRI?

Flexion extension CT scan, supine.

 

[Rlman]

Flexion extension CT scan, supine.

@jeff_w , if a person only shows abnormalities on the scans in flexion or extension positions and not in neutral, does that mean the person would only feel symptoms when their head/neck is in flexion or extension and not when in a neutral position?

 

[jeff_w]

@jeff_w , if a person only shows abnormalities on the scans in flexion or extension positions and not in neutral, does that mean the person would only feel symptoms when their head/neck is in flexion or extension and not when in a neutral position?

No. I had severe symptoms no matter what position I was in, including neutral. This is typical, given what I've read of many people's experiences.