Tracking CCI / AAI MRI & Treatment outcomes

[Hip]

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.

Very sad. Looks like the operation was her only chance, as she would have died anyway, due to the paralysis of breathing muscles. Jeff mentioned here a girl who died of CCI, which caused her to stop breathing in her sleep.

 

[MEPatient345]

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.

Yes, he diagnosed me w the descent but not chiari, I was wondering what that meant, thanks for the info. On my imaging, the tonsils do not look like they are slipping down that much. I’m interested to see what Bolognese says...

It is really devastating about Gigi.. she must have been suffering so much.

 

[User891]

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

That is very very sad. Thank you @Countrygirl for providing further context, from what I understand Vascular EDS is very rare and serious and can significantly limit life expectancy (with or without CCI) unlike the other forms of EDS.

 

[bombsh3ll]

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

When I went there for the consult and had the cineradiology he said that showed cranial settling too.

B xxx

 

[bombsh3ll]

@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 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.

That's just what I copied from his report. It looks like you have flagged something odd there that I was not aware of as I did not know what BAI was.

B xxx

 

[bombsh3ll]

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.

I just saw that. Such a shame, & a very brave lady going into it with those odds. May she rest in peace.

B xxx

 

[Capecodder]

I just saw that. Such a shame, & a very brave lady going into it with those odds. May she rest in peace.

B xxx

I am looking into whether my son, age 15, diagnosed with ME/CFS has CCI. I have a few questions. An upright MRI was ordered, with flexion/extension. However, I spoke with Dr. Bolognese’s office, and they want a supine MRI for consult??
I called Dr. Henderson, and they don’t do phone consults. We live in NC, so would prefer to not have to travel unless we know there is an issue.

Has anyone seen either of these doctors? Both my son and I have EDS as well as ME/CFS.

BTW, traction has made my neck and symptoms worse. I’m not sure if that rules out CCI for me. Too scared to do it on my son, risky.

 

[Capecodder]

That is very very sad. Thank you @Countrygirl for providing further context, from what I understand Vascular EDS is very rare and serious and can significantly limit life expectancy (with or without CCI) unlike the other forms of EDS.

Very sad to read this.

 

[bombsh3ll]

An upright MRI was ordered, with flexion/extension. However, I spoke with Dr. Bolognese’s office, and they want a supine MRI for consult??
I called Dr. Henderson, and they don’t do phone consults. We live in NC, so would prefer to not have to travel unless we know there is an issue.

Dr Gilete will review imaging remotely for a fee. A rotational MRI is also required.

B xxx

 

[Capecodder]

Dr Gilete will review imaging remotely for a fee. A rotational MRI is also required.

B xxx

Thanks, where is Dr. Gillette?

 

[Joly]

@Capecodder Dr G is in Barcelona, in Spain. His website is https://drgilete.com and you can contact his team through the email form there.

@Silencio Have you checked your flexion scan? From what I've been reading, often tonsil descent is more apparent on these which is why some doctors prefer F/E scans to diagnose Chiari.

 

[Hip]

Does anyone know, is it only Dr Gilete who sends patients a detailed report containing his diagnosis and all the measurements (like CXA, Grabb-Oakes, BAI, etc)?

Because for the 5 members who very kindly posted their report and measurements above (thank you!), all 5 reports were from Dr Gilete, and none from Dr Henderson or Dr Bolognese.

So far, 39 members have voted in the poll at the beginning of this thread, and collecting the details and measurements from these members via my questionnaire (which I am working on) might be very useful for researchers such as Ron Davis to see. That's why I am asking if all these neurosurgeons put the measurements in their reports they send back to the patients.

 

[MEPatient345]

Have you checked your flexion scan? From what I've been reading, often tonsil descent is more apparent on these which is why some doctors prefer F/E scans to diagnose Chiari.

I’m not sure visually if worse.. I haven’t measured angles myself..

 

[MEPatient345]

Does anyone know, is it only Dr Gilete who sends patients a detailed report containing his diagnosis and all the measurements (like CXA, Grabb-Oakes, BAI, etc)?

Because for the 5 members who very kindly posted their report and measurements above (thank you!), all 5 reports were from Dr Gilete, and none from Dr Henderson or Dr Bolognese.

Bolognese seems to just respond with a line or two saying whether or not you are a candidate. Then he sends a report after you’ve had Skype appt with him. @debored has a report from him..

Henderson, Patel and Sandhu you must see in person. So I think bc so many of us are housebound or abroad, that’s why the gilete option is the most common. Apparently it is common to have slightly differing measurements by a mm or two. And differing opinions on what is pathological. My Medserena report had my tonsilar ectopia measured at 8mm, which seemed wrong. I have written back to them asking for the other measurements.

 

[Hip]

Henderson, Patel and Sandhu you must see in person. So I think bc so many of us are housebound or abroad, that’s why the gilete option is the most common.

Ah that makes sense.


@debored13, do you know if your report from Dr Bolognese includes all your CCI-related measurements (CXA, Grabb-Oakes, BAI, etc)?

 

[AnkeC]

I have a report from Dr Gilete, and am waiting on one of Dr Bolognese's famous two liners. Since I would not choose the US for surgery (if I were to choose surgery) I don't think I will invest too much in consultations with Dr B. But I will have to wait and see what he says first.

In my report most of the measurements fell into normal limits..... apart from Trans BDI. But He obviously saw other things, which combined with my symptoms, led him to come to his diagnosis.

Here is my report @Hip , with personal & identifying info removed:

She had reported on previous questionnaires that her main symptomatology was:
· Post exertional malaise
· Body pain & weakness
· Headaches
· Right sided, neurological, stroke-like symptoms
· Cognitive deficits & memory issues

She also reported symptoms in relation to brainstem compression, which represents a 65% Brainstem Disability Index.

Her symptomatology seems to clearly correspond to a cervicomedullary syndrome.

Pain questionnaires
Oswestry: 44%. (41%-60%: Severe Disability)
VAS: 5,4. (That's like the 1-10 pain scale they ask you at hospital.)
Karnofsky score: 70. (Cares for self; unable to carry on normal activity or to do active work. )

Neuroimaging

We evaluated:
· Several Brain MRI from 2015 to 2019
· Flexion-extension & rotacion CT scan 29/5/19

Craniocervical Junction

The tip of the odontoid peg lies below level of Chamberlain's line.

I cannot do measurements in an upright MRI images, as usual. Nevertheless the measurements in the different neuroimaging provided are:
Neutral CXA 144°.
Flexion CT scan: BDI=3,8; CXA 156°.
Extension CT scan: BDI=7,8; CXA 176°.
Translational BDI: 4 mm.

Rotary AAI looking left and right.
In some sequences of the MRI imaging, I can guess that there is bilateral internal Jugular vein compression at the level of digastric muscle and lateral mass C1. Signs of damage at left C0-C1 joint.

Mrs XYZ meets clinical and neuroimaging international criteria for diagnosis of craniocervical instability (CCI)* and atlantoaxial subluxation(AAI). *Consensus Statement . 2nd International CSF Dynamics Symposium. 2014. Abbreviations: BDI: basion dens interval, CXA: clivo axial angle, BAI: basion-axial interval. ADI: atlantodental interval

Cervical Subaxial
Global disc protusion C5-C6.
Right disc herniation C6-C7.
Cinerradiology will be recommended to finally assess subaxial instability.

Impression
This is an online evaluation without face to face consultation. Clinical personal examination is a part which should be done to evaluate properly the case.

That is a missing part in online consultations, obviously, and in Mrs. XYZ's case is especially important. We mainly focused in evaluation of the craniocervical junction & cervical spine.

After reviewing Mrs. XYZ'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. At least C0 to C2.
AND
Depending on positive related symtomatology: Anterior discectomy and fusion (ACDF) C5-6 and C6- C7.

Further testing recommended

We recommend completing Mrs. XYZ's evaluation at this point of time with the following:
o Cinerradiology
o MRI Venogram, gadolinium enhaced, in order to see all intracerebral venous system and cervical (neck) venography in all internal jugular vein trajectory from lateral sinus to subclavian vein.

___________________________________________

If there’s a chance for a personal consultation, besides some special details that should be considered in her case, we will discuss with her the proposed surgical procedure and also the potential risks. Notwithstanding these very consistent findings, there is no guarantee that the surgery will significantly improve her craniocervical instability but if there is something it can be done in her case, this is, in our opinion, the proposed fusion and decompression, as there is a high probability of improvemen

In our experience, symptoms only worsen if left untreated. Surgery is currently the only proven treatment for advanced cases of instability.

According to the responses provided on the questionnaires, her symptoms present themselves as disabling as her brainstem disability index is 0,65 and pain is scored as severe disability pain.

Diagnoses

Principal diagnoses related to current situation:

1-Craniocervical Instability (CCI)
2-Atlantoaxial Instability (AAI)
3-Global disc protusion C5-C6
4-Right disc herniation C6-C7
5-TO BE CONFIRMED: bilateral IJV compression

Recommendation

Occipitocervical posterior fusion stabilization with intraoperative reduction (traction).
Number of levels: depending on DMX or cineradiology once subaxial instability evaluated. At least C0 to C2.
AND
Depending on positive related symtomatology: Anterior discectomy and fusion (ACDF) C5-6 and C6- C7.

___________________________________________

So yeah, unexpected to say the least.

 

[valentinelynx]

BTW, traction has made my neck and symptoms worse. I’m not sure if that rules out CCI for me. Too scared to do it on my son, risky.

All I can add is that Dr. Kaufman & by extension, I gather, Dr. Bolognese, emphasize that manual cervical traction should only be performed on EDS patients by physical therapists with extensive experience with EDS patients, so they know the need to be gentle and slow.

 

[frozenborderline]

Does anyone know, is it only Dr Gilete who sends patients a detailed report containing his diagnosis and all the measurements (like CXA, Grabb-Oakes, BAI, etc)?

Because for the 5 members who very kindly posted their report and measurements above (thank you!), all 5 reports were from Dr Gilete, and none from Dr Henderson or Dr Bolognese.

So far, 39 members have voted in the poll at the beginning of this thread, and collecting the details and measurements from these members via my questionnaire (which I am working on) might be very useful for researchers such as Ron Davis to see. That's why I am asking if all these neurosurgeons put the measurements in their reports they send back to the patients.

I thought I mentioned that I got results from bolognese and gilete , both included measurements but bolognese only included measurements in neutral. I don’t remember all the measurements he included, having really problematic

Ah that makes sense.


@debored13, do you know if your report from Dr Bolognese includes all your CCI-related measurements (CXA, Grabb-Oakes, BAI, etc)?

I believe so (but am on the road and doing very very poorly so am too tired to check atm ). I am sure that it’s included neutral cxa, but notably didn’t include cxa in flexion and extension. It included some other measurements that I didn’t note in the moment Bc I was so fixated on cxa

 

[Hip]

I have a report from Dr Gilete

Thank you for posting your report, AnkeC.

I think in your case, the measurement that led to the CCI diagnosis may be your translational BDI of 4 mm, which I think may be pathological

I have not seen the translational BDI measured in other people's reports posted on this thread; it's usually the translational BAI that Dr Gilete measures. A translational BAI of more than 2 mm is pathological, and I assume that a translational BDI of more than 2 mm will also be considered pathological (though I am not entirely sure, as I have not seen any studies or videos where the translational BDI has been mentioned).

Both the BDI and BAI are termed Harris measurements.


Your other craniocervical instability measurements look normal.

Your CXA of 144º in neutral is normal (135º or less is pathological).
Your BDI of 3.8 mm in flexion and 7.8 mm in extension are normal (greater than 12 mm is pathological).
Your translational BDI of 4 mm may be pathological (I am assuming greater than 2 mm is pathological).

I cannot do measurements in an upright MRI images, as usual.

I don't understand why Dr Gilete has written the above statement in your report. I thought Dr Gilete specifically requested upright MRIs in neutral, flexion and extension positions in order to perform his measurements. Maybe he meant to say "I cannot do measurements without an upright MRI".

It seems he may have had some difficulties in making his measurements on your MRI and CT scan images, as he has not included the BAI and the translational BAI in your report, which he has included in everyone else's reports so far. Maybe that's why he switched to using the BDI and translational BDI as a substitute, as perhaps those were easier to measure on your images.

 

[Joly]

"I cannot do measurements without an upright MRI".

I also read it as this or perhaps: "I cannot do measurements from upright MRI images, as I usually do."
Meaning because they were not available?