CCI SURVEY – for all ME/CFS patients tested for CCI and related conditions (please answer whether tested positive or negative)

[Hip]

Craniocervical Instability, Chiari and Spinal Stenosis Survey

For ME/CFS patients diagnosed positive or negative for CCI and related conditions

This survey is for ME/CFS patients who sent their MRI or CT scan to Dr Gilete, Dr Bolognese, Dr Henderson, Prof Smith or another neurosurgeon and received a positive or negative diagnosis for craniocervical instability (CCI), Chiari, cervical spinal stenosis or related conditions.


➤ Click HERE to go to the survey (it employs Google Forms, and is anonymous)


Please fill out this survey whether you were tested positive or negative for CCI and related conditions (it is very important that people who tested negative for CCI and related conditions also answer this survey if they kindly would, so that we can see the difference in characteristics between ME/CFS patients who are positive for CCI, and ME/CFS patients who are negative).

The purpose of this survey is to examine the link between CCI and ME/CFS, by looking at the patterns of symptoms, infections, co-morbid diseases, craniocervical measurements, etc in ME/CFS patients diagnosed positive as well as negative for CCI.

The survey is intended to answer many questions, such as:

• The difference in symptom profile between CCI ME/CFS patients vs non-CCI ME/CFS patients, and whether CCI patients have for example more dysautonomic symptoms like POTS
• Whether CCI ME/CFS patients typically have the cervical medullary syndrome (CMS) symptoms caused by CCI, such as severe headache, neck pain, "bobble head", etc
• The type of CCI ME/CFS patients typically have (there are different forms of CCI)
• Whether CCI ME/CFS patients previously suffered a major physical trauma to their head or neck at some point in their life
• Whether CCI ME/CFS patients often have conditions such as hypermobile Ehlers-Danlos syndrome or rheumatoid arthritis, which are known to predispose to CCI
• Whether CCI ME/CFS patients had sudden or gradual onset of ME/CFS, and whether they had a viral or non-viral trigger to their ME/CFS
• Whether CCI ME/CFS patients typically have chronic active viral or bacterial infections
• The outcome of surgery, prolotherapy or stem cell therapy for CCI, for those who had these treatments

If you have any difficulties in filling out this survey and would like assistance or to ask a question, please post your question in this thread.

If there is wording in the survey that you find unclear, please point it out in this thread, so that the wording can be improved.

If you know of any ME/CFS patient who was tested for CCI, but does not come to these forums much, I'd be most grateful if you could draw their attention to this survey.

You need a Google account to answer this survey. Google ensures your identity remains anonymous. If you do not have a Google account, you can easily set one up here: accounts.google.com/signup

Using a Google account has the advantage that because your answers are linked to your account, you can come back to the survey at any time in the future to update or add to your answer responses — useful if you have new info to add, such as the results of surgery, prolotherapy or stem cell therapy if you opt for these. (If you return to the survey to update your answers, you will see a message saying "You've already responded", but underneath that message, if you click on "Edit your response" you will be able to amend your answers).

Prof Ron Davis and Dr William Weir are interested in the CCI connection to ME/CFS, so they may like to see the survey results.

Thanks to @JenB, @jeff_w, @bombsh3ll, @Silencio, @valentinelynx, @RiverJewel and others for their help and advice in creating this survey questionnaire.

@mattie also set up a very simple Phoenix Rising forum poll here, which those tested for CCI may want to answer as well.


Note: do not give anyone else the URL link to your personal survey answers. Everyone has to log into the survey via their Google account, and then this directs you to a special URL for the survey which is uniquely yours. If you share that unique URL with others (which you should not do), then other people can change your answers.




View Survey Results

The summarized results in graph form HERE.

Note: in these graph form results, in some questions, the percentages in the pie charts are not really correct, because unfortunately not everyone who took the survey responded to every question. So the pie chart percentages are based on the number of people who answered each question, not the total number of people answering the survey. In some cases however this is appropriate (for example, in the questions about the CCI diagnosis rate of each neurosurgeon, we want only those who sent their scans to a particular neurosurgeon to respond to the question). But in general, to calculate the percentages accurately, based on the total number of people answering the survey, it is better to use the data in the spreadsheet, which is found here:

Summary page in spreadsheet format HERE (recommended).

Responses (raw data) in spreadsheet format here.

You can flip between these two spreadsheet pages by clicking the Responses and Summary tabs at the bottom of the spreadsheet page.

Some Results From the Survey

Some findings from the survey, as the results stand on 23 November 2019. These results are derived from the spreadsheet data.

• 91% of patients tested positive for at least one diagnosis, out of CCI, AAI, Chiari, cervical spinal stenosis, subaxial instability, tethered cord, syringomyelia, cerebrospinal fluid leak, intracranial hypertension and intracranial hypotension.

• 79% of patients tested were positive for craniocervical instability (CCI).

• In those positive for CCI, around 70% also had atlantoaxial instability (AAI). It was rare to have AAI on its own without CCI.

• In those positive for CCI, 48% had their ME/CFS start after an infectious episode (such as a flu-like or gastrointestinal illness, or mononucleosis). Out of these infectious onsets, 57% had a rapid onset (ME/CFS appeared within days of the infection), and 29% had a gradual onset (they slowly descended into ME/CFS over a period of a few months after the infection).

• Physical trauma was the trigger of ME/CFS in only 9% of those positive for CCI. However, around 59% of these CCI positives reported they experienced a major trauma to the head or neck, or a serious motor vehicle accident, previously in their lives.

• About 27% of those positive for CCI were diagnosed with hypermobile Ehlers-Danlos syndrome (hEDS). And another 27% report that they suspect they have hEDS, but are not formally diagnosed.

• The average number of cervical medullary syndrome (CMS) symptoms for those positive for CCI is around 17 out 32. This suggests most ME/CFS patients with CCI will manifest many CMS symptoms as well as their ME/CFS symptoms. About 84% of those positive for CCI have 12 or more CMS symptoms.

• Of those positive for CCI, a pathological translational BAI is found in around 46%, a pathological CXA is found in around 38%, a pathological retroflexed odontoid is found in around 21%, a pathological Grabb-Oakes is found in 16%, a pathological odontoid over Chamberlain's line in 16%, and a pathological BAI is found in around 9%.

• Chiari was found in 12% of patients answering the survey.

• For those positive for CCI, there were a lot of severe or very severe ME/CFS patients: 30% were severe, and 20% very severe.

• In those positive for CCI, 36% experience transient paralysis in certain head/neck positions or worsening symptoms in certain head/neck positions. For those with such head/neck position sensitivity, there was a correlation with having a retroflexed odontoid (RO), or an odontoid 3 mm or more over Chamberlain's line (OOC). In those with head/neck position sensitivity, 56% had RO and 31% had OOC; whereas in those without such sensitivity, 7% had RO and 7% had OOC. So it may be the odontoid impinging on the brainstem that causes the transient paralysis and worsening of symptoms in certain head orientations.




This survey is not able to determine the percentage of ME/CFS patients who have CCI or related conditions, for two reasons:

Firstly, people getting tested for CCI are self-selecting, and most likely decide to get tested because they have cervical medullary syndrome symptoms such as neck pain, severe headaches, etc, or they have previously had a neck injury, or they notice that their symptoms get worse in certain head and neck orientations. These are all suggestive of CCI. So those getting tested will likely have a higher prevalence of CCI than the general ME/CFS population.

Secondly, those who test negative for CCI and related conditions tend to lose all interest in the subject, and thus do not answer to the survey. Thus there is bias of towards only CCI-positive patients answering the survey.

 

[Joly]

I was diagnosed with hindbrain herniation that I was told was specifically not Chiari... how should I answer? It is below 5mm, so would that count as a 'no'?

 

[Hip]

I was diagnosed with hindbrain herniation that I was told was specifically not Chiari... how should I answer? It is below 5mm, so would that count as a 'no'?

Yes, you can answer "No" for that one, if your protrusion (ectopia) is less than 5 mm. The threshold for a Chiari diagnosis is 5 mm or more.

Hindbrain herniation is just another name for Chiari.

 

[Joly]

Yes, you can answer "No" for that one, if your protrusion (ectopia) is less than 5 mm.

Hindbrain herniation is just another name for Chiari.

Thanks! I'll put No. As for the classification, my radiologist report from Prof. Smith stated:

"There is a minor degree of hindbrain herniation, where the cerebellar tonsils extend into the foramen magnum posterolaterally in a pattern characteristically seen in an individual suffering from one of the hypermobile syndromes. The appearances are not those of a classical Chiari malformation, but are typical of the hindbrain herniation seen in patients with the hypermobile Ehlers-Danlos syndrome and which was formerly, erroneously called Chiari malformation, which is a different condition."

But I haven't been able to find any further information on this.

 

[Hip]

@Joly, thank you very much for filling out the survey. You are the very first person to respond!

Just a quick question for you: I noticed in the headache question of the survey that you said you experienced every type of headache, out of the list of:

• Tension headaches = mild to moderate pain on both sides of the head, sometimes involving pain or pressure behind the eyes, which last from 30 minutes to a few days.
• Migraine headaches = moderate to severe throbbing pain that lasts for a few hours to several days, normally on one side of the head only, and possibly also behind one eye only.
• Cluster headaches = severe burning or piercing pain behind the eyes or temples on one or both sides, lasting up to 3 hours; cluster headaches can appear once daily, or recur several times on the same day.
• Occipital headache = severe jabbing pain in the back of the head and neck, which may sometimes spread behind the eyes and forehead, lasting for hours to several days.
• Coat-hanger headache = pain in the head along with a tight sore trapezius shoulder muscle on both sides, or on one side only.
• Sinus headaches = deep and constant pain in your cheekbone, forehead or bridge of your nose which often lasts days.
• Other type of headache

Is it really the case that you get all these different forms of headache?

I know the headache question is a little complicated. I originally I did not have all these different types of headache in the question, but it was pointed out that a regular tension headache (which you can get rid of with an aspirin) is really no comparison to the severe types of headaches that are often found in CCI, Chiari, etc.

So that's why I specifically included in the question the more severe types of headache like migraine, cluster headaches and occipital headaches.

But I am not entirely happy with this headache question, because it is a bit complicated.

 

[Joly]

Is it really the case that you get all these different forms of headache?

Unfortunately yes, it is true! For 'other' I also get ice pick headaches which are not fun at all.

 

[toyfoof]

@Hip I'm waiting on the radiologist's report from my upright MRI last week, and then I'll be sending it on to a couple neurosurgeons for official diagnosis, so I'll wait to fill out your survey till I have those results.

Thanks for putting this together, I know it was a lot of work. I hope you get a lot of responses.

 

[valentinelynx]

A couple of comments...
• Regarding traction the questions ask if your symptoms feel better "under traction". In my experience, I feel better after traction. During it, I'm not moving so don't notice much, but afterward I feel good in various ways.
• A problem I have with many such questionnaires: my symptoms have changed over the 26 years of my illness. I had recurrent sore throats in the beginning but no more; I used to have frequent headaches of various types, but rarely do now; I used to have a lot of neck pain, but rarely do now, etc. I answered the questions with a "Yes" if I had ever experiences the symptom in question.
• Perhaps there should be an option for "other" infections? For example, I likely had both Lyme disease and bartonellosis at the time I became ill.

Thank you for assembling this. You put a lot of work into it! I hope you get enough respondents to show interesting results.

 

[Hip]

• Regarding traction the questions ask if your symptoms feel better "under traction". In my experience, I feel better after traction. During it, I'm not moving so don't notice much, but afterward I feel good in various ways.

Does that feeling good period occur immediately after traction for just a few hours, or does the feeling good period last a day or more?

• A problem I have with many such questionnaires: my symptoms have changed over the 26 years of my illness. I had recurrent sore throats in the beginning but no more; I used to have frequent headaches of various types, but rarely do now; I used to have a lot of neck pain, but rarely do now, etc. I answered the questions with a "Yes" if I had ever experiences the symptom in question.

I think that makes sense to answer "Yes" if you have ever had an ME/CFS symptom at any stage in the course of your ME/CFS.

• Perhaps there should be an option for "other" infections? For example, I likely had both Lyme disease and bartonellosis at the time I became ill.

That's a good idea. OK, I've added an extra question to the survey entitled: "Were you tested positive for co-infection with Bartonella, Babesia, Brucella or Borrelia?"

If you have already responded to the survey, you can go back to the survey and answer this extra question, not forgetting to press the SUBMIT button at the bottom of the page.

Unfortunately the issue of infection testing in ME/CFS is fraught with difficulties. For example we know from Dr Chia's work that most antibody testing techniques are too insensitive to detect chronic enterovirus in ME/CFS; it is only antibody tests using the neutralization method (like the ARUP Lab enterovirus antibody tests) which are sufficiently sensitive. So a lot of people may think they are negative for enterovirus, but may just have taken an insensitive test.

 

[Mere72]

@Hip I'm wondering about capturing Dr Bolognese's reviews. If people get turned down by the practice, it doesn't necessarily mean they are not pathological. Maybe under B we should add something along the lines of "this does not include non-acceptance into his practice, if you were not given a reason for denial." Maybe you've got this bias captured elsewhere, but wanted to bring it to your attention.

Secondly, want to confirm, b/c I'm sure it will be asked... Are you including general radiology reports that indicate pathological measurements as diagnostic by "any medical professional"? I'm assuming not, as it's not technically a diagnosis, rather a finding, but want to be sure.

 

[Hip]

@Hip I'm wondering about capturing Dr Bolognese's reviews. If people get turned down by the practice, it doesn't necessarily mean they are not pathological. Maybe under B we should add something along the lines of "this does not include non-acceptance into his practice, if you were not given a reason for denial." Maybe you've got this bias captured elsewhere, but wanted to bring it to your attention.

Yes you bring up a tricky issue that does not have an easy solution, as far as I can see.

For simplicity, in the survey I just stated that if any neurosurgeon tells you that "you do not meet our parameters for CCI" (which is the sort of phrase Dr B uses) and you are rejected as a patient, then to count that as a negative diagnosis. But in the case of Dr B, people may still be positive for CCI even though they are rejected by his patient intake criteria.

Because these neurosurgeons are overloaded with patients, I am guessing that Dr B want to ensure that the patients he does take on are very clearcut cases, because I imagine he will be able to do more for these patients via surgery.

So with Dr B, when we interpret the survey results, we have to bear in mind that if you got a positive diagnosis from him, that's correct; but a negative diagnosis does not rule out the possibility you may have CCI.

Because of the recent surge in ME/CFS patients sending their MRI or CT scans to these CCI neurosurgeons, I understand Dr B is now asking patients to get a neck traction test from a physical therapist, and he will now only examine your scans if your neck traction test was positive (ie, if your symptoms got much better during neck traction).

Are you including general radiology reports that indicate pathological measurements as diagnostic by "any medical professional"?

I am glad you brought that up. No, for the survey, it only counts if a CCI expert neurosurgeon (like Dr G, Dr B and Dr H) or CCI expert such as Prof Francis Smith provides you with either a positive or negative diagnosis for CCI. We only want to record diagnoses from these CCI experts.

I've just changed the wording in the questionnaire to reflect that: it now says "This section asks if you were given a positive or negative diagnosis for craniocervical instability (CCI) after sending your MRI or CT scans to one or more CCI neurosurgeons or CCI experts."

 

[Sushi]

Because these neurosurgeons are overloaded with patients, I am guessing that Dr B want to ensure that the patients he does take on are very clearcut cases, because I imagine he will be able to do more for these patients via surgery.

Wouldn’t there also be the possibility of one of these neurosurgeons not accepting a patient due to them being too high a risk for surgery?

 

[Mere72]

@Hip Thanks for clarifying.
Along those lines, you might consider verbage in the section "Were you diagnosed with craniocervical instability (CCI) by any medical professional?"
Followed by "If ANY medical professional gave you a positive diagnosis for CCI, please indicate that positive diagnosis here. In this question, only answer "Diagnosed negative" if ALL the medical professionals who viewed your scans gave a negative diagnosis for CCI."

 

[Mere72]

Wouldn’t there also be the possibility of one of these neurosurgeons not accepting a patient due to them being too high a risk for surgery?

I don't think we fully know what Dr B is currently basing his decisions on. I think he has been inundated with cases, and has to find a way to narrow down who he accepts. I believe he is considering how much function is affected by pathologies. Call that the "more serious" cases, or the cases with lower Kornafsky scores. He is also looking at traction outcomes, and severity of imaging, applying his prioritized measurements and diagnostic methods. I would venture to guess that he is taking the more serious cases, as opposed to those who are at too high a risk for surgery. But I don't know if we can say with certainty. But it is worth noting that he doesn't indicate whether or not you are surgical/ pathological when he rejects patients. Second opinions are important!

 

[Hip]

Wouldn’t there also be the possibility of one of these neurosurgeons not accepting a patient due to them being too high a risk for surgery?

That may be the case, but I would not have thought it would affect the actual diagnosis given.

Incidentally I've heard that Dr B will go out on a limb to help and take on the more extreme and risky cases that other CCI neurosurgeons may not want to deal with. So in the case of Dr B, I don't think he is rejecting patients because of high surgical risk. But since I suspect he has more applications that he can slot in to his surgery schedule, I imagine his may be very sensibly selecting the patients who he can help the most, for whom surgery will likely make the greatest improvement.

Along those lines, you might consider verbage in the section "Were you diagnosed with craniocervical instability (CCI) by any medical professional?"

I've just now removed that question entirely, because I noticed some people were getting confused by it, and answering it wrongly.

So now instead of that question, I've added a new question at the end of the CCI section entitled "Did any other CCI expert diagnose CCI?"

Thanks very much for your suggestions, @Mere72, they are appreciated.

 

[Mere72]

Thanks very much for your suggestions, @Mere72, they are appreciated.

My pleasure. I feel strongly about getting the most accurate data we can. And I've noticed trends in the questions people ask with the polls in the CCI Facebook group (BTW I am the same person asking you questions about this in that group).

 

[valentinelynx]

Does that feeling good period occur immediately after traction for just a few hours, or does the feeling good period last a day or more?

The benefits occur immediately after the traction and last probably an hour at most.

That's a good idea. OK, I've added an extra question to the survey entitled: "Were you tested positive for co-infection with Bartonella, Babesia, Brucella or Borrelia?"

Thank you for adding this question. However, I'm afraid I disagree with the notion that the CDC 2-tier test for borrelia is more reliable than IgeneX or Armin labs. The CDC's 2-tier test is notoriously unreliable (insensitive), for one because it requires the use of an ELISA test first, which has a lower sensitivity than the Western blot. Testing for borrelia is difficult, and the recommendation of many experts, including Columbia University's Lyme and Tick Borne Diseases Research Center is that Lyme should be diagnosed with clinical criteria rather than laboratory testing. Bartonella is also vey difficult to find on laboratory testing, and those with a high clinical suspicion for this infection would probably be best served by testing through Galaxy Diagnostics. The unfortunate politicization of the science of tick-borne diseases has done a great disservice to patients (quite similar to that regarding ME/CFS). There is bad information available from multiple sources with different points of view and one really has to read the scientific publications with a highly critical eye.

 

[Mere72]

Because of the recent surge in ME/CFS patients sending their MRI or CT scans to these CCI neurosurgeons, I understand Dr B is now asking patients to get a neck traction test from a physical therapist, and he will now only examine your scans if your neck traction test was positive (ie, if your symptoms got much better during neck traction).

I have just been accepted into Dr B's practice without first doing traction. But I had numerous pathologies indicated on my general radiology report from my imaging, and I explained to him that b/c of that I was hesitant to try traction before having my case reviewed by a specialist, but would try it if he found it necessary after reviewing my case. I also explained that I have responded poorly (become worse) with each of many trials of PT, manual massage, or other modalities, thus my caution. He has now requested traction though, prior to appointment.

Also, Dr B has said that improvements from traction indicate pathology, but he has also said a negative reaction to traction could also indicate a more severe pathology. So I think Dr B looks at the full picture, but has had to become more selective with various parameters to accommodate so many requests. I do think including traction with your initial request might improve your chances of getting accepted.

 

[Hip]

However, I'm afraid I disagree with the notion that the CDC 2-tier test for borrelia is more reliable than IgeneX or Armin labs. The CDC's 2-tier test is notoriously unreliable (insensitive), for one because it requires the use of an ELISA test first, which has a lower sensitivity than the Western blot.

I appreciate the issues with insensitivity with the CDC testing protocol. But I think you would agree that if you test positive with the "conservative" CDC protocol, then you can be fairly certain you have Borrelia. Whereas if you test positive with ArminLabs or IGeneX, then you may or may not have Borrelia. Threads talking about the high false positive rate of these two labs here and here.

So for research purposes when selecting a study cohort, if you wanted to make sure someone definitely does have Borrelia, the CDC protocol would seem to be the right test. But if you are an individual patient wondering whether you might have Borrelia or not, the CDC protocol is not ideal.

Generally, I don't think the survey questions asking about chronic active infections are likely to get accurate results, because the various different ME/CFS doctors use different labs and different criteria for determining active infection. Stanford for example use a coxsackievirus B test that is insensitive for chronic infection. And Stanford use different criteria for active herpesvirus infection than the criteria Dr Lerner used to use.

So in terms of figuring out whether patients in this survey have infectious involvement, I think it will be more reliable to look at whether their ME/CFS onset involved an acute infectious episode (which is one of the questions of the survey).

 

[valentinelynx]

But I think you would agree that if you test positive with the "conservative" CDC protocol, then you can be fairly certain you have Borrelia.

Sure, I'd agree with that: this would catch a small percentage of those will those infections. Even the rigid "Lyme is easy to diagnose and treat" people know that borrelia cannot be found on antibody tests in all patients. For example, early exposure to antibiotics leads to negative tests. That's why a clinical diagnosis is recommended rather than a test-based diagnosis. Also, I'm not well versed regarding Armin labs, but I would believe IgeneX results. I don't have the energy to dive into the arguments, but know that the naysayers are using old results. Even New York State now accepts IgeneX lab testing.

It's a good point regarding the active infection issue. I suspected I had some sort of infection at first, but no testing, and testing after 20 years is worthless, so? Those who had a high fever at onset have good reason to suspect infection, but there are many non-infectious causes of fever as well.