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

[SK2018]

I personally know of 2 people that for years had a CFS diagnosis and were finally found to have a chiari malformation and their issues resolved after minor surgery.
The symptoms of chiari are almost identical to CFS.

Just a thought...

 

[Countrygirl]

Hi @Shawn Yes, it is interesting possibility and I recall this possibility was raised a few years ago. I don;t think it was thoroughly investigated though, but thank you for alerting people to the possiblility again.

 

[mattie]

Would a chiari malformation show up on a standard brain MRI?
Or do they need to look closer to rule this out?

 

[bombsh3ll]

Sometimes a Chiari doesn't always show up on a supine MRI. To conclusively rule this out an upright MRI is best, preferably with views taken with the neck in flexion, extension and neutral. I am in the UK and had to pay privately for this. Mine was normal (I was kind of hoping to find something treatable) but it was worth it to rule it out. I have features of EDS so I was particularly keen to rule this out as it is more common in EDS.

 

[Tally]

Seems to have some similarities with CFS, but PEM seems to be a clear differentiator (immune symptoms also seem to be absent in Chiaru malformation). Still it seems like a great idea to check for people who don't have a clear-cut ME/CFS.

 

[JES]

• Neck pain
• Unsteady gait (problems with balance)
• Poor hand coordination (fine motor skills)
• Numbness and tingling of the hands and feet
• Dizziness
• Difficulty swallowing, sometimes accompanied by gagging, choking and vomiting
• Vision problems (blurred or double vision)
• Speech problems, such as hoarseness

I wouldn't say any of the Chiari symptoms I found on a quick Google search are particularly common with CFS/ME. Perhaps dizziness, but even that is more related to POTS.

 

[Gingergrrl]

@Shawn, I'm tagging @jeff_w as he knows a lot about this issue. BTW, how are you doing w/the Rituximab and other treatments?

 

[jeff_w]

Would a chiari malformation show up on a standard brain MRI?
Or do they need to look closer to rule this out?

It won't show up on a standard brain MRI. You need an upright, flexion-extension MRI. And even then, the vast majority of radiology reports will come back negative, even with obvious Chiari. You need a Chiari-knowledgeable neurosurgeon to interpret the image, not to simply read the written radiology report.

So, the steps to rule in/out Chiari Malformation:
1. Get the correct imaging. (Upright flexion-extension MRI)
2. Send it to the correct neurosurgeon. (There are only about 3 or 4 specialists in the USA)

 

[jeff_w]

Seems to have some similarities with CFS, but PEM seems to be a clear differentiator (immune symptoms also seem to be absent in Chiaru malformation). Still it seems like a great idea to check for people who don't have a clear-cut ME/CFS.

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?

 

[Rlman]

@JES just wanted to add to your post that the first part of the article you quoted says:
"Headaches, often severe, are the classic symptom of Chiari malformation. They generally occur after sudden coughing, sneezing or straining." and below it:
"Less often, people with Chiari malformation may experience:

• Ringing or buzzing in the ears (tinnitus)
• Weakness
• Slow heart rhythm
• Curvature of the spine (scoliosis) related to spinal cord impairment
• Abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep

 

[Nickster]

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?

@jeff_w Did you have digestive or neuropathy issues?

 

[jeff_w]

Did you have digestive or neuropathy issues?

Yep. SIBO and peripheral neuropathy.

 

[Nickster]

These are my sons issues. Can you tell me or pm me the neurosurgeon/neurologist you went thru? What is the name of the procedure your having? Currently Nick is seeing Dr Chheda which is Dr Kaufmans associate.

Thank you for sharing your experience.

 

[Nickster]

These are my sons issues. Can you tell me or pm me the neurosurgeon/neurologist you went thru? What is the name of the procedure your having? Currently Nick is seeing Dr Chheda which is Dr Kaufmans associate.

Thank you for sharing your experience.

I see your experience in the prior threads. What made you pursue the neurosurgeon? That seemed an area that you did not give up on.

 

[jeff_w]

These are my sons issues. Can you tell me or pm me the neurosurgeon/neurologist you went thru? What is the name of the procedure your having? Currently Nick is seeing Dr Chheda which is Dr Kaufmans associate.

Thank you for sharing your experience.

Hi, @Nickster -

Dr. Cheda will need to write an MRI order for your son. The exact order should be an MRI of the skull and cervical spine, upright with flexion-extension. Odds are, Dr. Cheda won't be familiar with this procedure. But, if she's like Dr. Kaufman, she will listen to you and write the order, especially if you bring in research supporting the need for this type of MRI.

During the upright MRI, your son needs to flex his head forward as far as he can. He'll then have a neutral image, without flexing. Then, he'll need to bend his neck backwards as far as he can. He needs to demonstrate his entire range of motion for this test. (The radiology techs don't always give detailed instructions, so I included them here. In my case, he gave me the incorrect instructions, 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 his 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.

As for me, I'm having neurosurgery with Dr. Bolognese on January 31st (as long as my insurance authorizes it). He'll be doing a craniocervical fusion from my skull to my second vertebrae. It's technically called a C0-C2 fusion.

This C0-C2 fusion usually results in a 40% loss of range of motion in the upper neck and skull. But! I chose Dr. Bolognese, because he does a more advanced fusion that only results in a 10% loss of range of motion.

Good luck to you and your son with all of this!

 

[bombsh3ll]

Radiologists aren't trained to look for this problem, craniocervical instability (CCI). So, the written report is meaningless for this purpose.

That's scary. I was reassured when I had normal upright results in flexion extension etc. I am also waiting to see a geneticist re possible EDS.

At what point did you learn you had EDS - before the MRIs or once the instability was discovered?

I wish you the best with your surgery, please do update on your progress!. I too was hoping for a treatable cause.

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.

could you post a link to the product you are wearing - a trial of this could be very informative. many thanks.

 

[Rlman]

@jeff_w was there a charge for the neurosurgeon to review your MRI? Thanks for all your help and posts!

 

[Jessrose21]

I'm very interested in getting tested for Chiari Malformation but I have a cardiac pacemaker, so an MRI isn't possible. I guess a CT scan or x-ray would be what they would use to diagnose me? I wonder if a neurosurgeon would do a trial with a halo vest first?

I've been very sick with ME/CFS for nearly 25 years now and severe aches and pains in the base of my neck have always been a symptom, especially when I've overdone it. I get headaches that I call the "helmet of pain" and must lie completely still for hours in the dark, unable to even think without experiencing waves of pain and nausea. Even on a "normal" day I have to sleep on my stomach or side with my chin tucked into my chest just to be comfortable. No idea if those are Chiari symptoms. I also get tingling in my hands and feet, and also have Raynaud's syndrome. I see stars and feel very light-headed if I have to crane my neck back and raise my arm over my head to reach something, for example. I also experience severe PEM just from taking a shower or going to a dr appt. I'm basically housebound and sit in bed or on the couch all day, propped with pillows. It would be a dream come true if CM was my problem and an operation would fix me!

 

[jeff_w]

I'm very interested in getting tested for Chiari Malformation but I have a cardiac pacemaker, so an MRI isn't possible. I guess a CT scan or x-ray would be what they would use to diagnose me? I wonder if a neurosurgeon would do a trial with a halo vest first?

@Jessrose21 -

A CT scan can sometimes catch this problem (Chiari as well as CCI). You would need to get a flexion-extension CT. A regular one is likely to miss the problem, if you have CCI.

A halo vest will help if you have CCI, but it won't if you only have Chiari.

You'll need to send your imaging (flexion-extension CT scan) to one of the three neurosurgeons in world who can diagnose these problems accurately.

I've been very sick with ME/CFS for nearly 25 years now and severe aches and pains in the base of my neck have always been a symptom, especially when I've overdone it. I get headaches that I call the "helmet of pain" and must lie completely still for hours in the dark, unable to even think without experiencing waves of pain and nausea. Even on a "normal" day I have to sleep on my stomach or side with my chin tucked into my chest just to be comfortable. No idea if those are Chiari symptoms. I also get tingling in my hands and feet, and also have Raynaud's syndrome. I see stars and feel very light-headed if I have to crane my neck back and raise my arm over my head to reach something, for example. I also experience severe PEM just from taking a shower or going to a dr appt. I'm basically housebound and sit in bed or on the couch all day, propped with pillows. It would be a dream come true if CM was my problem and an operation would fix me!

All of this could fit Chiari and CCI. You could also have Tethered Cord. Good luck!

 

[Jessrose21]

@Jessrose21 -

A CT scan can sometimes catch this problem (Chiari as well as CCI). You would need to get a flexion-extension CT. A regular one is likely to miss the problem, if you have CCI.

A halo vest will help if you have CCI, but it won't if you only have Chiari.

You'll need to send your imaging (flexion-extension CT scan) to one of the three neurosurgeons in world who can diagnose these problems accurately.


All of this could fit Chiari and CCI. You could also have Tethered Cord. Good luck!

Thank you for your quick reply! Just to clarify, a neurosurgeon is the one who would order the CT scan right, not my primary care doc?

Wow, I just looked up Tethered Spinal Cord and one of the symptoms is discoloration of the lower back. I have red striations on my lower back, right above my butt, and my dad has the same markings. His mother has the marks on the nape of her neck, I think. And Dad has always had back problems.

Dr. David S Bell also thought I had symptoms of EDS, because my fingers and wrists were hyper mobile, though they've stiffened up over the years.

All these pieces must fit together somehow, someday!