Tracking CCI / AAI MRI & Treatment outcomes

[MEPatient345]

I didn't actually think there was anything wrong with my CXA but note it is 129 in flexion. Does the above refer to the neutral position only?

Yes, 135 and lower refers to neutral. Jen gathered some info on mepedia:
https://www.me-pedia.org/wiki/Craniocervical_instability#Measurements

For ME/CFS patients who have sent their MRI scans to neurosurgeons such as Dr Henderson, Dr Bolognese or Dr Gilete, and received back a diagnosis and report, could you please detail what is in that report?

My report from Dr Gilete included a summary of my symptoms questionnaire, and was as follows:

The tip of the odontoid peg lies below level of Chamberlain's line.
Cerebellar tonsils ectopia: 4 mm.

Craniocervical Junction Measurements:
CXA Neutral 139° / Flexion 125 / Extension 158
BAI 1,5 / Flexion 8.6
Grabb 7,24 / Flexion 11.4 / Extension 4.8
Translational BAI: 8,6 mm
C1-C2 neutral misalignment.
Rotary AAI looking left and right.

Cervical Subaxial:
Signs of subaxial instability at:
C2 to C6.
Cinerradiology will be recommended to finally assess subaxial instability.

Impression
After reviewing Miss. X’s case, though further investigation is needed (see below) and
although surgery is the last option to be considered, we think that she could be candidate for:
Occipitocervical posterior fusion stabilization with intraoperative reduction (traction).
Number of levels: depending on DMX or cineradiology once subaxial instability evaluated. Probably C0 to T1.

Diagnoses
Principal diagnoses related to current situation:
1-Craniocervical Instability (CCI)
2-Atlantoaxial Instability (AAI)
3-Subaxial Instability probably up to C6-C7 (to be confirmed by cinerradiology)
4-Cerebellar tonsils descent

 

[Hip]

Thank you very much, @bombsh3ll and @Silencio, for posting the details of your reports from Dr Gilete and Dr Bolognese, it's very useful for me to see, to help organize the questionnaire I am working on.

I didn't actually think there was anything wrong with my CXA but note it is 129 in flexion. Does the above refer to the neutral position only?

I am not entirely sure; I've assumed the pathological reference range for these measurements refer to the neutral head position (except in the case of the translational BAI, where it is the change in the BAI from the flexion to extension head positions that you measure). But in this 2019 paper by Dr Henderson says:

The CXA measurements were taken from the flexion image, when it was available.

Henderson also mentions the CXA in this video at 8:17, but does not indicate whether the angle is to be measured in neutral or flexion positions.

Yes, 135 and lower refers to neutral. Jen gathered some info on mepedia:
https://www.me-pedia.org/wiki/Craniocervical_instability#Measurements

Are you certain of this, because I could not find any sources which explicitly say that it is in the neutral position that these reference ranges for the various measurements apply. Although Dr Bolognese mainly uses supine MRIs for his diagnosis without flexion and extension views, so in his case it's only the neutral position he examines (however he says he does sometimes order upright MRIs with flexion and extension views).



Here are the reference ranges for the various measurements:

Sources: here and here.

Clivo-axial angle (CXA)
An angle of 145º to 160º is normal. Borderline is 136º to 144º. An angle of 135º or less is considered pathological and indicates a possible mechanical deformity of the brainstem and upper spinal cord. Source: Dr Bolognese in this video at 14:28.

Grabb-Oakes (B-pC2)
Less that 6 mm is normal (see this video at 49:30). A distance of 9 mm or greater is pathological and indicates brainstem compression. (Some use 8.5 mm as the threshold for pathological — see this video at 49:30)

Basion-axis interval (BAI)
Greater than 12 mm is pathological and indicates craniocervical instability (see this video at 10:34, and the Harris study).

Basion-dens interval (BDI)
Greater than 12 mm is pathological and indicates craniocervical instability (see this video at 10:34, and the Harris study).

Translational BAI
Less than 1 mm change between flexion and extension is normal; greater than 2 mm is pathological and indicates craniocervical instability. Dr Henderson stipulates the translational BAI must be greater than 4 mm in order to be a candidate for fusion surgery.

Chamberlain's line
If the tip of the dens (also called the odontoid peg) is more than 3 mm above Chamberlain's line, it indicates basilar invagination (vertical craniocervical instability), causing brainstem compression as the dens impinges on the brainstem or upper cervical spine. Source: here. See also this video of Dr Gilete at 1:16.

Chiari malformation
Chiari I malformation may be diagnosed if the cerebellar tonsils protrude more than 3 to 5 mm below the opening at the bottom of the skull (the foramen magnum). Source: here. In one study, all patients with greater than 12 mm of protrusion were symptomatic for Chiari, whereas approximately 30% of those whose protrusion measured between 5 and 10 mm remained without symptoms. Source: here.



Dr Paolo Bolognese in this 2018 video at 54:46 says CXA and Grabb-Oakes are more sensitive to horizontal craniocervical instability, whereas the basion-dens interval (BDI) is more sensitive to vertical craniocervical instability.

Dr Bolognese in the 2018 video at 1:12:44 says some patients have pathological measurements indicating CCI, but show no symptoms. No surgery is advised for these patients.

Regarding the CXA and Grabb-Oakes: in the case when one is positive and the other negative, Dr Bolognese and Dr Henderson have different opinions as to which takes precedence. Henderson thinks the CXA should take precedence, Bolognese thinks the Grabb-Oakes should take precedence. See the 2018 video at 41:48.



@bombsh3ll, am I right in thinking that both Dr Gilete and Dr Bolognese found that you were negative for CCI?

@Silencio, not that I know much about this, but it looks like it might be your translational BAI of 8.6 mm that resulted in your diagnosis of CCI, as this is higher than the > 2 mm reference range threshold.

 

[User891]

No doubt you have seen this, but Dr Gilete discusses craniocervical instability in this video and mentions the CXA at around 6:30

I am not at home and cannot access the full report (password protected) but I recall my CXA was
128° neutral, 126° flexion, 133° extension, and that the tip of the odontoid peg was above the level of Chamberlain's line.

Is anyone aware of what Dr Gilete has been recommending prior to 'last option' surgery, if anything?

 

[MEPatient345]

Are you certain of this, because I could not find any sources which explicitly say that it is in the neutral position that these reference ranges for the various measurements apply.

No, I thought I had seen it in the Henderson paper you posted.. am having quite a hard time keeping track of info w brain fog. It’s v overwhelming.. thanks for any additional sense you can make of it.

@Silencio, not that I know much about this, but it looks like it might be your translational BAI of 8.6 mm that resulted in your diagnosis of CCI, as this is higher than the 2 mm reference range threshold.

Indeed.. this is what the EDS patients are telling me.. have had a number of remarks about my BAI on Facebook! although I think the doctor also will make a call based on entire Measurements picture and symptoms, and in Bolognese’s case — response to traction.

 

[bombsh3ll]

Clivo-axial angle (CXA)
Normal is around 155º, whereas 135º or less is considered pathological and indicates a possible mechanical deformity of the brainstem and upper spinal cord.

Mine was 135 neutral, 129 flexion so probably not great anyway if normal is 155.

Translational BAI
Less than 1 mm change between flexion and extension is normal; greater than 2 mm is pathological and indicates craniocervical instability.

Mine was 2.6mm

Basion-dens interval (BDI)
Greater than 12 mm is pathological.

Mine was not measurable - I don't know if there is a lower cut off for this or not, but Dr G felt it indicated cranial settling.

@bombsh3ll, am I right in thinking that both Dr Gilete and Dr Bolognese found that you were negative for CCI?

No, this is what bothers me. I got the report above from Dr Gilete then went to Barcelona and had DMX which he said also shows cranial settling. The instability in c2-4 turned out to be very minor on the DMX & only relevant in the context of increasing risk of adjacent level syndrome if fused C0-2, not something that would ever cause a problem in its own right.

Dr B said I had no instability, BUT he mentions no measurements and advised me to get checked for CSF leak, which WAS the third scan I sent (a whole brain & spine MRI with gadolinium contrast, which was done for that purpose & showed no sign of a leak), so I am not convinced he had a very detailed look & also didn't see the DMX which was done in Spain.

I would have felt better about it if both opinions had concurred though.

B xxx

 

[Hip]

@bombsh3ll, looks like your CXA and translational BAI measurements are on the borderline of pathological, rather than well into the pathological range.

Also, your 40% score on the Brainstem Disability Index (which is a questionnaire-based score) I am guessing is not particularly high (although I am not certain of this), so perhaps that might have been a factor in diagnosis. I believe these neurosurgeons take into account whether or not you have the symptoms of CCI before they give a positive diagnosis (or at least before they recommend surgery).

Your report from Dr Gilete said:

The tip of the odontoid peg lies at the level of Chamberlain's line and BDI could not be realistically measured so it can be a cranial settling sign.

So it seems he is suggesting cranial settling might be a possibility, but he does not make a definitive statement about that.

Is anyone aware of what Dr Gilete has been recommending prior to 'last option' surgery, if anything?

Note sure about Dr Gilete, but Dr Henderson in this paper says non-surgical conservative treatment includes physical therapy, activity modification, pain medications, neck brace, and in some circumstances, chiropractic, electrical stimulation, massage.




Does anyone know whether the upper neck MRI used by these neurosurgeons for detecting CCI is also sufficient to detect:

Chiari malformation
cervical spinal stenosis
tethered cord
syringomyelia

In other words, if these conditions were present in the upper spine, would Drs Henderson, Bolognese and Gilete see them in the MRI scan, and would they notify you that you have them? All the above I believe can cause ME/CFS-like symptoms.

Looks like from Dr Gilete report to @bombsh3ll posted earlier you need rotational MRI images to detect atlantoaxial instability (AAI):

We cannot evaluate possible AAI due to the fact that the the MRI does not contain any rotational head-neck images.

 

[Capecodder]

I have been tested. But it was only read by local Neuro/radiologist. Nothing noteworthy but bulging discs in entire neck, and narrowing of exit spaces.

Was recently diagnosed with EDS 3. Here is my history:
1. 2012, injured SI joint walking downstairs. Was athlete/distance runner.
2. 3 months later, still in pain. See Chiro who does full body high velocity adjustment (didn’t know I had EDS). Entire back goes into spasm, feel like I am in straight jacket. Lose mobility in neck, never had neck problems previously.
3. A few weeks later, my CRP is high, positive ANA and RNP, diagnosed with UCTD. Given steroids. Help a little with pain, but not significantly. Develop excercise intolerance, diagnosed with ME/CFS and Fibro.
4. Develop muscle spasms in traps, rhomboids that do not respond to manual treatments. Can’t stand for more than a few mins. Or walk distances without back spasming.
5. Develop headaches/migraines in 2016
6. 2017, have Botox for migraines. It leaks. Weakened muscles, severe pain in neck after holding it in extension for period of time. Traction worsened it. Developed OI.
7. Develop Atypical Trigiminal Neuralgia 6 months later..
8. Recently used Hard cervical collar and worsened sitch. Instead of back of neck hurting, now my scalenes and pecs are spasming.
9. Still have lower back pain.

Should I have my MRI read by Dr. Henderson? Does he do consults? How to differentiate CCI from ligament instability?
Next steps? Should I consider prolotherapy? Thanks in advance!

 

[MEPatient345]

didn't see the DMX which was done in Spain.

Is it not possible to get the DMX on a disk drive or CD to send to Dr. B in NY?

 

[nandixon]

Does anyone know whether the upper neck MRI used by these neurosurgeons for detecting CCI is also sufficient to detect:

Chiari malformation
cervical spinal stenosis
tethered cord
syringomyelia

In other words, if these conditions were present in the upper spine, would Drs Henderson, Bolognese and Gilete see them in the MRI scan, and would they notify you that you have them? All the above I believe can cause ME/CFS-like symptoms.

Just in regard to a tethered cord, I think a tethered cord is most typically thought of as occurring at the base of the spine, in which case imaging of the lumbar area would be necessary to identify it. Although perhaps it can sometimes occur higher up.

I don't think I've seen (on PR) where either @jeff_w or @JenB have referred to their lumbar areas with respect to the surgeries they both had for a tethered cord (after their surgeries for CCI). Perhaps it's basically understood though that a tethered cord release means a surgery in the lumbar area.

Either way, if a tethered cord can be identified by imaging in someone who also has CCI, it seems to me that having the tethered cord release done first makes the most sense since that is a much simpler surgery than a fusion, and perhaps the CCI would be less of a problem (or even be asymptomatic) if the CCI was not exerting its displacement/impinging effects on an overly taut spinal cord. I'm not sure if that's the correct way to think about it though.

 

[Hip]

Just in regard to a tethered cord, I think a tethered cord is most typically thought of as occurring at the base of the spine, in which case imaging of the lumbar area would be necessary to identify it. Although perhaps it can sometimes occur higher up.

That makes sense.

I did read recently (but I forgot where, it may have been one of Jeff's posts) that a tethered cord may only show up on MRI after fusion surgery for CCI. I think this is because the surgery changes the tension in the spinal cord and makes a tethered cord more apparent.

So I guess this means even if the tethered cord were located in the upper half of the spine, it might still not be spotted on the initial upper spine MRI until after surgery. Jeff said that tethered cord is very common in CCI.

 

[frozenborderline]

For ME/CFS patients who have sent their MRI scans to neurosurgeons such as Dr Henderson, Dr Bolognese or Dr Gilete, and received back a diagnosis and report, could you please detail what is in that report?

In particular, does the report include figures for these measurements:

Clivo-axial angle (CXA)
Grabb-Oakes measurement (B-pC2)
Basion-dens interval (BDI) — Harris method
Basion-axis interval (BAI) — Harris method

It includes all of those plus translational bai. But gilete did his measurements in flexion, extension, and neutral , and bolognese only in neutral.

 

[frozenborderline]

The four above measurements made on the MRI image, and are used to diagnose CCI. For example, a clivo-axial angle (CXA) less than 135º is considered pathological, and indicates CCI.

My cxa in neutral was 136 or 135 depending on if you ask g or b. I tjink a cxa below 150 or especially 145 is considered abnormal, whereas a cxa below 135 is considered definitely surgical. I don’t know why bolognese doesn’t include flexion. I know that there are no consensus standardized cutoff points for flexion, but if the cxa is basically a measurement correlating to actual pathology (kinking of the brainstem by the odontoid) then a more acute cxa in flexion is still relevant, because it would indicate neural deformity and perhaps damage. Mine was 128 in flexion and I didn’t flex very far.
Both gilete and bolognese recommended surgery but bolognese has more hoops to jump through before surgery, he is more conservative.

 

[Yuno]

@Hip

Here is my report by Gilete:

She describes a posible cervicomedullary syndrome, with a 0.4 Brainstem Disability Index, KS 40 and pain questionnaires as severe disability pain.

The Upright MRI (30/7/2018) shows:
Neutral CXA 134°
Flexion CXA 130°
Extension CXA 150°
Grabb 6,3 neutral and 6,5 in flexion and 5,6 in extension.
Translational BAI: 4,3 mm (Neu:3,5, Flex: 4,3, Ext: 0)."
No significant cerebellar tonsils ectopia.
C1-C2 neutral misalignent.
Rotary AAI looking left and right.
Signs of subaxial instability at:
C2-C3 & C3-C4. C4-C5 with modic endplates changes. Also, in extension, signs of instability at C6-C7 causing spinal cord impingement.

Thus I think that she possibly has:

1- Craniocervical Instability (CCI)
2- Rotary Atlantoaxial Instability (AAI)
3- Subaxial Instability possibly up to C6-C7 (to be confirmed by cinerradiology)

So, I recommend to complete study by means of cervical spine cinerradiology or DMX to evaluate subaxial instabilities and full spine radiology. This could be done in Barcelona, prior to consultation or surgery, if she decides to come.
So in her case, though final proposal will depend on clinical exploration during a face to face consultation and Cinerradiology, I would probably recommend:
Posterior fusion stabilization initially C0 to C3 though final fusion extension (levels to be fused) would depend on cervical cineradiology, with intraoperative reduction (traction)

 

[moonell]

@Hip

She also reported the following symptoms in relation to brainstem compression, which represents a 95% Brainstem Disability Index

The tip of the odontoid peg lies below level of Chamberlain's line.
No significative cerebellar tonsils ectopia.

CXA - neutral 145°, flexion 138°, extension 167°
BAI - neutral 4, flexion 5,5, extension 1,6
Grabb - neutral 4,6, flexion 7, extension 3,4
Translational BAI: 3,9 mm
Flex BDI: 3,8mm. Ext BDI: 8,1mm.

C1-C2 neutral misalignment.
Rotary AAI looking left and right.
Cervical Subaxial
Cinerradiology will be recommended to finally assess subaxial instability.
Moonell meets clinical and neuroimaging international criteria for diagnosis of craniocervical instability (CCI)* and atlantoaxial subluxation(AAI).

No clear signs of subaxial instability. Loss cervical lordosis with slight kyphosis CL 7.8°.

After reviewing Moonell’s case, though further investigation is needed (see below) and although surgery is the last option to be considered, we think that she could be candidate for:

Occipitocervical posterior fusion stabilization with intraoperative reduction (traction).Number of levels: depending on DMX or cineradiology once subaxial instability evaluated. Probably C0 to C2.

 

[Hip]

Note that Mattie asked that this thread be only used for posting MRI results, and the results of surgical treatment. A better place for general discussion on CCI might be this thread.

 

[Hip]

Thanks very much for posting your reports, @Yuno and @moonell.

Not that I have much knowledge in interpreting this figures, but from what I can work out:

@Yuno:
Your CXA of 134° is pathological (since 135º or less is pathological).
Your Grabb-Oakes at 6.3 mm is more-or-less normal (less than 6 mm is normal, 9 mm or more is pathological).
Your BAI at 3.5 mm is normal (greater than 12 mm is pathological).
Your translational BAI at 4.3 mm is pathological (> 2 mm is pathological; > 4 mm required for surgery).

So I think these two pathological measurements would in part explain why Dr Gilete diagnosed CCI. I understand it's not just the measurements, but also the clinical symptoms picture which is taken into account to arrive at a CCI diagnosis.


@moonell:
Your CXA of 145º is normal (135º or less is pathological).
Your Grabb-Oakes at 4.6 mm is normal (less than 6 mm is normal, 9 mm or more is pathological).
Your BAI at 4 mm is normal (greater than 12 mm is pathological).
Your translational BAI at 3.9 mm is pathological (> 2 mm is pathological; > 4 mm required for surgery).
Your BDI at 8.1 mm in extension and 3.8 mm in flexion looks normal (greater than 12 mm is pathological).

So I think this pathological measurement is in part why CCI was diagnosed.

 

[Hip]

I tjink a cxa below 150 or especially 145 is considered abnormal, whereas a cxa below 135 is considered definitely surgical.

Did you read or see that somewhere? I've been trying to find more info about the gray areas in between a normal measurement and a pathological one. For the CXA for example, healthy and normal is 150º or greater, whereas 135º or less is pathological. But what about in something in between 150º and 135º? Could that be creating symptoms or effects even if not considered severe enough to warrant surgery.

For example, an in-between CXA might be significant enough to affect autonomic functioning, which in turn may impede the immune system from clearing infections, or create immune dysfunction. Maybe a slightly pathological measurement is enough to cause some immune dysfunction.


Interestingly in the Grabb-Oakes measurement, the threshold between normal and pathological seems quite hair-splitting.

A normal Grabb-Oakes measurement is less than 6 mm, and the threshold for pathological is 9 mm; but some neurosurgeons argue that the threshold should be 8.5 mm instead (see Bolognese in this video at 49:30). Well, if just 0.5 of a millimeter can make a difference, that seems quite a precise threshold.

I don’t know why bolognese doesn’t include flexion.

Dr Paolo Bolognese in this 2018 video at 37:02 explains why he uses standard supine MRIs, not upright MRIs with flexion and extension head positions (he only occasionally uses uprights as an additional test).

One of the reasons he states is that upright MRIs are all 1 tesla, and thus have a lower image resolution than supine 3 tesla MRIs. You also get motion artifacts in uprights, because the head is not positioned in an immobilizing frame, and so moves, blurring the image. Furthermore flexion and extension are not standardized in terms of head position.

 

[Hip]

@bombsh3ll, can you check your BAI figures and translational BAI figures, because they don't seem to add up. Thanks. The figures you gave are:

BAI neutral 7.45
BAI flex 9
BAI ext 7.44
Translational BAI 2.06

Translational BAI I believe is calculated by subtracting BAI ext from BAI flex, but if you do this the answer comes as 1.56 mm, rather than the 2.06 mm you stated.

 

[Hip]

Cerebellar tonsils ectopia: 4 mm.

Silencio, did Dr Gilete diagnose you with Chiari malformation? Because in this paper it says Chiari malformation is defined by cerebellar tonsillar position greater than 3–5 mm below the foramen magnum. See image below to see these tonsils.

I see he has diagnosed you with "cerebellar tonsils descent", which I assume might be another way of saying Chiari malformation, but I am not entirely sure.

Chiari malformation

 

[Countrygirl]

An Austrican patient with EDS and CCI has just died as a result of her neck fusion surgery in Spain.

She was told she had a 50% chance of not surviving the surgery.

She had started fundraising for it on 8th June, I believe. Her husband is continuing with it to bring her body home.

Story

I need a lifesaving Surgery in Spain, at the Chiari and Hypermobility Center in Barcelona. Performed by Dr. Gilete. My Cervical Spine is unstable and causes Brainstem compression. I'm in Agony. The Surgery costs will not be covered with Insurance in Austria. I'm between Life and Death. I do have vascular Ehlers danlos syndrome, I'm at high risk of Death. Maybe I won't survive the Surgery. My Husband needs the Money for the Surgery costs and eventually to carry my body home for the Funeral. I was given a 50% Chance of Survival. This would be my only Chance, without Surgery I will have to Suffer in Agony till I die of the Instability and Brainstem compression..Paralysis, Breathdifficulties till Suffocation.

Gigi Brecsik • June 8, 2019