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

[SWAlexander]

minimalist: Suggesting to seek a second opinion from a university clinic.

 

[kewia]

This thread is 113 pages long, I don't have the ability to read all that, so forgive me if this has already been addressed. Does swelling of the brain cause this?

I think CCI can have many causes: injury, you're just born with, and probably infections degrade ligament tissue causing instability.

"Bilateral internal jugular vein compression" is because of CCI, and what I can do now.

Good question, I can hardly imagine that, but if it does, it would depend on the severity, I think. However, there might be other reasons, too.
This thread may help you.
Note, you can also give a vote of things you have in this CCI survey.

 

[minimalist]

Thanks, @kewia!

 

[valentinelynx]

This thread is 113 pages long, I don't have the ability to read all that, so forgive me if this has already been addressed. Does swelling of the brain cause this?

Not sure what you mean, exactly. Is it a general question about the cause of craniocervical instability? If so, the answer is, no, it's not caused by brain swelling. I can think of a way they could be connected—an inflammatory process that causes brain swelling could also cause inflammation with subsequent degeneration of the ligaments at the base of the brain, namely, in the craniocervical junction.

Craniocervical instability is usually caused by ligamentous laxity at the junction of the brain and spinal cord, leading to hypermobility at the very complex craniocervical junction. This hypermobility causes the brainstem to be compressed or intermittently pinched by the top of the spinal column, the odontoid. Ligament laxity can result from traumatic disruption, as in a car accident, genetic connective tissue disease, like Ehlers Danlos Syndrome, or inflammatory processes like infections or autoimmune disorders. Other causes can include congenital deformities of the skull and spinal cord, such as in Down Syndrome, or build up of inflammatory tissue impinging on the brainstem, as in the pannus of RA.

A good introduction to the topic can be found on this ME-Pedia page: Craniocervical Instability

 

[Violeta]

Not sure what you mean, exactly. Is it a general question about the cause of craniocervical instability? If so, the answer is, no, it's not caused by brain swelling. I can think of a way they could be connected—an inflammatory process that causes brain swelling could also cause inflammation with subsequent degeneration of the ligaments at the base of the brain, namely, in the craniocervical junction.

Craniocervical instability is usually caused by ligamentous laxity at the junction of the brain and spinal cord, leading to hypermobility at the very complex craniocervical junction. This hypermobility causes the brainstem to be compressed or intermittently pinched by the top of the spinal column, the odontoid. Ligament laxity can result from traumatic disruption, as in a car accident, genetic connective tissue disease, like Ehlers Danlos Syndrome, or inflammatory processes like infections or autoimmune disorders. Other causes can include congenital deformities of the skull and spinal cord, such as in Down Syndrome, or build up of inflammatory tissue impinging on the brainstem, as in the pannus of RA.

A good introduction to the topic can be found on this ME-Pedia page: Craniocervical Instability

I was referring to this: "The cerebellum still pushing downward and forward because the skull is just not big enough for the brain." Is the brain swollen.

I should have been more specific.

 

[SWAlexander]

I have found many different answers. In my case it is congenital. My father and his father had Chiari.
I´m also have a diagnosis for myalgia and brain inflammation/CSF.
Some neuro-surgeons I have seen believe myalgia and brain inflamation is relatet to Chiari, two others say it is not.
I believe it is related to muscle weakness. I have leg and arm weakness since early childhood.
May I suggest reading: https://bobbyjonescsf.org/diseases/
and https://www.hopkinsmedicine.org/health/conditions-and-diseases/chiari-malformation-type-i

 

[minimalist]

Hi everyone!
I was diagnosed with CCI by Dr.Gilete.
I would like to have another opinion, I thought to do an online consultation with Dr.Henderson.
Has anyone managed to consult with him online? What is the procedure and how I can contact him?
Thanks.

 

[valentinelynx]

I was referring to this: "The cerebellum still pushing downward and forward because the skull is just not big enough for the brain." Is the brain swollen.

I should have been more specific.

Ah, OK. No, it's not because of brain swelling, but rather because, as the sentence says, "the skull is just not big enough for the brain". The definition of Chiari I (there are 4 types of Chiari malformations, Chiari I is the most common), according to the Mayo Clinic website is:

"Chiari malformation (kee-AH-ree mal-for-MAY-shun) is a condition in which brain tissue extends into the spinal canal. It occurs when part of the skull is misshapen or smaller than is typical, pressing on the brain and forcing it downward." [Emphasis mine]​

​

In the context of Dr. Bolognese's practice, however, it's useful to know that he and his colleagues have done research showing that there is a variant of Chiari associated with tethered cord syndrome (TCS), another spinal condition commonly associated with craniocervical instability, EDS, and, now it's becoming evident, ME/CFS. In this variant, the downward tension on the spinal cord caused by the abnormal filum terminale (a thin cord of tissue that anchors the end of the spinal cord to the coccyx) can cause the tonsils of the cerebellum to be pulled lower so that they enter the foramen magnum (hole at the base of the skull through which the spinal cord enters) or occlude it. In patients with TCS, the Chiari malformation doesn't appear to involve an abnormality of the size of the posterior skull, unlike traditional Chiari I. In this, it's more like Chiari II, which is a herniation of the cerebellum associated with spina bifida or myelomeningocele (congenital spinal cord abnormalities) in which a spinal cord abnormality in the lumbar region anchors the spinal cord, causing downward tension on the brain.

Here's a link to a paper on Chiari associated with TCS: Milhorat, T. H., Bolognese, P. A., Nishikawa, M., Francomano, C. A., McDonnell, N. B., Roonprapunt, C., & Kula, R. W. (2009). Association of Chiari malformation type I and tethered cord syndrome: preliminary results of sectioning filum terminale. Surgical neurology, 72(1), 20–35.

 

[Violeta]

Thank you, @valentinelynx, for the explanation.

 

[lenora]

Hello Everyone....I'm just about 75 and have Syringomyelia, Arnold Chiari Malformation, ME, FE and tethered cord syndrome....which should have been operated on many, many years ago but because of other problems, wasn't, I also took bromocriptine for a pituitary tumor for probably 35 years....I'm OK now, or I'm just fine as far as I'm concerned. My doctors feel the same way.

I don't think enough effort is put into those who have SM, Chiari or CCI, etc., because of accidents, birth difficulties...even mild ones. Plus more and more I see that we're returning to trying exercises instead of constant surgery. This is always something that you have to listen to your doctor talk about in a careful, concise manner. Other people have different reasons....please remember that. For some, surgery is a godsend, but it will always give you some problems to overcome. When we're younger it's easier to fight these things off.

Are you still going to have weak arms or hands? Legs that always feel strange (until my broken pelvis, apart from RLS I was fine...but change has occurred), neck pain (mine did go away), eye pain (went away and then reappeared many years later). PT really means committing yourself to some hard, painful work....will you do that? You should see some good results, but bear in mind that surgery is only the beginning.

A good surgeon won't be too quick to operate. As a matter of fact, you'll be sent away with a lot to think about, what he/she can do and what can be done for yourself. Just be realistic, that's all. Good luck and may better health be yours. It happens. Yours, Lenora.

 

[lenora]

Let me add that I did have early SM surgery and then ACM surgery which "cured" both the Chiari and SM problems....at least for a number of years. Confusing answer from me, Yours, Lenora

 

[SWAlexander]

Chiari malformation: several papers point to a connection with:
Ehlers-Danlos syndromes: https://pubmed.ncbi.nlm.nih.gov/28220607/
and
Tarlov cyst: https://operativeneurosurgery.com/doku.php?id=tarlov_cyst
Etiology:
"There is significant clinical evidence that collagen mutations or connective tissue disorders such as Marfan's, Ehlers-Danlos, Sjogren's, Loeys-Deitz, Lupus, MCTD (mixed connective tissue disorders), UCTD (undifferentiated connective tissue disorders) are predisposing or contributing to the cysts developing. A number of Tarlov cyst patients have also been diagnosed with a connective tissue disorder, and many more of their biological family members, who may or may not have Tarlov cysts, have been diagnosed with one of the above mentioned connective tissue disorders (CTD)."

 

[Husband of]

Does anyone know if jeff, Jennifer, or others who have had these issues and have successfully been treated had an acute onset with viral-like symptoms?

also, this symptom of a heavy head, surely everyone with moderate to severe me/cfs gets that? After standing or sitting upright for a little while you feel like you no longer have sufficient energy to hold your head up?

also, Jen apparently had a flare up after flying. My wife got me/cfs the day after flying. Did anyone else develop me/cfs within days of flying?

 

[valentinelynx]

Does anyone know if jeff, Jennifer, or others who have had these issues and have successfully been treated had an acute onset with viral-like symptoms?

Both @jeff_w and Jennifer Brea describe their illness onsets as occurring after viral illnesses. My onset was also sudden—I started feeling ill suddenly while on a hike. I thought I had some kind of flu, from which I didn't recover. I'm pretty sure that having a sudden onset of illness does not make CCI as a cause of ME/CFS symptoms any less likely. Infections are known to be one cause of damage to the craniocervical ligaments. The classic form of this is called "Grisel's Syndrome". It's most common in young children, typically caused by a pharyngeal strep infection.

Personally, I never had the "bobble head" syndrome, but I think it is more typical of CCI than just a heavy head caused by fatigue. "Bobble head" refers to the sensation that your head is too big or too heavy for your neck, like it's perched on a tiny support or a spring, like the bobble-head dolls.

 

[Violeta]

I still am thinking that sudden onset could be because of brain swelling, which can be caused by a virus. I would look into it before surgery if it started that way.

 

[lenora]

A cautionary tale from a 75 yr. old female. I had one of the first MRI"s in Dallas Country probably 40 yrs. ago. At that time, Chiari did show up as a problem and the machines were nothing like they are today. However, I want to add that I was fortunate....my neurologist and neurosurgeon both knew what to look for.

Prior to that I also had a pituitary tumor (controlled by meds for years...and then went away), plus another neurological illness that caused great distress, Syringomyelia (SM). SM and ACM are often cousins, although it's common to develop Chiari after say an accident (gymnastics or auto....anything involving the neck). Most are actually caused by accidents.

Those who suffer from scoliosis & co in their early years, especially of the l. side are more apt to develop neurological problems such as Chiari or SM. A yearly MRI of the type determined best for you will help track the progress of the illness.

I had two surgeries....one for the worst, SM, and it was highly unsuccessful....pain on top of pain, miserable pain. The ACM did contribute to pain also, but in all honesty even after surgery the biggest change was that my neck pain went away....at least to the level it was. Further deterioration was the main goal. Please don't think that surgery alone will cure you....many find themselves in even worse condition. I'm truly glad that both Jeff and Jen found themselves in better condition.

In my case, congenital problems were the reasons for my ongoing problems. One of the reasons folic acid is so important in early pregnancy (but go according to doctor's instructions).

It's good to finally have an explanation of why everyone seems to leave a Sleep Clinic with a tramadol prescription....not so much for sleep as for energy.

My surgeries did not help my lifelong sleep problems, mainly b/c the sleep center was already involved. I spend night after night awake, really don't let it bother me any longer and know that I won't die or even not function the next day. That helps. My optimum sleep hours are from 4-9 a.m., before that it's useless for me to even try, and even at that there are days/weeks when sleep simply doesn't come. Even my neurologist has more or less given up trying to help me...and I understand, boy do I ever.

I have a love affair with reading and I think that helps me not only pass time, but keeps me engaged in learning, peeking at how other's live, and so many things. My biggest enjoyment in life after my family. To the person who can only read for a short time, I would suggest that you keep at it....find an easier and perhaps more enjoyable book (books on tape, although I haven't tried them). Put something like Johnny Mathis (yes, there is such a person) on and learn what relaxation really is. Even if you extend it by a minute/day you've accomplished a good thing and given yourself satisfaction.

Don't expect to go to your normal level....I found that out about 2 yrs. ago and thought I was a failure. Well the failure was in not reading, and I upped my game. If I couldn't remember, I started over and it finally worked. I'd say that I'm back to almost back to where I was. Others may find other enjoyment.

After my brain surgery I could no longer remember musical tunes, games, puzzles so there have been losses that meant a lot to me. I was determined that reading wouldn't be one.

I understand that Jeff is now at The Chiari Institute so you may find help there. At least today we do have places located throughout the U.S. I can remember when UCLA, Denver and Chiari were the only places that could help. Now there are so many, although a lot of the people I knew have retired. All things change. Best of luck. Yours, Lenora.

 

[SWAlexander]

I had two surgeries....one for the worst, SM, and it was highly unsuccessful....pain on top of pain, miserable pain.

Lenora, reading your post again comparing your surgery and symptoms with mine, I wonder if my endocrinologist back in Jan 2019 was right. She believed I could have a CSF leak because of enormous headaches and clear fluid, tasting like metal, coming out of my ear and nose. At the time there was not a stand-up-MRI available in NM, nor any specialist who know how to search for leaks anywhere in the spine or diagnose a CSF leak.
Now I wonder if CSF is a part of CFS since the overwhelming number of symptoms, suffering from POTS, migraine, chronic pain and muscle weakness for years, fit exactly.
In my seemingly endless search for answers, I found confirmation and explanation in this presentation:

 

[lenora]

Hello @SWAlexander.....This is such a multi-faceted illness and yes, I do believe that things like CCI, Leaks, ACM & SM are yet to be discovered as a main cause for so many of our symptoms.

As you noted from the video even many "experts" miss many of the accompanying diagnoses...a reason I'm constantly advocating for people to have as many doctors, including radiologists, even Physicans' Assistants look at the "pictures" (as they're often referred to). In my case, all of my problems were picked up by 3 different doctors...all experts. Amazing, isn't it? I don't blame them....am actually grateful for being told I didn't need surgery in many cases and confusion when I received two differing opinions for the same problem. Today this may even be more likely b/c of the proliferation of specialists and equipment itself.

Listen carefully to why a specialist feels you shouldn't have surgery....it's important. Are you going to be left with a problem that's even worse? Often these doctors appear as quite brusque, but I've found over the course of many years that some people actively seek out surgery as the answer when it always isn't. This is difficult to deal with and we have to learn as much about possible problems as we can.

I always look to the future (although at 75 it's not long for me). What is going to be discovered? What is going to be repaired by surgery, or definitely not known ? Parents will want surgery because their children are suffering (and who can't understand that). Doctors are perplexed even though they're top specialists, it's a terrible situation to be in. Always be interested for others needing help will follow you.

A tip: I have found over the years that if I take the first appt. in the a.m., I'm the first seen. Now it's possible that the appointment is at 6:30 a.m. and I'm not seen until 10:00 a.m. (b/c of an emergency). Take snacks, water in bottles, something to read and the time soon passes....no matter how many chairs there are, people are left standing and at least I'm not dealing with sore feet and a sore back. Have someone accompany you...someone who will listen and make note of what's being said.
What does one do about a doctor you just can't get along with...and yes, there are some. Find another specialist is all that I can recommend.
I'm about 3/4 of the way through the video, will definitely finish it because change is the one constant in this field. I know that dealing with my anxiety helped me tremendously. If you'd asked me if I was anxious, I would have replied "No"....but the odd thing is that I was being hindered by it and many of us do suffer from it.

Yes, there are plenty of horror stories about coming off meds, but how many do you read about people who were being helped? No one can tell you what you should/should not do with respect to your health. You're just on an information gathering mission.
Better health. Yours, Lenora.

 

[GlassCannonLife]

Didn't Jen Brea get worse again (was it after covid.?) despite having her ME put fully in remission by CCI and tethered cord surgeries?

 

[valentinelynx]

Didn't Jen Brea get worse again (was it after covid.?) despite having her ME put fully in remission by CCI and tethered cord surgeries?

I have no personal connection to Jennifer Brea, so I can only talk about what I've read of her accounts. From what I recall, she had COVID-19 and that knocked her back pretty badly. Before that, she had some additional issues that needed correcting, such as jugular vein stenosis caused by the omohyoid muscle, and spontaneous CSF leaks in the lower spine. These weren't caused by the CC fusion, but rather seem to be additional consequences of her underlying illness. The CSF leaks are intriguing because they suggest a connective tissue disorder, although she didn't have other symptoms of EDS. It appears that some people with ME/CFS may have an acquired CT disorder, perhaps triggered by an infection that leads to connective tissue degradation. Some speculate about metalloproteinases that degrade collagen and are increased by some kinds of inflammation.

I don't know anything about her current condition, health-wise. From her Twitter posts, she got COVID in April 2020, followed by "long-COVID" and in December 2021 said in a post that she had a full recovery after 6-9 months.