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

[justy]

@jeff_w thanks so much for coming back to PR to let everyone know about your experience.

You say you also had MCAS symptoms - do you still have them? are they unrelated to the spinal issues (although it is related to EDS so...)

I don't have a solid EDS dx as i'm 48 and not as flexible as i used to be. I have a dx of hyper mobility syndrome. I also have a MVP. a double pelvic organ prolapse, MCAS, possible POTS, children that are hypermobile with health issues.

Is it worth someone like me with classic severe M.E symptoms pursuing this? (i also have immune abnormalities).

My illness began over 20 years ago with terrible shoulder and neck pain, which i no longer suffer from. As a chil i always had the feeling that my neck hurt, was too heavy for my body to carry etc. I also have tinnitus, dizziness, and tingling in hands and feet (pins and needles).

 

[bombsh3ll]

Justy, I notice you are in the UK as well -

For anyone else interested in the UK, I had my upright head and neck MRIs at the Upright MRI centre in London. You can refer yourself without needing to go through a doctor as no radiation is involved.

Upright head plus neck in flexion, extension and neutral cost £1400. You are sitting down for the scan. I cannot stand for more than a few minutes due to POTS and they also gave me a chair to put my feet up on during some of it. You get a CD with the images on to keep, and a written report. (Mine was reported as normal but like Jeff I plan to send it for an expert neurosurgical opinion).

There are also 2 other Upright MRI centres in Leeds and Birmingham I think but their scanners are not fully upright so only the London one is suitable for this purpose.

 

[jeff_w]

What was it that led to you exploring this avenue Jeff?

I pursued this once I could tell my neck was involved. For years (since 2011), I had fatigue. Then, in 2014, the fatigue became extremely severe, I developed POTS on top of that, and I became mostly bedridden. Then, in 2016, I developed MCAS on top of everything else. All this time, I had vague headaches and neck symptoms. But I had no clue that the head and neck was behind everything.

Then, in March of 2017, I could feel more severe problems with my neck. My head felt too heavy, and my neck started giving me a "bobble head" sensation when I'd walk. That's when I realized my neck was involved.

So I started looking into the neck issue, not yet realizing it was connected to everything else that had been going on for years. I then learned that head and my neck symptoms were indicating craniocervical instability (CCI) and about CCI's connection to POTS and EDS.

I read your earlier thread about IV saline, florinef etc and your presentation is very similar to mine. Did those earlier treatments that were so helpful eventually stop working?

Those earlier treatments were like applying band-aids to a severe wound. Those treatments helped control symptoms, to an extent, but they didn't help to heal the underlying problem.

I was still extremely impaired with treatment, just less so.

I have a feeling those three neurosurgeons are going to be kept busy looking at a lot of scans in the next while. Was Dr Bolgonese familiar with EDS and POTS? Just once I would like to not get a blank look and have to start from the top!

The vast majority of Dr. Bolognese's patients have EDS and POTS. Patients with these issues are his norm, not the exception.

 

[jeff_w]

You say you also had MCAS symptoms - do you still have them? are they unrelated to the spinal issues (although it is related to EDS so...)

I still have mild MCAS symptoms, but they're completely controlled with meds. I think the neck issue is related to MCAS based on my conversations with others. I know several people who had MCAS and POTS before their craniocervical fusion surgeries. Their POTS and MCAS went away after surgery.

I don't have a solid EDS dx as i'm 48 and not as flexible as i used to be. I have a dx of hyper mobility syndrome.

The diagnosis of EDS is supposed to be based off of how flexible you were when your flexibility was its most severe (usually in your teens and twenties). That's the official diagnostic criteria. If your doctor didn't evaluate you based on your flexibility back then, then you were incorrectly evaluated.

I also have a MVP. a double pelvic organ prolapse, MCAS, possible POTS, children that are hypermobile with health issues.

This all screams EDS.

Is it worth someone like me with classic severe M.E symptoms pursuing this? (i also have immune abnormalities).

I had classic severe M.E. symptoms with documented immune abnormalities.

So... Yes, it's worth it!

i always had the feeling that my neck hurt, was too heavy for my body to carry etc. I also have tinnitus, dizziness, and tingling in hands and feet (pins and needles).

I think it would be tragic to not pursue this. Good luck!

 

[Strawberry]

@jeff_w Very nice to see you back! You should be getting free care from Dr Kaufman and these 3 doctors now for all the work you have pushed their way! LOL. Thank you so much for sharing your experiences!

2 Quick questions, does Dr Kaufman know of this test, or did you go through someone else? I should see him again sometime soon and that answer would help my decision.

2nd question, a little odd... Have you ever wanted to be put on a stretcher or a rack? You know, those ancient torture machines? To relieve pressure at the base of the skull...

I have C2 C3 and atlas damage, and have often wondered if my neck was part of my problem. Yes, my head is too heavy for my neck. Hypermobile EDS, and all the fun stuff that goes along with it, although I am mild-moderate and am still able to work BARELY.

Oh, one more question. I see stars when I bend forward to spit toothpaste out in the sink. Ever have something like that? I don't seem to have POTS, but I do have OI, so it could be that or my neck.

Pretty sure I fit nicely into this same bucket... And I have always felt that justy and I matched symptom wise over the decades, she is just much worse than me. But I'm trying hard to catch up!

 

[Sushi]

Then, you'll need to mail the MRI CDs to one or more of the following three neurosurgeons: Dr. Bolognese (New York), Dr. Henderson (Maryland), or Dr. Gilete (Barcelona, Spain). These are the only neurosurgeons who consistently know how to read these images to diagnose CCI.

As for me, I'm having neurosurgery with Dr. Bolognese on January 31st

Thanks for all this great information Jeff. I'm so glad you discovered this and are having surgery at the right place. A few years ago there was a lot of interest in Chiari and a number of ME/CFS patients had surgery--but not with one of the few neurosurgeons actually qualified to do it. It didn't come out well. Unfortunately there are more neurosurgeons willing to do this surgery than are actually well qualified. Please keep us updated--I'm so glad for you!

 

[Wayne]

The exact order should be an MRI of the skull and cervical spine, upright with flexion-extension. An MRI that is flat won't help you. It needs to be upright with flexion-extension. Without flexion-extension, the problem will be missed.

Many years ago when I was looking into all this, I was told that not only did I have to have the correct MRI done, but that it needed to be interpreted by somebody who was an expert in the field of Chiari Syndrome. I contacted one of these experts in Chicago, and, as I recall, we set things in motion to have the MRI done locally, and then sent to him for analysis.

Unfortunately, nobody locally would do the MRI unless I agreed to have them analyse it as well. They assured me they knew how to do this, but again, as I recall, it didn't take long for me to get the sense they did NOT have the specialized knowledge to diagnose Chiari Syndrome correctly. It still mystifies me to this day that they would pass on doing something like a $1,000 MRI, just because they couldn't tack on an extra $200 or so analyzing fee.​

 

[Jesse2233]

@jeff_w glad to hear you found your cause and hope you experience significant improvement after the surgery!

I noticed on one of your old posts that you had some improvement from Valcyte. Any thoughts on the connection? Perhaps it's anti-inflammatory mechanism?

I ask because I'm curious if others who report improvement from Valcyte might benefit from having a chiari malformation ruledo ut.

 

[jeff_w]

@jeff_w Very nice to see you back! You should be getting free care from Dr Kaufman and these 3 doctors now for all the work you have pushed their way! LOL. Thank you so much for sharing your experiences!

Thanks. Please send Dr. Kaufman an email, stating this about the free appointments.

2 Quick questions, does Dr Kaufman know of this test, or did you go through someone else? I should see him again sometime soon and that answer would help my decision.

I did the initial research. I then presented Dr. Kaufman with my research. He evaluated it and agreed with me that craniocervical issues due to EDS were highly likely. He then wrote the very specific MRI order.

2nd question, a little odd... Have you ever wanted to be put on a stretcher or a rack? You know, those ancient torture machines? To relieve pressure at the base of the skull...

I wanted this, but the hospital refused.

I have C2 C3 and atlas damage, and have often wondered if my neck was part of my problem. Yes, my head is too heavy for my neck. Hypermobile EDS, and all the fun stuff that goes along with it, although I am mild-moderate and am still able to work BARELY.

These are all classic CCI/Chiari indicators, and hypermobile EDS is the biggest risk factor for CCI and Chiari.

Oh, one more question. I see stars when I bend forward to spit toothpaste out in the sink. Ever have something like that? I don't seem to have POTS, but I do have OI, so it could be that or my neck.

Yes.

Pretty sure I fit nicely into this same bucket...

Yep. You do. Good luck!

 

[perrier]

I had severe PEM for years, the classic ME/CFS differentiating type. Sometimes, even while lying flat, just watching a TV screen would cause severe PEM. I also had EBV (positive IGM and PCR) as well as low NK Cell function. At my worst, I couldn't speak, due to exhaustion. Overall, I've had severe POTS, MCAS, and CFS/ME. I've been hospitalized multiple times for MCAS.

It turns out that I have cranial settling, with my skull sinking downward onto my spine, causing brain stem compression. This sinking downward isn't outwardly visible, so you couldn't tell from just looking at me. (This sinking downward also is not a Chiari Malformation, but it is similar, as it can result in cerebral spinal fluid problems as well as autonomic nervous system issues.)

I needed an upright flexion-extension MRI to find the problem, and I then needed to send the MRI image to a knowledgeable neurosurgeon, as the radiology report was negative. The MRI revealed to the neurosurgeon that my skull has been sinking down into my spine, due to lax ligaments from Ehlers-Danlos Syndrome (EDS). The result is brain stem compression. My spine is protruding into my skull, touching my brain stem and compressing it. This is not supposed to happen! This causes many neurological problems, such as in fatigue (PEM-type), POTS, etc.

As for the immunological signatures unique to CFS/ME, it could be that neurological compromise results in specific immune disruption. Our nervous system and immune system are intertwined. A deficit in one system can result in the other system becoming abnormal.

At any rate, my craniocervical problem is entirely correctable with neurosurgery. I'm scheduled for surgery at the end of the month. But for now, I'm in a halo vest that's keeping my head from sinking onto my spine.

In the halo, I no longer have POTS. I no longer have PEM. My ME/CFS might have been, all along, a neurosurgical/structural problem.

Since my severe onset in 2014, I had angrily wished that this disease were curable with surgery or chemo. Well, it now seems like it is. At least the type that I have.

And how many others are just like me, but undetected?

Dear Jeff,
I'm so glad to hear from you, and I am really happy that you will finally be able to come out of this hell.

May I ask: how did you first get sick? Did you get the famous flu that never went away,or an infection?

 

[jeff_w]

@jeff_w glad to hear you found your cause and hope you experience significant improvement after the surgery!

Thank you!

I noticed on one of your old posts that you had some improvement from Valcyte. Any thoughts on the connection? Perhaps it's anti-inflammatory mechanism?

I think that Valcyte forced me to rest more, and that resting flat in bed relieved some (not all) of the brain stem compression that happened when I was upright.

It also could be that the neurological impairment from craniocervical instability weakened my immune system, and that Valcyte helped fight my reactivating EBV. But I don't think this is it. I think it was the bed rest.

I ask because I'm curious if others who report improvement from Valcyte might benefit from having a chiari malformation ruledo ut.

I think that anyone with ME/CFS would benefit from ruling this out. They'd need to rule out craniocervical instability as well as Chiari (they're separate issues but often occur together). I have craniocervical instability.

 

[jeff_w]

Thanks for all this great information Jeff. I'm so glad you discovered this and are having surgery at the right place.

Thank you!

A few years ago there was a lot of interest in Chiari and a number of ME/CFS patients had surgery--but not with one of the few neurosurgeons actually qualified to do it.

... And finding the right neurosurgeon is everything. But the neurosurgeons need to look for craniocervical instability (CCI) as well as Chiari. I actually have CCI, not Chiari, although the two often occur together, the specific testing is the same, and the (three) neurosurgeons are the same.

 

[perrier]

Thank you!



I think that Valcyte forced me to rest more, and that resting flat in bed relieved some (not all) of the brain stem compression that happened when I was upright.

It also could be that the neurological impairment from craniocervical instability weakened my immune system, and that Valcyte helped fight my reactivating EBV. But I don't think this is it. I think it was the bed rest.



I think that anyone with ME/CFS would benefit from ruling this out. They'd need to rule out craniocervical instability as well as Chiari (they're separate issues but often occur together). I have craniocervical instability.

Dear Jeff,
your suggestion about ruling this out is important. What would the first step be?Do you think that Kaufman now can check for this?

 

[jeff_w]

Dear Jeff,
your suggestion about ruling this out is important. What would the first step be?Do you think that Kaufman now can check for this?

The first step is getting the MRI order from Dr. Kaufman. He wrote mine.

The exact order should be an MRI of the skull and cervical spine, upright with flexion-extension. An MRI that is flat won't help you. It needs to be upright with flexion-extension. Without flexion-extension, the problem will be missed.

I can't say this enough: If it's not this exact order, it won't help you. Dr. Kaufman wrote this exact order for me. Be sure to check for and remind Dr. Kaufman of the exact wording if need be, just in case. Mention me by name, too, if you want.

Instructions for the MRI: The MRI happens in three parts: Flexion, neutral, and extension.

During the upright MRI, you'll need to flex your head forward as far as you can. You'll then have a neutral image, head above shoulders normally, without flexing. Then, you'll need to bend your neck backwards as far as you can. You need to push your neck to demonstrate your entire range of motion for this test, or the results will be invalid. (The radiology techs will give you some guidance, but they don't always give detailed instructions, so I included them here. In my case, he gave me really vague instructions, which gave an invalid image, so I had to repeat the test.)

A few days or weeks after the MRI, you'll receive a radiology report. Ignore it. Radiologists aren't trained to look for this problem, craniocervical instability (CCI). So, the written report is meaningless for this purpose.

You'll need to request multiple hard copies of your MRI CDs. Then, you'll need to mail the MRI CDs to one or more of the following three neurosurgeons: Dr. Bolognese (New York), Dr. Henderson (Maryland), or Dr. Gilete (Barcelona, Spain). These are the only neurosurgeons who consistently know how to read these images to diagnose CCI. If you try any others, such as a local neurosurgeon, you will be misdiagnosed.

Good luck!

 

[jeff_w]

Dear Jeff,
your suggestion about ruling this out is important. What would the first step be?Do you think that Kaufman now can check for this?

I'll add that Dr. Kaufman is extremely intellectually curious. He's driven to help us in a way that I've never seen before in a doctor.

He can't read the MRIs. That requires highly-specialized training, and only a handful of neurosurgeons in the world can diagnose these issues.

 

[Rlman]

I did the initial research. I then presented Dr. Kaufman with my research. He evaluated it and agreed with me that craniocervical issues due to EDS were highly likely. He then wrote the very specific MRI order.

So @jeff_w when did you do the Kaufman ordered MRI? before the hospital did the flexion-extension CT or after?

so flexion-extension is needed bc neutral position doesn't always show an issue? which is weird since wouldn't the patient be feeling bad in neutral as well?

all the best!

 

[jeff_w]

So @jeff_w when did you do the Kaufman ordered MRI? before the hospital did the flexion-extension CT or after?

Kaufman ordered this before I was hospitalized. I gave that MRI to my treating neurosurgeons in the hospital, but they didn't even look at it. They just did their own, invalid imaging.

so flexion-extension is needed bc neutral position doesn't always show an issue? which is weird since wouldn't the patient be feeling bad in neutral as well?

all the best!

The patient feels bad in neutral. I also felt bad just lying down, and lying down MRIs don't show the problem. The reasons for flexion-extension are: 1. The problem shows up more clearly; and 2. There are verified medical criteria for problematic flexion-extension measurements.

Here's an article from Dr. Fraser Henderson, one of three neurosurgeons in the world who can competently diagnose and treat CCI and Chiari:

"The computations derived from these models undergoing flexion and extension generate estimates of the stresses existing within the brainstem and spinal cord in the neutral, flexion and extension conditions. The estimated stresses reflect the dynamic change in stress exerted on the neural tissue."

Dr. Henderson's full article is here.

 

[Jesse2233]

@jeff_w is this a condition that can be precipitated by an acute infection?

 

[jeff_w]

@jeff_w is this a condition that can be precipitated by an acute infection?

Timing-wise, yes. But, it seems that this condition predisposes one to infection, rather than infection causing this issue. The autonomic nervous system is compromised in Chiari and CCI, which impacts the immune system.

 

[jeff_w]

@jeff_w is this a condition that can be precipitated by an acute infection?

I should also mention that prior to my ME/CFS onset in 2011 (which began as mild and later became severe), I had a severe infection that never went away.

I currently believe that this was due to CCI, not the infection itself. The CCI made it hard to clear the infection.