77. Neurosurgical Insights from Paolo Bolognese, MD and Guest Cohost Pradeep Chopra, MD

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Linda Bluestein, MD (00:01.821)
Welcome back every bendy body. This is the bendy bodies podcast and I'm your host and founder, Dr. Linda Bluestein, the hypermobility MD. This is going to be a great episode. So be sure to stick around until the very end so you don't miss any of our special hypermobility hacks. As always, this information is for educational purposes only and it's not a substitute for personalized medical advice.

Linda Bluestein, MD (00:31.429)
Before I introduce your guest, I would like to reintroduce you to my colleague and friend, Dr. Pradeep Chopra, who will be your guest cohost for this episode. Dr. Chopra is a pain management expert who specializes in complex pain conditions and has been a guest multiple times on the Bendy Bodies podcast. Be sure to check out those episodes if you have not heard them already. Dr. Chopra, hello and welcome back to Bendy Bodies.

Pradeep Chopra, MD (00:56.229)
Hello Dr. Bluestein and Dr. Bolognese, my pleasure to be here once again.

Linda Bluestein, MD (01:03.301)
Wonderful. And could you introduce our special guest for us?

Pradeep Chopra, MD (01:07.809)
Yes, our very special guest for us today is the wonderful Dr. Paolo Bolognese. He is a neurosurgeon. He is a founder of the Chiari Neurosurgical Center in New York. Dr. Bolognese is also the board of directors of the American Syringomyelia and Chiari Alliance Project, Inc.

He is also on the Scientific Education and Advisory Board of the Chiari Syringomyelia Foundation, which is also known as CSF, and a member of the International Consortium on EDS, HSD, and Related Disorders. The Chiari EDS Center is focused on the diagnosis and treatment of Chiari I malformation, syringomyelia, craniocervical instability, tethered cod, Eagle’s syndrome, idiopathic intracranial hypertension and intracranial hypotension. That's a lot of work you do. Dr. Bolognese’s surgical experience includes more than 1,600 Chiari decompressions and 900 craniocervical fusions — 300 of which with Condylar screws. 

Paolo Bolognese (02:17.874)
I feel tired already.

Pradeep Chopra, MD (02:32.249)
He is just a few short of mine.  He's also on the board of many national and international organizations focused on Chiari and Syringomyelia and has also made contributions in the field of intraoperative ultrasound and laser Doppler flowmetry. I have no idea what that is, but sounds really fantastic.

Paolo Bolognese (02:35.702)
Yeah.

Paolo Bolognese (02:58.218)
Did it.

Paolo Bolognese (03:03.042)
Thank you very much.

Linda Bluestein, MD (03:05.238)
Dr. Bolognese, hello and welcome to Bendy Bodies.

Paolo Bolognese (03:09.282)
Thank you for having me here. I'm very, very glad to be here.

Linda Bluestein, MD (03:12.381)
Oh, wonderful. We are thrilled to chat with you. And if you could start out by telling us briefly how you became interested in EDS and related conditions.

Paolo Bolognese (03:22.17)
It was a total accident. I still remember the room in which it happened. I trained twice in neurosurgery — once in Europe. And then my chairman, that was kind of his favorite, had the bad idea to expire. So, since Italy is kind of very politicized much more than here, it was easier for me to …

Linda Bluestein, MD (03:24.157)
Mm.

Paolo Bolognese (03:51.658)
go somewhere else and start from scratch. So I came to the United States; I retrained again. And the chairman who trained me decided to take me when he left his former hospital. So, he wanted to start the Chiari Malformation Center. And at that point, you know, there were just a few cases done nationwide. So the fact of having two neurosurgeons building a center together was kind of like building a Yankee stadium and you just have a couple of kids from the neighborhood going and playing. It did not make sense. What we did not know is that we were ahead of the curve of a big wave of incoming cases, which were newly diagnosed by MRI and by patient awareness. So long story short, we’re having this Chiari Center and with, you know,

Pradeep Chopra, MD (04:28.091)
Yes.

Linda Bluestein, MD (04:28.903)
Ha ha.

Paolo Bolognese (04:52.054)
We’re operating together. I was seeing the outpatients and I was seeing, you know, several patients in a day and several for Chiari  surgery means six in a day. And all of a sudden, I see that two of them had the syndrome [EDS]. And then the following time we’ll see patients. I saw another two and then the following week I found another four. And you know, just like it.

What were the chances for these things just to be associated by sheer chance? So I went to him and I said, you know, I think that there is something strange here. I found it too many times. He gave me a dose of healthy skepticism, you know, like because he was the older guy and I was the younger guy. And then one month later, I went back to him and I said, listen, you know, there is definitely … It is, I keep saying it, so it is not an accident. And he was again, a skeptic. And by the 89th patient over a few months, I made an Excel file for him and I showed him, okay, so we have patients who are younger, the ratio between women and men is instead of 3:1 is 9:1. They have a different phenotype, they’re younger, they have different other comorbidities and they tend to have all these things about which we know just to remember a couple of footnotes from school. Ehlers-Danlos Syndrome was not really a main thing in education, in medical school. So at that point, he is not that he was convinced, but he picked up the phone and he called the geneticist from Duke, who was the international leader in the field of genetics of care. And she says, you know, never heard it myself.

Linda Bluestein, MD (06:27.389)
Mm-hmm. Yeah.

Paolo Bolognese (06:47.67)
But statistically, you have seen these many Chiari these many EDS, the chances for this to be chance alone were, I don’t remember how many, several millions to one. So I said, I think that there is either that true-true-unrelated or there is a genetic association here too. I really encourage about, I’m going to look into genetically and you guys should keep working on it clinically. So since we did not know anything.

So he worked at NIH when he was younger, for two or three years, around the time of the Vietnam War. And so he picks up the phone and he calls the person in charge for connective tissue disorders. So on one side, it was Miller, myself, and on the other side, there was Miller, the equivalent, and her junior. And that person was Clair Francomano. And Clair Francomano was the director of the Department of Aging, which is practically connective tissue disorder and all those things. And all of a sudden it was a bizarre phone call because all of a sudden we were all four of us are giddy because we’re completing each other’s sentences. So all of a sudden we are, you know, we’re kind of, there is this chemistry instantaneously because they were having,

Paolo Bolognese (08:16.11)
pieces and vice versa. So they could not explain why there are so many neurological problems in their EDS patients. On the other hand, we’re simply ignorant about what, probably we shouldn’t have opened the textbook before talking to them. But so at that point, it was natural for us to start cooperating. So that’s how we started.

Then the following 20 years were not as linear. You know, the beginning here, it looks okay, fine. You know, we have this association. There are these patients have a problem here, easy. They have an instability. Sometimes we see it with trauma. Sometimes we see it with tumors. We can fix that. And now starting the problem because we did not know what we did not know.

And we didn’t know that things which were going to work technically in everybody else was going to not work the same in people with EDS. So, you know, at that point, there was really nobody else for us to call, you know, and you couldn’t pick up the phone and say, okay, so you did, you worked on this for some more years than us. We’re kind of, we’re so...

Pradeep Chopra, MD (09:24.881)
True.

Paolo Bolognese (09:39.966)
not the head because we’re smart, you were just stumbling to that. And so we couldn’t call anybody and we started doing surgeries and then we learned the hard way by eggs on the face, what was working and especially what was not working technically by applying the standard technique that we knew about. And so we started going at...

national meetings, we started talking about Ehlers-Danlos Syndrome and nobody bought into it. So for years, actually, we were told, I don’t think that’s the thing. I never seen the classical reaction of other colleagues, which was obvious, was I’ve never seen a patient with Ehlers-Danlos Syndrome my entire career among the Chiari population.

Linda Bluestein, MD (10:15.986)
Mm.

Linda Bluestein, MD (10:35.509)
Oh wow.

Pradeep Chopra, MD (10:36.237)
Hahaha

Paolo Bolognese (10:38.366)
And since I’m kind of, right now I’m better behaved, but back then I was very irreverent. Not for the fact of being irreverent, I just like was very zealous, so like if I see something, I want to show it to you. But I was not very diplomatic about it. So generally when you have a chairman for a major institution from that I’m not gonna mention. And...this guy says, I have never seen in my life. Usually you say you should just leave it alone. And instead I went directly after him. And I said, okay, I have a counterpoint and it was a patient and doctor meeting. And I said, okay, everybody in the room and I put a picture of the Beighton.

Paolo Bolognese (11:32.87)
Beighton score on the thing. Everybody can do this and one third of the room could do it. Everybody can do this one. And so I turned up, turned around to this other guy who was much bigger than me and I said, you see, if you never tested you would never know it. So that didn’t make me very popular and but that was my problem. But he took it.

Another number of years for that not to be accepted, but at least for other people to be open-minded. So this next person coming on the scene was Dr. Henderson. Dr. Henderson had a, like me, had kind of a strange career because he was coming from Australia. And like me, he went in the military as part of the regular draft.

And actually was in Beirut when the barracks were blown up by the terrorists. So he was there and he was a medical officer taking care of the Marines. I don’t remember how many hundreds of Marines were killed on that day. Anyway, so he came before coming to the United States, he made a stop in London in a place called Queen’s Square.

And Queen’s Square was at that point, was the center of the world for everything which was related to craniocervical instability. Because not at that point, not because of EDS, but because the old British ladies, they were developing this strange disease called rheumatoid arthritis, which is a sort of inflammatory reaction, which attacks the joints, et cetera. But instead of making just the ligaments weak.

Pradeep Chopra, MD (13:22.33)
Right.

Paolo Bolognese (13:27.202)
It creates an inflammatory mass called the panus, which compresses, pushes things around. So he was there and he was trained by the god of craniocervical instability called Crocker. So when he came in United States shortly afterwards, he had the same reaction. He said, all right, I started craniocervical instability, I know how to do craniocervical fusion much more than an average neurosurgeon in the States.

I understand the point because I’ve seen enough people with having destruction of the joints. So I understand the point, I’m going to start going ahead. So all of a sudden, we were two hospitals starting working on that. Now all of a sudden, a lot more and more patients started coming over. And at the beginning was Chiari I malformation and Ehlers-Danlos was like a comorbidity. So it is...what was called now complex Chiari, which means that the Chiari is now, the surgical management is rendered more complicated by the fact that the joint is defective. But the very first time that we met the patient was purely EDS was the following year, was in 2002, after the first time that we observed. And it was this girl that now, you know, would check all the boxes for the three of us.

Pradeep Chopra, MD (14:39.312)
Right.

Paolo Bolognese (14:56.95)
you know, very debilitated, age 17, three, four years of progressive debilitation, unable to get out to the wheelchair. Nobody sees anything, nobody is all in your head, you know, this is the usual thing. And the mother who had gone anywhere, she came to us because she thought that maybe as a form of Chiari I malformation, came there to check everything else. And I look at the MRI, the MRI was kind of some shade of gray, but was more towards, in the shade of gray, it's between full Chiari and normal, was more in the left side of the spectrum. But I was seeing this patient was really sick. So I don’t know what happened. I got up, I went around the table because I really did not know what to call, and I grabbed her head and I pulled her up, and all of a sudden, she woke up.

And she perked up, she was able to move around, she was able to swallow. And then I left it down and she went reverted. So she did five, six times. The mother was all excited because she hadn’t seen it that way, even for a few seconds for five years. So I went back to Mira, you know, my mentor, I told him, listen, this is a kind of a black box scenario. I do not know what’s inside.

Neither do you because right now the MRI looks normal-ish. There is no Chiari I malformation. There is no retroflexed odontoid. There is no nothing. But every time I pull her up, she feels better. Every time I let her go, she feels worse. So looks like some kind of craniocervical pathology resembling cranial settling like settling away of a house on the foundation. Don’t understand how it is, but we if

Pradeep Chopra, MD (16:43.242)
Right.

Paolo Bolognese (16:48.746)
make it feel like this makes it feel better, we can do a fusion. So at that point, there was already a jump from the complex Chiari, which the MRI was self-evident, to a scenario we have now, pure neuro-EDS with instability, in which the patient had that normal MRI, but a big clinical change, much more than a Chiari I malformation by itself. So...

You know, like Mira, they had the courage, professional courage to say, you know, like one time he’s a young punk, at that point was 42, not so young, but I was still a young punk compared to him. And I was saying, oh yeah, like we can do this. He could have said, you know, the heck we’re going to do it. I’m going to have half of the neurosurgical community after me. And instead he said, you know, I see the same. And so.

We had her coming back, we did a fusion, and now we know that it’s not a surprise for us, back then was the hell of a surprise to see that she went well. And then not only she went well, but her story was kind of like a Cinderella story. he ended up becoming a supermodel for the Ford agency in New York City. And she stayed there and became she was a high-end supermodel international for four or five years then she got married and now I’m uncle for the third time she’s every time baby is born she sends me pictures because I’m the uncle so I these were kind of two different paths and so those are the second patient was so kind of the Rosetta Stone of neurology it was the step

Linda Bluestein, MD (18:29.758)
Ha ha!

Paolo Bolognese (18:44.45)
forward from just the Chiari EDS. And then at that point is all the problems. The problems are diagnostic and the problems are technical surgery. And that they start here at that point. Like when do you call it? How do you call it? How do you grade it? What kind of workup you do rather than just shooting from the hip? Practically to create, you know, science is always something that doesn’t come pre-made as a package. It’s something that keeps going, sometimes accelerating, sometimes stagnating. And when there is no book to read, you just find out. It is not.

Pradeep Chopra, MD (19:35.353)
Well, you write the book. In that case, you write the book.

Paolo Bolognese (19:40.426)
You know, like some chapters you discover afterwards that you write them and then you retrospect their, the test of time says, okay, you have to do these other things instead, you know, like, but you’re always between the fog of war, the perspective, etc. is, you’re always as smart as the last lesson you learn.

What we did not understand at that point was that, again, being ignorant was, and having nobody to ask, was that the problem was way more complex than we were anticipating. The neuro EDS model was we were having so many additional pieces that we’re still finding out. And so then the diagnostic, and by again, how to...

Paolo Bolognese (20:35.114)
assemble a workup which makes sense and on the other, because there is a balance. You cannot be so zealous. You say, okay, it is what it is and I’m going to operate on it. You have to have some criteria. The concept of the criteria is that you have to be rigid and flexible at the same time. You have to be flexible about the idea strategically. The criteria that you have today

could evolve over time. But you have to be rigid tactically. So the version 3.1 or the set of criteria you have now, that’s a line in the sand and you do not pass it. You do not do a model again and you push on the other side when nobody looks. Because then otherwise you’re not gonna have a hard ground to stand on when you look back and you make an evaluation of the version 3.1.

When you discover there are some limits, and then you do the version 4.0. And then there were all the problems, which are purely technical about the surgery, about OK, you have a surgery that works for any other things. You do it. And then you discover afterwards, again, what works, but especially what doesn’t work, and you move forward. So it’s a long answer, as usual.

Pradeep Chopra, MD (22:03.461)
No, no, that was a great story because we wanted to know how you began. And it looks like you and Dr. Henderson were the pioneers in this field. And it’s not, I mean, even today, after all these years, we’re still learning and understanding the nuances of EDS. And you were in the middle of the...

I mean, the spine itself, even without EDS, is such a complex structure. And even in the spine, the cervical spine is probably the most complex structure. You know, it balances the heavy head and you are the pioneers in this surgery. So the question that I have is that, you know, if I suspect somebody has

a patient with EDS has CCI, craniocervical instability or Chiari I malformation and I say, hey, listen, I want you to see Dr. Bolognese. What is your process of evaluation? How do you start? So our listeners want to know like, how do you evaluate them?

Paolo Bolognese (23:20.658)
Okay, again, what I have, the version that we have now is the current permutation of all the things we have done over the years. And the good thing for me in terms of mentality is that I had three mentors, which is like extremely lucky for somebody to have.

had the biggest influence over me was Miller. Miller was OCD to the core and he was just

He was never focused on, yeah, it is like the shepherd that has 99 sheep and they’re all fine and he’s okay, but he loses his sleep over the one sheep that is lost or he’s struggling or whatever. And every now and then I would say, okay, so you know, the textbook says that the complication rate for this is good, well, we’re better, so we’re fine. I know, it’s not good enough.

Give me an idea like the Chiari I malformation surgery back in the 80s had a solid 50% complication rate So when we went down, yeah, so when we went down to you know like around 10% complication rate to what there was an enormous improvement. Obviously comes from yeah it’s not the not be genius like tying your shoes you do it over and over and you push yourself and

It’s inevitable you’re going to get better numbers. But I would say 10%, much better. And he would say, no, it’s not good enough. And one of them was the most important complication was cerebrospinal fluid leakage for a number of different reasons. So we went down to 3%, and it was still not good enough. And then we went down to 0.3%. And at that point, all right, just statistically, it’s going to be.

Paolo Bolognese (25:31.006)
It’ll be difficult to go lower down. And so he was always pushing. So we’re, and again, going through the other direction, which is the diagnostic. The issue is not so much how many times you have it correct with your procedure, but how many false negatives and false positives you have. And you do not realize the false negatives you realize later on when

you know, either somebody else operates or you change your criteria and stuff. This was negative before, but that was wrong. But the false positives are the most important in terms of overall heat and overall, you know, eggs in the face for the, for evolving discipline or sub-discipline because a false positive is going to potentially translate in a surgery.

So at that point, you have a surgery that has not worked for, can be because of poor selection or poor execution of a technique that you know, but you did not execute it correctly, or simply because the surgery was indicated, was correctly executed, but that technique is not enough, and you had to

resort to something different that is not in the book yet. So after the first five or six years, we’re already having, you know, something like 300 cases. And we’re all excited because, OK, this is a new, you know, craniocervical instability is not an unknown pathology. But linked to EDS,

to go to that degree and with those connotations was something new. So we’re excited about, we’re going to do 300 and then we’re going to report it. We’re going to wait some years because you cannot just do the surgery and say, OK, we observed for three months and we’re fine. No, you have to have a good post-op. And we look at that and the complication rate was higher than the standard, for example, trauma

Paolo Bolognese (27:53.278)
or other pathologies like congenital in children, not enormously higher, but higher enough that the implication was if we publish, we’re not gonna look great. And so also the pathology is not gonna look that great because it’s not gonna get a good reputation because...

It kind of can sound disingenuous. They say, yeah, we had 10 times more than next level disease in this patient, but it’s not us. It’s not because of us. It’s because of the pathology. A reviewer looks into that as, yeah, right. We’re not going to publish it. So at that point, we did not publish intentionally the first hundreds of cases we did that we did with a bar plate.

because they were not, you know, in order for something to be credible, you have to have at least the same outcome and the same complication rate of a conventional technique, conventional pathology. And then you can sell the new pathology for what it is. But anyway, like going, I took credit for it. What is the, so we had the same problem about the, about the diagnosis. 

And you look at the MRI, it looks normal-ish. Your patient’s EDS, and that was pretty much it. And so over the years, we added more and more. So what is the situation now? The situation now is that we have an approach which is multi-legged or that one of a puzzle. And we pass in a situation in which the patient comes to be evaluated, already pre-screened by somebody else.

level of suspicion before we send them to trial. It’s like you are the DA and you listen to the police, you look at the body of evidence to see if somebody qualified to be indicted, we send to trial. So we look at clinical history, provocative testing, radiology, and test trial. So the clinical is

Paolo Bolognese (30:18.794)
is simple. There is a series of symptoms which are typical of every anatomical region. So if you have a sore, a broken ankle, you’re going to be limping out. If you’re going to have a bad stomach, you’re going to have problems digesting. So there is a lot of cables and structures passing in the craniocervical junction, so there’s going to be a long list of signs and symptoms.

Those are not exclusive of craniocervical instability, but they’re linked to the region. So if you have a trauma, a tumor, a congenital malformation, or neuro-EDS affecting there, you’re going to have the same symptoms with different kind of severity, different kind of grouping, et cetera. But if you look at the list, you’re going to see that list becomes hot in the sense of how many positives you’re going to have.

So that’s number one. Number two is provocative testing. So it is like if I have a broken arm and I would go like this, even if the arm is, even if the two stumps are aligned, but they do like this, the patient is gonna complain. So it’s like kicking the tire. You play with the joint and if the patient is these chief complaints and the symptoms of these area get worse or better respectively, that means that you are onto something for that.

So that’s number two. Number three is the history. So if somebody, for example, is already flexible before, one time falls on the head and twists their neck and all these symptoms start afterwards is one of the many parameters. Now recently, for example, we are seeing a lot of patients coming up with deteriorating EDS or a sort of acquired form of EDS.

in long COVID patients with a long COVID kind of pushing the people over the edge. We’ve seen a lot of tropical diseases. So the history now is the third thing. The fourth one is morphometrics. You know, the majority of these patients when they come in, they do not have so much in terms of radiology. So there is a big asymmetry between the chief complaints, which are devastating. Quality of life is poor.

Paolo Bolognese (32:43.938)
And neurological exam quite often is normal-ish. So you don’t have so much of a massive, you know, the massive hyperreflexia or clonus you can find in some patients. Most of the times the neuro exam is, the signs are less than the symptoms. And the quality of life is enormously out of proportion with what the MRI presentation is. So.

What we use at that point is the morphometrics. Morphometrics were introduced to us by my good friend Misano Shikawa. Misano Shikawa is currently the chairman of neurosurgery in Osaka. And he wrote one of the very first articles about morphometrics and Chiari I malformation back in the late 90s. Obviously, he knew Dr. Mira, and one day he asked to come.

is a not so young a neurosurgeon, but he wanted to accumulate a lot of additional data with morpho-medicine. So he asked, can I come for a few months? A few months became six years. We’re having the largest database about Chiari I malformation ever assembled at that point. So he came and we said, all right, it’s going to take him two years to do all the measurements. It was done in three months.

because the guy is insane. And he was doing without computers. Back then there was nothing, but he was just using a goniometer and a special kind of paper that called millimeter paper. But he was doing everything by hand. It was super fast. So at that point, when we started meeting the first patient with EDS, came the point of, all right, so he looked into the existing literature about morphometrics.

And all the morphometrics which were available at that point were for trauma. And there were about 18 parameters. Noticeable, the Grabb or PBC2 did not exist yet. It was introduced a few years later. But there were 18 parameters back then. And the only principle was, whatever parameter you use, you cannot just use one, you have to use two for the sole principle. The sole principle is you look at the sole, the fish.

Linda Bluestein, MD (35:05.411)
Mm.

Paolo Bolognese (35:09.546)
like this looks like gigantic, like this, it looks different. So you have to have at least two complementary parameters to make an evaluation. Otherwise, you’re going to have too many false positives, too many false negatives. So we tested for during invasive cervical traction a bunch of these. And we discovered that he was coming to us with his 18 parameters. And we were working together.

And we were having an understanding about what was abnormal with the pathophysiology joint down to the levels which were extremely sophisticated. But it was very counterproductive in terms of screening. So we boiled it down to three parameters, which then became four after the adduction condition on the Grabb. So certain things we noticed were not really that helpful, like the power ratio, for example, in EDS.

or the chamberlain, et cetera, but in others which were more helpful than usual. So what we’re doing now, we’re using these four params. Go ahead.

Pradeep Chopra, MD (36:18.657)
Dr. Bolognese, just one question for our listeners. When you say morphometric measurements, you mean measurements on the MRI, the angles that we are talking about, right?

Paolo Bolognese (36:32.142)
Yeah, it is almost correct in sense that the morphometrics are measurements of distances, angles, or areas or volumes that you can perform on different neuroimaging media. Okay. For example, all the morphometrics of the craniocervical junction, which were born several years ago, and then there were

became a sort of a canon around the late 50s. They were all done on X-rays. The CAT scan happened afterwards. The MRI happened afterwards. And when, so we started applying into CAT scans and MRIs at that point. So we boiled it down to four and then we started applying to MRIs and CAT scans. And we realized that there was one issue, you know, the

orthodoxy of using the morphometrics measurement from X-rays was, okay, has to be the bone, has to be the bone, that line has to be on the bone. And then we were talking and, you know, since I’m a moron, I was saying, oh, yeah, like, the ligament here is the pathology, we can, so there are, we should do two different kinds of parameters. One is a hard parameter, just the bone, and one is the soft, including the ligament in the...

Pradeep Chopra, MD (37:34.267)
Right.

Paolo Bolognese (37:57.754)
So that eventually passed around or somebody else had the same idea because it’s not particularly brilliant. So at that point, we started using incorporating, first we were having the two parameters, then on MRIs we just do it incorporating the ligaments in the calculations. So right now, again, clinical, the provocative, the history and

on the MRIs, if there is something obvious, fine from the beginning. Otherwise, we get an MRI in supine, high definition, and we start doing the first set of four parameters. Then the fifth one is the diagnostic trials. Diagnostic trials means let’s have somebody having repeated trials of traction at home or wearing a collar for a certain amount of days at home.

And then if they have a, it is like turning a switch on and off all the time. If you have a consistent help every time you do certain maneuvers and you go back to square one, when you remove the collar or you move the traction, by that point, we have five points. Sometimes all five are positive and sometimes four are positive, one is negative, but you raise a sort of a body of evidence to have a level of suspicion.

But at that point, we don’t finalize the diagnosis at the surgical level, which means for anybody who’s a neurologist or a pain specialist, et cetera, that is enough just to say, okay, you have EDS, you have neuro EDS, and you have a problem with the craniocervical junction. There is nothing wrong with that. But if somebody’s referred to a surgeon, it’s the responsibility of the surgeon to go to the Supreme Court

before you start labeling somebody with that, because as a neurosurgeon, they diagnose this as a heavier connotation. So at that point, we decided to have the test of invasive cervical traction. The beginning was just like, all right, so if the patient does the traction at home, who tells them they’re doing the right way? Sometimes they’re putting this leg like this, or knee like this, sometimes they have a bulled neck, sometimes they have a short mandible.

Paolo Bolognese (40:19.766)
So we just want to do something in the last minute before doing the surgery that confirms indeed that we can see with our eyes. And then we start adding the invasive cervical, sorry, the morphometrics to that. And then we reach the conclusion that of giving scores. So again, first we have the pre-screening by the...

by the local physician or the EDS specialist comes to us. We put those five points together. And then we say, OK, now you qualify. Most likely you have this, but you qualify for verification. So our standard of giving the diagnosis, you have to pass this test. So they come to do the test. And with the test, we give scores. The scores are two. One is clinical. And one is radiologic. 

The clinical is, it does not matter that at that point, we already know that pulling your head up is going to make you feel better. We just want to do it under standard conditions. But what’s important next, since they come into a neurosurgeon, is, fine, you had this diagnosis most likely, but you passed a certain threshold of severity, yes or no. So what we consider the patient...

is having a positive clinical score when the chief complaints improve by at least 75% from the baseline when off traction in the sitting position. So at the end of the test, if they have a test, you know, the chief complaints improve by 80% at that point, they have a severe craniocervical instability, at least with a positive clinical score, then yet in order to have a positive test,

they also have a positive radiological score. The reason is this, as you know very well, there are a lot of people with EDS out there who have no symptoms. They’re excellent ballerinas. They’re phenomenal track and fielders. They get the gold for gymnastics for the US Olympic team. So that’s a demonstration that EDS is also

Pradeep Chopra, MD (42:19.17)
Right.

Paolo Bolognese (42:45.49)
evolutionary mutation, which has brought advantages, but becomes a medical problem when somebody falls off the cliff and thinks they compensate. So the same things which make these people amazing, you know, skin which makes them look younger than what they are until a late age, phenomenally attractive because the skeletal features are those, you know, demigods on Earth, both men and women.

Pradeep Chopra, MD (43:13.509)
Hahaha

Paolo Bolognese (43:14.89)
And athletic performances which are superior because of the way their tissues are, you know, these are the, you know, the captain of the football team and the drum major from, you know, the homecoming queen. Those are the people who are the idols. But if things starting going in their own direction, they become our patients. So, you know, like if you have somebody who’s hypermobile, who cares?

So you can have just a very positive morphometric change of certain parameters. That doesn’t mean you’re unstable. That means that you are hypermobile unless you also have a positive clinical score. So if you have a positive clinical score above a certain 75% of improvement, and you have...

Pradeep Chopra, MD (43:58.925)
Right.

Paolo Bolognese (44:08.954)
movements which are above the normal, but defined normal for the normal people, not normal for EDS. At that point, if you have the two together, at that point, invasive traction is positive and then we move on. And the thing is that if the patient at 35 pounds feels great and they’re happy, they’re kind of joy, recently for the last year or so, when they’re really happy,

since they’re going to go back down and be unhappy and with their brain fog, we remove the traction. When they’re happiest, I give them my phone and I have them calling their relatives. And we take pictures, take videos. So whatever happens, they describe from the inside, they’re always very touching. So like, bye-bye.

Pradeep Chopra, MD (45:02.445)
Is that the is that what is the called the Jesus moment?

Paolo Bolognese (45:08.522)
Yeah, because you can have also the patient that wants to have it, and they come in, whatever, and they tell you the song and the dance like you’ve seen very often in the office. Oh, yes, I have this. Nothing wrong. They can be up at mobility, yes, whatever, but they’re convinced they have kinesthetically instability. These patients are not...

Not necessarily that fakers, sometimes there is the person that for legal implications can have best interest, but some people can just have a kind of their own ambition to rescue themselves. So these people, you go up and they say, how’s the improvement? And they say, yeah, it’s 80% because they heard, you know, the thing, but you see on their face, the people who really have the 80%.

Oh my God, it was not like this for the last five years. Remember, it was this mother who was, she was having phonophobia, so she could not, she was going around with noise canceling things. And she’s in traction at 35 pounds, we removed it, she came with the sunglasses and the noise canceling, we removed everything. She looks in the surgical lights

and we have a boombox we put into the max and she can tolerate it. All of a sudden, she starts crying and she says, I’m so happy now I can listen to music with my daughter. You know, these kind of things you cannot fabricate. On the other hand, the person who’s leading of convenience is going to tell him, yeah, I think it’s 80 percent. You know that they don’t have it even if they want to have it.

Linda Bluestein, MD (46:53.314)
Right.

Right. Can you... I’m sorry. So you gave a couple of examples of clinical signs and symptoms that people are presenting with. And because I think people are probably listening to this and chomping at the bit and going, wait, what are some of the other symptoms that people would be presenting with that you’re looking for that 75 or 80% improvement? Could you share some more of those examples?

Paolo Bolognese (47:00.002)
Good.

Paolo Bolognese (47:24.522)
Yeah, it’s actually, it’s one of those things that you start with is like eating cherries, and then you discover that things are way more complex than you were anticipating. For example, we were having patients that they were coming in and they were having improvement to the symptoms. And at that point, you ask yourself, okay, how much is coming from

there and how much is coming from another mechanism? Okay, for example, you pull your head up, good, great, and you can affect the instability, but can also affect in certain conditions, the jugular outflow coming out to the skull. Okay, so how you demonstrate one for the other. So at that point, we added another piece. Another piece is this.

Linda Bluestein, MD (48:11.366)
Hmm.

Paolo Bolognese (48:23.298)
There are certain signs, neurological signs, which come from the lower part of the brainstem. For example, one is the coordination of swallowing comes from the lower part of the brainstem and involves a number of cranial nerves. And it takes about 14 different movements coordinated in the specific order for stuff to go from the mouth to this orifice and it goes on its own. And...

They’re quite complex. The things which are a little bit counterintuitive is that the swallowing of the solids and the swallowing of the liquids are not the same. And the swallowing of the solids, they tend to be pathologic mostly for mechanical problems. Like you have a cancer in the neck, or you have a hematoma, or simply somebody is doing like this to you. You cannot really swallow a piece of bread.

But is pushing down a piece of solid food is actually relatively an easy task for all the muscles and the nerves. On the other end, swallowing liquids, which will look like, okay, you pour down is easy, is actually the most complicated. It requires a lot of things. It’s the difference of you have, you know, like, one of my grandfathers was an intellectual, the other was a farmer, so I’m going to go with the farmer now. 

It is like you have to go through a gate with one cow or with 25 sheep or 25 cats would be better. So the cow is just beat them on the ass and it’s going to go in the direction of just one. But if you have 25 little animals, it’s going to be the same. So the liquid is going to be more like the 25 cats that really need to be or 25 sheep really need to be

they are active specifically. So, dysphagia for liquids is a typical neurological problem coming from, which can be pinpointed in one of the many things, because if I cut the growth of pharyngeal obviously later on, but everything converges to the lower brainstem. The second thing which is there is breathing. So, automatic breathing and all the regulation thereof, they’re integrated

Paolo Bolognese (50:51.154)
in the brainstem as well in the lower part. Then there is a third one, which is the vision, but not so much vision acuity, but vision coordination. So there is the medial longitudinal fasciculus extends throughout a big chunk, a big section of the brainstem is mostly central and allows us to keep us focused with our two no matter what we’re doing with our neck, no matter what we’re 

doing with our eyes, no matter what the target is doing, no matter if we are in a car and the target is moving on a walking on the sidewalk in the direction. Usually when I want to be s buffoon, I tell the patients, okay, so imagine that you are in the car and you’re driving in this direction, all of a sudden there is Brad Pitt, because most patients are women, and he’s walking in the other. You want to look at him and looking very, very well. And

for that image to be burnt inside your brain. So you don’t want to, you would be disappointed to have a blurry or a jumpy picture. So at that point, the medial and genital fasciculus works in overdrive. So we put these three symptoms at the bottom of our list of the chief complaints. So now we’re going to have two different scenarios. One in which the patient improves in everything. Then the other one in which

you have the chief complaints do not improve, but the brainstem symptoms do, and then the vice versa. So now you realize that you have a different kind of way of understanding. So the first scenario in which everything goes gives you a higher level of confidence that not only the traction is making the chief complaints better, but since it would be

at that point very, very difficult for all these things, including the chief complaints, which should be part of the clinical list of the things which could come from the brainstem. Plus, those three selective things were tested at that point. It becomes more difficult to explain that these things are not coming from the brainstem, they’re actually coming from the brainstem being manipulated by the traction. On the other hand, if the symptoms improve, the chief complaints improve

Paolo Bolognese (53:16.426)
but the brainstem markers don’t, at that point you can have some kind of a level of a doubt of I’m not that convinced that it is coming from there. At that point, you put the brake on before you give the housekeeper seal of approval, of craniocervical instability as told by a neurosurgeon. And now I give you also the...

golden ticket for the surgery if you want. So all these things are observations that you kind of see a number of times, then finally come from unconscious to conscious, and then you build a system around it. Now, it’s still going to be the permutation I’m going to use when three years from now. I do not know, but right now it makes sense to me. Because the major problem is not so much. You have to keep it

even when you are enthusiastic about what you’re doing, and you’re all sold and invested in the disease, you have to be very skeptical for the individual patient. So every time you have to be, I’m not saying in love, but obviously all the three of us and many others, we’re already convinced that neuro-EDS can affect the kinesthetic instability. But it is...

much better for the cause if we are sceptics and dragging our feet, especially if the surgery is a potential end for the individual patient. So again, strategy being optimistic, but tactical level for the individual patient to be the opposite. And that’s what with a kind of strange

Paolo Bolognese (55:14.286)
forced into my head, otherwise I would have been just, yeah, let’s go, we’re not in one, come on, let’s go.

Pradeep Chopra, MD (55:21.349)
So Dr. Bolognese, one of the questions that we as practitioners get a lot is, oh my God, invasive cervical traction, you know, sounds invasive. And can you tell our listeners, like how, like number one, I mean, can you explain to us why you do invasive cervical attraction?

Paolo Bolognese (55:38.529)
Yeah.

Pradeep Chopra, MD (55:51.065)
And that was a very great, good explanation. But they also worry that is it painful? Is it difficult? And how, like, they want to know a little bit more about, is it a painful procedure?

Paolo Bolognese (56:09.846)
Okay, the first thing that I’m going to say is a little bit tongue in cheek, which is this. The best selection is to put the high bar to discourage the faint-hearted. And I’m saying like this, you know, obviously there is a funnel going towards a neurosurgical specialty.

or the other people who are in my field. If we’re seeing every single neuro-EDS with any degree of instability, we wouldn’t do anything else. We wouldn’t have the time to operate on it, sir. So it is good, it is like you’re waiting in line and there are a lot of people who are kind of wasting your time. If you have a patient who really needs surgery, is really sick, if there are 99 patients ahead of them,

to see your surgeon XYZ that they have mild degree of the disease. That’s a delay of care for the poor devil who’s in... So it makes sense for us to have scary things outside of the door. So the people who really have mild degree of the disease, they think twice. On the other hand, the people who are really sick, they’re going to tell you, it doesn't matter that you have to cut my arm, cut my arm. I have enough with this. I’m so sick.

Fine, you get to resist the temptation of cutting the arm to make him happy. But if somebody is squeamish about the idea about having an invasive testing, which is for their own help of avoiding a potentially unnecessary surgery, and they’re squeamish about coming, good, that’s already a selection done of a big chunk of people.

So I do not intentionally, I do not want to downplay the teasing. Oh, it’s not really invasive. It’s just, no, it’s invasive period. You know, you have two pins which are going through the skin in the outer layers of the scalp, take it or leave it. Right. That said, there are other things. One is the fact that we are doing the test and other surgeons were in the field, they’re not doing the test automatically means that

Pradeep Chopra, MD (58:34.97)
Yes.

Paolo Bolognese (58:36.986)
is not that we are right and they’re wrong, or this is the standard or vice versa, this is not the standard, just a waste of time. This makes sense for us at this point of our journey for all the things we have done for the last 23 years, period. That said, in a polite way, it’s either this way or the highway. If you do not want to do the traction,

ends there. So like don’t try to come brow beat me with 500 emails per day. Oh, can I skip the trial of invasive traction and go straight for the surgery? The answer is no. And hey, I respect that. Fine is your decision. The test is elective. So but without a positive test, without not only without doing the test in a positive result of the test. The surgery is never going to be an option

from our part. All right, so that was the thing. Then how was the test done? The patient comes in the operating room and we give him MAC anesthesia and local anesthetic. So MAC anesthesia is like going for a colonoscopy. We are old enough, you know. And the reason why we give MAC is that we don’t want any kind of other medications like

Paolo Bolognese (01:00:04.842)
Fentanyl versus whatever to make the patient better or to make the patient sleepy. We want the patient fully awake when he comes back from probably easily irreversible. And then we also give local. Now we all know that maybe some patients doesn’t know by now but if you use just lidocaine, it does not work. But if you use a mix of lidocaine and marcaine for any kind of procedure, including a dentist, it works much better.

Certain patients who put lidocaine and it is like you’re not given anything at all. And we also use a little touch of epinephrine, unless there are counter indications, because that prolongs the use of the local anesthetic. So by that time, the patient is asleep with local anesthetic and once the secret of any kind of hardware in the body, wherever you put it, internal, external, whatever, is that once you put something attached to a bone...

Pradeep Chopra, MD (01:00:38.875)
Yes.

Paolo Bolognese (01:01:04.182)
and that piece of hardware is fixed and stable, it doesn’t move, you can pull the patient up like this like a pail of water and it’s nothing. It’s like they don’t feel anything. The problem starts if you do not put it loose, if you don’t put it tight enough, it becomes loose at that point in

Paolo Bolognese (01:01:31.978)
automatically how much is too much, how much is too little, it’s there. So that’s kind of routine. Then we wake him up, we just wait for the MAC, for the propofol to wear off. And then we start. About applying the traction, we go up to 35 pounds for cases of a broken neck, any post-traumatic, you go up to 50, 60. Nothing gets broken there. 35 is a safe

level for our patient population to do the things we need to do. Now, there is the issue of the fact that this is not a therapeutic procedure. So if you feel better during the traction, guess what? When we remove the traction, you go back to square one. So if the patient says, oh, I tried it at home, the traction doesn’t work because once I remove it and the symptoms come back, first of all, you should read the instruction we send you.

Pradeep Chopra, MD (01:03:27.717)
Right.

Paolo Bolognese (01:03:39.206)
Is kind of expecting too much from a diagnostic test. It’s like, I tried this test, but cancer came back afterwards. Yeah, that’s not therapy. That’s just doing a biopsy to get some tumor cells out to make a test; doesn’t cure your cancer. So that’s the same kind of reasoning. The issue is that some patients can have rebounds. And actually the rebound,

which some patients say, ah, you know, not only did not work and the symptoms came back, but for the first, even noninvasive traction, my symptoms were worse for a few hours afterwards. That actually is a sign that you are very unstable, that you’re on the right side of the spectrum. So when somebody tells me I tried noninvasive traction, I told them when I had rebound, I already know that the patient is going to be positive on the ICT.

So a rebound after the invasive cervical traction is going to be quite intense, but we keep the patient in the hospital for hours afterwards. So if they are in pain, guess what? You can anesthetize there, they can shoot you with something, something you cannot have in the comfort of your own home. Then there is the overall complications. We do something like between two and four invasive tractions per week. We’ve been doing it for several years. And...

So far, nobody had complications at the pin side, which you do the math is not quite favorable. Nobody had a broken neck as a result of that. And there were two patients who were already scheduled for surgery two weeks later that they rebounds and they already knew they were very severe.

that the rebound was particularly intense and lasted more than a few hours, went into the following day. And so instead of doing the surgery two weeks later, we anticipated to the same hospital state. So that per se was not a complication, was just, you know, a rebound in somebody was severe, it would have happened even with the noninvasive traction. But again, this is an elective test that we do. If somebody is worried,

Paolo Bolognese (01:06:01.199)
Not only I’m not going to strong arm him to come in or try to convince him, I’m actually happy because you’re not that sick after all. There is something strange if you are ready to commit for a surgery, but you’re emotionally uncomfortable about doing an invasive test which is used for selection. It doesn’t make sense, which brings to the next issue that we have developed over the years.

Paolo Bolognese (01:07:30.45)
And back in the days before the MRI was used routinely, there was a five to 10-year delay between the onset of the symptoms and the corrective surgery for the severe cases. The EDS, yes, they have the paranoia coming even worse from the there is nothing wrong with you, the MRI is normal. But their personality is different. These are type A personality, the two of a sudden get frozen. And then once they’re corrected, they bounce back

and you practically have to put the brake on them. Yeah, we are doing too much. So there are actually patients who are very, there’s a lot of satisfaction because you know that, you know that you’re given something and they’re gonna use it. But at the same time, we’re having a kind of a different mix of psychiatric disorders. All right, I’m gonna go to something uncomfortable now, but first I’m making the point. The end point was that

At the beginning, we were asking for a psychiatric screen only on the patients that in our early encounters or whatever on the way to the surgery, either were having some heavy diagnosis on the history, like attempted suicide, et cetera, or they’re having heavy-duty drugs, psych drugs on board, or some heavy-duty diagnosis from before. So at that point, you say, okay, I send you to a unit. We need a psych clearance.

But then I had a few patients who tricked us, uh, few patients who did not tell us their diagnosis or they withhold information or other patients that they acted a part, but then they decompensate at the time of the surgery. And these surgeries are big, so you need the full cooperation. If you cannot have somebody sabotaging their own recovery, because then they’re going to derive, they’re going to be exposed to more

risk of complication. They’re going to have, they’re going to squeeze less out of the entire adventure. So we decided that we need to have a psych clearance on everybody. But we had, we fabricated a new, the current parameters is this, you get the clearance from somebody at home because somebody at home knows you for a long period of time, but they know jack shit about what

Paolo Bolognese (01:09:59.29)
EDS and the surgery is about. But the guy at home who knows you needs to tell us, okay, is the patient optimized? Is the patient reliable in telling us the symptoms and their intensities? The patient has a support system at home. Is the patient resilient enough, can do all these things? And then there is a second independent screening, psych screening with us, with our neuropsychologist.  

Pradeep Chopra, MD (01:10:11.739)
Right.

Paolo Bolognese (01:10:27.89)
Our neuro-psych doesn’t know these people from before, because the encounter is just once, but knows very well two things. Number one, what the neuro-EDS constellation implies in terms of psych evaluation. And number two, what the surgery is about and what’s ahead. So he is gonna be the guy that pushes back and kind of pokes him inside just to see, okay, you know, I kind of challenge him, but you’re really sure?

Yeah, you know, like, it is all is. It’s not that he’s a game. It’s just because I say right now it’s going to happen. But he’s all in, you know, just for the patient to have one last really moment of insight. Because sometimes they’re so invested, oh, I’m going to see a neurosurgeon. I’m going to have the surgery, I’m going to be good. But that is just like, okay, that little shaking that somebody needs.

You know, like, and we don’t do it before the surgery. We do it at the time of the invasive cervical traction. Because if you’re coming, let’s assume, like we did, for example, several years ago, we were having the psych clearance two days prior to the surgery. At that point, it’s a kind of a loaded circumstance. The patient is already involved, the insurance is approved, you know, the machinery is going, you know.

Pradeep Chopra, MD (01:11:32.602)
Yes.

Paolo Bolognese (01:11:54.966)
It’s a different kind of psychological pressure. On the other end, when they come for the invasive traction, they don’t have a clear cut, clear cut. They don't have a confirmed diagnosis yet. And they don’t even know if they’re going to be a surgical candidate or not because they don’t know what the score is.

All right. What we notice, yeah, good.

Pradeep Chopra, MD (01:12:14.073)
So Dr. Bolognese, I had another question, I’m sorry. Firstly, your neuropsychologist is amazing. A lot of the patients that I have sent, who have seen him, actually want to keep them as their psychologist.  So he’s, I can't remember his name right now, but they love him. He’s great. And he’s very...

Paolo Bolognese (01:12:32.782)
Yeah.

Paolo Bolognese (01:12:38.966)
Klein. Yep.

Pradeep Chopra, MD (01:12:45.617)
Like you said, he helps them understand exactly what they are going to go through. And a lot of them feel like, yeah, this is the first time they’ve met somebody who really understands. My question was that we are spread apart. Like, Dr. Bluestein is in Colorado, I am in Rhode Island, and there are other physicians everywhere. And you are in New York City.

And you have something called the second opinion report, which I have read many, many times. And I think it is phenomenal. It is a really good report. So can you tell our listeners?

Paolo Bolognese (01:13:31.654)
Yeah, I tell you a secret. Some of the things that they report, how they structure, actually I copy from you.

Linda Bluestein, MD (01:13:39.229)
Ha ha ha ha 

Paolo Bolognese (01:13:42.05)
So that’s why I like it so much.

Pradeep Chopra, MD (01:13:44.473)
Well, so for patients who live in far off places, as you know, New York is an expensive city to come to. So the second opinion report really helps them understand what’s going on with them and what to expect and what should be the next steps in the diagnosis. Can you tell us a little bit more about that?

Paolo Bolognese (01:14:09.462)
Yeah, it is a good idea, but obviously, yeah, it’s actually coming from something coming from COVID legislation. Telemedicine, as you know, is something that is relatively recent, but since more than 90% of our patients since 2001 were from outside the tri-state area, it was kind of

problematic to ask the patient, okay, come the first time, and then you do this testing, and then you come a second time, and then finally I’m going to give you a diagnosis. Before you know, it is at least two trips, and two trips around country by somebody who’s sick, they need to come for with their companion who has to take time off. It gets complicated. So in 2004, we’re looking into telemedicine with our health system back then, and there was no legislation nationwide.

Actually, one state, Texas, was back then was prohibiting any kind of doctor to call on the phone from outside Texas because they want their patient to be poached. So our health system said, you know, good, you guys want to do telemedicine, guess what? You cannot do it. So it took several years afterwards, and then we were allowed to do telemedicine, you know, to have this. Then COVID came.

And back then, really, there was no legislation was kind of, there were not so many people doing it. But then COVID came and it started becoming everybody did it. And with that, the legislation became more precise. So when you see somebody with that, there are specific things and things about licensing, et cetera. So at that point, we had

a decision to make. A decision to make was, do we go back to the model before in which the patients come multiple times, or we do something similar? And by that point, in some institutions, and Stanford, I guess, was one of the first, they did the second opinion model. Second opinion model means you send, they did something structured, which was already up in the air with other names. But you send in your

Paolo Bolognese (01:16:36.938)
your clinical information gets reviewed by a committee, then a junior attending or the poor guy at the bottom of the barrel has to become the scribe and then sends it back to the patient. And that’s it. So we saw some value in that. And the value is this. Instead of we see you in the office and then we tell you this is the work you need to do or whatever, we front load everything. And

We say, okay, these are the things you had to do before. Once everything is in, once you fill up our, you know, long, long multi-page clinical essay, once you’re gonna have done with this and you enter the history and blah, then if you see other specialists good at their letters too, you know, I know that when I have one of your letters, I can just kick my feet up because you already done everything. I just...

The only thing I had to say is the following diagnosis have been suspected by Dr. Chopra, and I fully agree. So that’s an easy thing. But anyway, so at the end of that letter, what we can say is, OK, we have suspicions about this and the clinical, historical, provocative testing, radiological, and things we’ve done so far, because they already give us in a

we already told them the homework, raise suspicion, a critical mass of suspicion for the following diagnosis for which we need additional testing. These are additional testing. We tell them, okay, so you need this, and this. And if you want us to confirm it, you come here for invasive testing. So at that point, the first encounter is done with us. And we’re already, you know, three...

three, four steps ahead with what had been done in the past. In the past, where they've reached that thing like after six or nine months, after two or three visits with these patients, poor guys going back and forth. So the second opinion for sure, you know, avoids that. But then we figured out another thing was that a lot of patients, since, you know, we didn’t make it so expensive, a lot of patients were just curious and not particularly sick, they were writing in.

Paolo Bolognese (01:19:04.258)
So at that point we needed somebody to do a screening to separate the patients who were not very severe, not to go to the surgeon, but to go to the other components of our team who were just for the conservative part, because obviously don’t throw surgery to everybody, anybody. But at least to prevent the people who were sick to have a long line, 50% of which was made by patients who were just mildly sick.

Obviously, we’re still refining the process, but so far it’s working pretty well. Yeah, because at the end of all that, when the patient comes in the hospital, all the muscle part, your notes are as long as mine. And it takes a long, long time to put all the things. So when they come into the hospital, it is, okay, all this is already in the bank, it’s already worked out. Now we just need to...

Pradeep Chopra, MD (01:19:35.75)
Right.

Paolo Bolognese (01:20:03.318)
check on these three, four items. And everything else has already been digested, they already read it, whatever. So now we focus on this. So the encounter in the hospital doesn’t become less sort of a meandering ... I already talk too much myself, but it doesn’t become like a meandering adventure with a lot of loose ends that then can never be tied up. So everything becomes more effective.

Pradeep Chopra, MD (01:20:29.241)
So one of the, so now I think we’ve, what we understand is, and I’m just summarizing it for the readers, listeners is that the first thing you do is you ask them to fill out some forms, upload their images, radiological images. And then based on that, you come up with a suspicion of the following diagnosis. And then if the patient has whatever,

Paolo Bolognese (01:21:07.158)
Then actually, then actually depends if the patient has mild symptoms. For example, you can have a monster Chiari I malformation on the radiology, but they have mild symptoms in a very good quality of life at that point. There is no need for them to come here for further invasive testing. And sub there and say, okay, uh, bye. And, uh, in two years do another MRI and touch base with me, but

on the other hand, you have somebody with the craniocervical instability, severe compromised quality of life. That point the plan is going to be, if you’re interested to go further, come here, we’re going to do the invasive testing and that, you know, we kick it up a couple of notches.

Pradeep Chopra, MD (01:21:56.077)
OK, so once they get that second opinion report, and if you feel that they need to come and see you because they are clinically very symptomatic, and their data shows.

Paolo Bolognese (01:22:10.482)
is just going to be directly for invasive testing in an hospital setting. So it’s not going to be like, I see you in the office and you’re going to come back. You just come here, we do, and it’s not only the invasive traction. For example, some people have suspicion of disorder of the intracranial pressure. We’re going to do an intracranial pressure monitor with positional testing. Or there is a problem with the glossopharyngeal nerve. We’re going to do glossopharyngeal block.

Pradeep Chopra, MD (01:22:13.472)
Right.

Pradeep Chopra, MD (01:22:20.174)
And right.

Paolo Bolognese (01:22:40.218)
or there is a suspicion of neurogenic bladder, we’re going to do urodynamics. So again, this is you, since we are more aggressive with our toolkit, the responsibility of the diagnosis has to go on a stronger and more objective level. So invasive testing is kind of the highest level of...standard diagnostic standard we can apply.

Pradeep Chopra, MD (01:23:15.281)
So that reminds me about one condition that you treat, and you treated very well, called Eagle’s syndrome. Can you tell our listeners what are the symptoms of Eagle’s syndrome?

Paolo Bolognese (01:23:32.028)
All right.

Paolo Bolognese (01:23:35.638)
There is a bone at the base of the skull, which is converging from below the ears, which is converging towards the voice box, and it’s called the styloid process. And it’s usually a small and thin piece of bone, and it is the attachment for a number of about three tendons, which are attached to muscles which go to the voice box. And they’re held forward with a number of tendons.

a number of movements that we routinely do.

Paolo Bolognese (01:24:09.818)
The Eagle’s syndrome is when you, this bone becomes very big, very cumbersome, very long, and in doing that creates a number of different things. In the past, it was called Eagle’s syndrome only when it was poking the trachea and or the esophagus. All right. That was called the... Then they found out that sometimes other vessels can...

Passes passing by like the jugular or the carotid can be impinged. So that was called the vascular Eagle’s. Then I was, you know, I was very excited because, oh yeah, these patients, they have, they have a lot of symptoms who come from the glossopharyngeal nerve. So I was ready to publish it when finally the ENT figured it out after years that they were debating it. And so they kind of beat me to the punch, but there are a number of cranial nerves passing by

that get stretched, pushed, or nudged by these bones, because they are in the right place, and so this bone, but this bone is bigger, is kind of pushing and shoving. Now, like everything which is related to EDS, people with EDS styloids, they’re like the princess and the pea. They’re super sensitive for everything. So the majority of the people

They don’t care that they have a styloid which is a little bit bigger or a little bit thicker. And so the major, the longest series about Eagle’s are actually coming from the Indian subcontinent because for some kind of reasons over there, the, the styloids are enormous when they, when they’re pathologic. And, yep. The largest series that come from there.

Pradeep Chopra, MD (01:25:55.093)
Indian like me? Really?

Paolo Bolognese (01:26:03.594)
Yeah, the largest also because of 1.3 billion people. But they’re really phenomenally big. So there is also kind of a genetic component to that. People with EDS is a different thing. Like I said before, the clinical presentation is mostly affecting, mostly functional of the glossopharyngeal nerve.

Paolo Bolognese (01:26:35.535)
the first tract of the styloid. So if it becomes thicker and just passes by, that’s the little extra stretch. You know, our very touchy-feely, EDS-people, it’s enough to create trouble. And now comes the issue, because the glossopharyngeal nerve is very complex. And if I tell somebody, okay, you know, I go to the doctor and say, okay, I have palpitations.

And I have a ringing in the ear and I have a sensation of metal taste in my mouth. This guy’s going to say, you’re freaking crazy. And instead those things come from three branches of the center, the glossopharyngeal nerve. So if you understand, if you remember medical school and neuroanatomy, that’s easy, but apparently my ENT is calling. They, it took him a while to figure that out. So what you do is this.

Pradeep Chopra, MD (01:27:24.474)
Right.

Paolo Bolognese (01:27:33.522)
like is the same principle when you go to the dentist and you have a big problem with your tooth and the dentist gives the local, you feel better. So if the glossopharyngeal nerve is crying uncle by because it’s pushed by the styloid, you just inject some local anesthetic in the neighborhood. And if the symptoms

calm down, that means they’re linked to the glossopharyngeal and therefore they’re linked to, it’s because the glossopharyngeal is getting kicked around by this bone. Now comes the tricky part. I remember the association between Chiari and styloid, hypertrophic styloids, and EDS and hypertrophic styloids, we figured out a long time ago. That was not the issue.

Since we were not trained as neurosurgeons to do these cases, we’re trained about other things, we’re sending these patients, a few in between, to the ENTs. And I remember that one time I sent a patient to an ENT with my health system, like several hospitals ago. And this patient was 2005. The patient came back with a tracheostomy and a gastrostomy tube.

Pradeep Chopra, MD (01:29:02.661)
Ooh.

Paolo Bolognese (01:29:04.106)
Yeah, I know. And obviously, at that point, we became more prudent about saying it, because I thought, OK, this is a dangerous surgery. I did not know about the, like, all right, before I send another one, I’m going to wait for somebody to be at least as sick as this one, if not more. So we sat on hundreds of these patients over the years, Chiari and EDS, until one day, it’s all right. This patient will really have it.

big star of it, and severe EDS, and a craniocervical fusion. Actually, there were two at the same time, and we booked them something like if, in two consecutive days, and they knew each other, and they were calling themselves like the, like the, from Dr. Seuss, they’re calling themselves Thing One and Thing Two. Just like, whatever. And so they were having, they were having craniocervical fusions.

Paolo Bolognese (01:30:03.35)
So that was raising some prompts about the intubation. So I said, all right, I’m gonna do it myself. I don’t wanna send you somewhere else. So I go to the library and the days before, and I go through textbooks of head and neck surgery, again, different specialty. And I go through all that, whatever, take my notes to the operating room that day, and I start step one, you put the patient on the table. Step two, you turn the...

I can’t turn anything because the patient was fused. So at that point, they threw the notes away and I realized that the surgery had to be done completely different. So a large patient population of the with Eagle’s, they had EDS and former fusions. That creates an enormous technical problem because it is like you’re trying to change the, an engine in a car.

but you open the hood just like this. And not only, but most of the dissection instead of doing with tools, I do it underneath the mandible, blind with my finger, and then I dislocate the thing and then you can go on. So practically we had to adapt the surgery to the circumstances with maneuvers that were not in the book. And I don’t think that I realized afterwards is by that point,

Pradeep Chopra, MD (01:31:02.586)
Right.

Paolo Bolognese (01:31:28.962)
I already had, you know, a number of years of experience with EDS patients. So I knew that they were not tolerating a lot of dissection nonsense. So I realized what happened to the ENT in the past. The ENT did the surgery like the ENTs do using the bovie knife, which is the electrocautery, the fire stick, we call it jargon, and the, you cannot do that to get around nerves in general.

Pradeep Chopra, MD (01:31:49.894)
Right.

Paolo Bolognese (01:31:59.158)
But nerves of an EDS, that’s the case with that. So at that point, once I pass the skin, I don’t use any cautery at all. So it’s all blunt dissection, and nothing else. So it was, so far, it has worked, whatever. But that doesn’t mean that complications are never going to happen. But that’s an example about how to adapt the.

Pradeep Chopra, MD (01:32:09.753)
Makes sense.

Paolo Bolognese (01:32:25.622)
how to adapt the technique to a specific subset of patients that are kind of strange and different. About the diagnostics is, okay, you know, we know the problem now. It’s obvious when everything is normal, it’s always when everything is like textbook enormous. And the finesse is where do you, where in the shades of gray you draw the light. So right now the thing is, we do a 3D CT

because it shows us the structure, the length, and thickness of the styloid. It has to be a CT angiogram because then we see how the especially the jugular passes by and the carotid passes by and how they’re affected in 3D. And then we do a glossopharyngeal test. But again, before we get to that level, there is clinical. We have a series of

all the symptoms which are linked to the glossopharyngeal nerve, check, check. Then there is the provocative test. You push here and the patient suddenly jumps or you put inside the mouth and they jump. That means that nerve is hurting. The same way do you do this to a tooth with decay. The third one is the history. So, you know, the

Paolo Bolognese (01:33:52.802)
patient had with progressively those symptoms got worse and, very often happens after a tooth extraction or other things. So there are certain kind of patterns we keep seeing. Then there is the fourth, which is the radiological part. The tier for once is self-explanatory. And then the fifth, which is the glossopharyngeal block for the provocative test, invasive. And if

whatever symptoms improve, at that point, we can link him for sure to the glossopharyngeal nerve, and we can go ahead in chopping up that thing. Now, we do one at a time and separate it by two months. So in case there is a problem or a complication, you’re not gonna have somebody with tracheostomy and a bag again, like it happened to that colleague.

Pradeep Chopra, MD (01:34:55.161)
Right. So we talked about craniocervical instability. We talked about Eagle’s syndrome. We also want to talk to your other expertise as in Chiari malformation. And you mentioned cranial settling. And to my understanding is that cranial settling is a very big issue in this population.

Pradeep Chopra, MD (01:35:24.653)
How would you want your patients, what kind of imaging do you want in patients for Chiari malformations to show, like can we do a supine MRI? Do we need an upright MRI?

Paolo Bolognese (01:35:47.598)
Okay, first of all, cranial settling is an old timey term for what in a simpler way is the vertical dimension of craniocervical instability. So you can have a rotational component, you can have an anterior posterior component, like for example, C1-2 instability pure. The rotational component would be the Bowhunter syndrome which people do like this and the vertebra gets choked.  

And then there is the vertical. Going back to the morphometrics, basion dens interval, the normal distance between the base of the skull and the top of C2 should be between 4 and 5 millimeters in supine. And when you go sitting, you should just settle by a millimeter, millimeter and a half tops. If you extract the head like this, should go up one millimeter and a half tops.

So that is the normal range. Now, people with EDS, if they are hypermobile, they can have much more than that without symptoms. That’s hypermobility. But if they become sick because of it, that point becomes instability. Hypermobility plus symptoms equals instability. So you have the sense that something is wrong when in the supine position, on a high definition MRI,

two or three millimeters. That’s already an indication that maybe that patient has cranial settling, vertical instability. The second thing is that when we do the traction, we pull up, if you have a movement, a difference between off traction and on traction of two millimeters or more, that’s vertical instability, cranial settling.

That’s actually one of the surgical qualification criteria. And so cranial settling can happen in many different things. Traditionally in neurosurgery is described in pathologists which destroy the joint, like inflammatory things like rheumatoid arthritis or tumors in the area, or traumas which, you know, vertical traumas which break down the condyles.

Paolo Bolognese (01:38:13.706)
That’s a cranial, classical cranial settling, but our patient, the viscosity of the joint is totally altered. So it is like you have a cylinder inside.

You have a piston inside the cylinder. You know, they go up and down, but there is oil and they do not go from one to the other. All of a sudden you see the, boom, falls down, okay? And then if you had the explosion, poof, it goes up, it goes out to the wood, out to the hood of the car and it jumps in the street. That is abnormal, okay? It collapses too much and expands too much. So that is craniocervical instability in car terms.

Pradeep Chopra, MD (01:38:37.367)
Right.

Paolo Bolognese (01:38:59.478)
Now, different doctors have their own things. Generally, what I start with is the MRI in supine of 3 Tesla at least, but 3 Tesla is fine. 1.5 is acceptable, but 3 nowadays is very easy to be found anywhere. Supine position, nothing else. Because we had the best definition of the joint.

Once you start going for an upright MRI, upright MRIs by definition have a very small magnet, which is 0.5. I actually know very well the guy who invented the upright MRI was one of the very first inventors of the MRI principle. And actually lives nearby here, and he was together with us in our old university. But the story is. 

Although it's a very good and helpful thing, you'll take a good picture. And all of a sudden, all those morphometric measurements, the execution of which rely on crisp definition of bone versus ligament gets blurred out. Number one. Number two is if you send somebody and do the flexion and extension, it is not standardized.

Pradeep Chopra, MD (01:40:16.165)
Right.

Paolo Bolognese (01:40:26.338)
Because if that day that is Mr. Smith, the technician who is nervous about doing it, is going to say, okay, move a little bit, move a little bit, I’m nervous because you’re sick. Other times, if you can have a flexion, there are two different kinds of flexion. If you have a flexion like this, the stress of the flexion is in the lower part of the cervical spine. Okay? If I do like this, the stress is on the higher part of the cervical spine all the way to the junction.

So you can have two people doing a major effort, but stressing two different parts of the spine simply because the technician is used to that. The majority of technicians who do MRI Flex-Ex (flexion-extension) are to test degenerative joint disease in the mid-lower cervical spine. So already something strange. But the thing which is the most

absurd, but then you think about it, it makes sense, is if you have somebody who’s hypermobile from EDS, but zero symptoms, they’re going to give you these scary contortionist levels of flexion and extension, especially in extension. They’re absurd, but they can do it because they have zero symptoms. On the other hand, the patient on the opposite side of the spectrum who’s super sick, he would rather get shot.

than move even just a little bit. So all of a sudden, what is this upright flex-ex MRI, even if we get standardized, gets you. I know. Becomes helpful from, so if it’s a screening is not that great, it has all these pitfalls. But four specific things can be helpful. For example, you have.

You want to test if there is a gliding, an excessive gliding between two and three and one and two. Fine. At that point, you have a specific thing and you use that like as a sniper rifle. But as a screening tool, also realistically, the MRI is not going to authorize 500 tests and especially 500 MRIs. So if they authorize one MRI of the cervical spine, then these patients very often need an MRI of the lumbar.

Pradeep Chopra, MD (01:42:33.922)
Right.

Paolo Bolognese (01:42:52.986)
And sometimes they have problems inside the head. Before you know it, they have MRI lumbar and cervical, for sure. You also had the MRI Flex-EX and MRI upright. They’re going to pick one. And if you pick up an MRI upright, at that point, you’re going to have a crappy MRI of the cervical spine of the area that you really want to see with data that are not really going to be standardized. So that’s why I start with the MRI cervical supine high definition.

because I can always order the other one later in small amounts for a specific subset of problems. But if you use it as a screening, you shoot yourself in the foot. Because then if you really need a good picture, you have to wait until the insurance tells you yes.

Pradeep Chopra, MD (01:43:31.863)
Yes.

Pradeep Chopra, MD (01:43:42.533)
So for patients with Chiari malformation, what kind of imaging do you want to see?

Paolo Bolognese (01:43:47.842)
Yeah, that’s okay. In the past, people were saying, okay, I’m gonna get an MRI of the brain because of the disease of the brain. That’s actually, if you have one shot and one MRI, that’s not the best MRI to do. Best MRI to do is an MRI supine high definition of the cervical spine. Because MRI of the cervical spine, classically done, shows you the entire posterior fossa and the entire cervical spine. And about...

Paolo Bolognese (01:44:17.982)
50% of the Chiari I malformation have an associated syringomyelia cavity. So that single test puts you ahead of the game already. So that’s a good way to start. Then, obviously, you want once you have that screening, you pass it to a neurosurgeon, and they’re going to do other things. Like in the past, I remember when

first came out in the late 90s, there was cine MRI. First came out, we were excited. Oh, fine, we’re gonna see the cerebrospinal fluid (CSF) flow. Fast forward 10 years, and all the experts, while the other people were speaking it up, all the experts dropped it because they were saying, the cine MRI, the vast majority of the times, doesn’t tell me something I do not know already. If I have a patient with visible herniation and the patient is sick like a dog and the symptoms are coming from there.

Guess what? 90 plus times I’m going to do the cine MRI is going to show me that there is some blockage posteriorly. Big, big, damn surprise. I already knew before. On the other hand, the cine MRI can be helpful if you have an herniation that is not particularly, you know, vertically is not that deep. There is a discrepancy between the symptoms which are Chiari-like and very strong and the appearance of the MRI that looks a little bit

not vanilla, but less than what you would expect. At that point, you use the cine MRI as a tiebreaker.

Pradeep Chopra, MD (01:45:53.125)
So from what I understand is that you’re looking for a high definition 3-Tesla supine MRI of the neck in cases of Chiari I malformation.

Paolo Bolognese (01:46:06.339)
Yeah, 1.5 is acceptable, yeah.

which is the same image that I need to investigate craniocervical instability. So everything wrong from here to here, MRI cervical supine 3.0.

Pradeep Chopra, MD (01:46:17.454)
Right, exactly.

Pradeep Chopra, MD (01:46:25.085)
Now, a lot of these patients with Chiari malformation also present with intracranial hypertension.

Paolo Bolognese (01:46:32.382)
Yeah, that’s a good, that’s a classic thing of the chicken and the egg. Uh, first of all, we, we had to talk about semantics and there are a lot of actually people in the field that they’re very loose with the semantics and they create confusion for themselves and for the patients. Um, so the thing out there is that, uh, many

Paolo Bolognese (01:47:02.774)
many people, and wrongly so, they see the cerebellar tonsils coming down and out of the skull where they belong inside the skull. And they say, that’s a Chiari I malformation. That’s bullshit. Okay? The herniation of the tonsil is just the anatomical effect of 18 different mechanisms, which can be summarized in four.

I can push the tonsils out of the skull. I can squeeze the tonsils out of the skull. I can pull them down from below or they can dangle like the cheeks of a bulldog. The classic key area in the formation is the purest. Is the tonsil herniation caused by a volumetrically small posterior fossa? Is squeezing a zit?

The posterior fossa is a craniosynostosis of the posterior fossa, the Chiari-I malformation, and then herniation of the tonsils is its effect. So it’s disorder of the scalp, which is squeezing out the brain. So squeeze is Chiari. There is also another, you know, you can have syndromic Chiari, you can have other forms of craniosynostosis, you can have a pinhead, opposite, Chiari is gonna come down. Then you can push. If you have a tumor in the posterior fossa,

is going to push all along, including the tonsils for the path of least resistance outside of the hole, which is the bottom of the skull. Big hole in Latin means foramen magnum. So you push them down simply because the posterior fossa, the skull over here, is normal in size. But there is a big fat guy who’s pushing you around. So we have the squeeze, and we have the push. We have something pulling down. Like if you have.

Severe forms of tethered cord, like you can have a Chiari II malformation, et cetera, or also tight filum terminale which is very tight, can displace the tonsils downwards in different degrees. So Chiari II malformation, lipomyelomeningocele can actually pull the spinal cord down to the pelvis, to the sacrum, at a very young age. And then the brain, not only the tonsils, but a part of the cerebellum, leave the scalp.

Paolo Bolognese (01:49:27.39)
and going to the spine. And other times, people with tight filum terminale, they can actually have a minimal displacement. Now comes the confusion. There are a lot of some of my colleagues to make a thing more complicated called Chiari 0, Chiari 0.5, Chiari 0.75. For God’s sake. Then there are the five-millimeter rule, which was decided by a neuro radiologist who never seen patients and couldn’t know if the patient has symptoms or not.

He decided the Chiari malformation was just with the tonsils with 5 millimeter or more. But if 4.75 was not Chiari, what kind of bullshit is that? So whatever. So practically, you can pull them down. Because if you pull down the spinal cord and the brainstem, the brainstem is attached with three prongs. So the cerebellum is attached to three prongs to the brainstem. And again, it comes down. The first piece of the cerebellum coming down are the tonsils. So we push, squeeze.

and pull and that is the dangle. You know, people with EDS saggy things, saggy here, saggy there, the uterine prolapse, you know, so it’s not a surprise that something can also sag. Now, the gravity of the nervous system is not gravity for the all the rest because the nervous system is bathing in cerebrospinal fluid, so there is this kind of thing which is buoyant, but is more like the boat.

which is moored to the dock. So you’re not a boat in the middle of the ocean. You’re moored to the dock and you’ll be like there and somebody would see it with EDS, you’re expecting some degree of sagging. The typical tonsils should be three millimeters above the foramen magnum, plus, you have one nose, I have a bigger nose, she has a different cuter nose, you know, like so comes for the tonsils. So there is a...

It’s not that everybody’s three millimeter. Some people, they are above. Some people, they’re below. So there is a spectrum. And unfortunately, this spectrum of normal overlaps slightly with the spectrum of abnormal. So wait a minute. But without going for an hour, half an hour on this thing, that’s why there are the experts out there. But unfortunately, a lot of the confusion comes from the fact that when you start calling Chiari, everything, regardless of the...

Paolo Bolognese (01:51:55.31)
because you’re creating confusion. So, Chiari actually started being described by this guy who was a pathologist and was actually, geographically was Italian, but at the time he was living in the Austrian Empire and he was the pathologist in Prague. And, you know, back then, what was the major pathology for the end of the 19th century?

TB, tuberculosis. So he was doing autopsies on people who had TB. These people were having hydrocephalus secondary to TB infection. And that was the first radiation you saw was actually the push level, the squeeze level, because the hydro was pushing things down. Then he started seeing afterwards. But he was doing a, it was a pathology-based and anatomically-based description.

Paolo Bolognese (01:52:54.97)
Afterwards became an MRI based description, but the best ground to stand is a pathophysiology mechanism based because if you understand the mechanism, understanding what you have to do to fix it is easy. So if somebody has a Chiari malformation because he’s herniating from the squeeze, you have to do a Chiari surgery. But if it is coming from a push.

At that point, you’re barking up the wrong tree, which brings me to one of the questions that I didn’t answer before, which was the intracranial hypertension. That’s a chicken and the egg situation. In some cases, you can have the squeeze level, herniation going down, that’s typical Chiari, with mild elevation of the pressure, which normalizes after the decompression.

bingo, end. On the opposite end, you can have a patient with pseudotumor cerebri in which the pressure inside the head is very high, uniformly. The skull in the back is normal in shape. And that’s a push situation. So the pseudotumor is causing the herniation. Fine, there are some anatomical details which are different, but that’s the principle. And then there are situations in which you have both.

You have the Chiari, you do the decompression, the patient still has high pressure if you do an intracranial pressure monitoring. So in this way, the patient has one plus one makes three situation. So in order to, again, go to physiology, you need to treat both for the patient to have a full recovery. So in the past, we were called pseudo tumor because he was

They didn’t have CAT scans and the people were opening the skull of people looking for a tumor and there was none. And then it was thought that it was a cerebral spinal fluid disorder because they were doing the lumbar puncture and CSF was spraying out of the needle. Then it was understood afterwards that the problem was not the CSF, the problem was not something else, it was tumor. The problem was a swollen brain. But the swollen brain from what?

Paolo Bolognese (01:55:19.17)
Fast forward several years, it was swollen brain because the venous circulation was choked up, which is easy because I choked myself like this. My face was gonna become purple, imagine to the brain, which has a lot more of dealing with a lot more blood flow. And there are only two ways out, which are the two jugular veins. 

Paolo Bolognese (01:55:40.802)
Fast forward to last year and to actually to a few years ago. And pretty much at the same time, Dr. Higgins from Cambridge and myself and our guy, we kind of stumbled on a number of patients. I was coming from a different direction. We’re looking at styloids. And next to the styloid was the tubercle of C1. And that was very often I was seeing the jugular sandwiched between the two.

So I was looking, I was removing the styloid and then sometimes I was seeing the jugular re-expanding and the pressure normalizes. So wow, that’s great. On the other hand, Higgins at Cambridge was looking in a different direction. He was looking at C1 and he was seeing the C1 like a starfish around the rock, doing like this around the tubercle. So long story short, coming from different direction, we...

hit the same thing with the compression of the jugular, either in the sandwich or unilaterally at the level of C1. And then we started noticing other things. People with EDS, especially EDS with cranial settling, imagine that you have a burger, OK? And you push it like this. What does the burger do? So patients with EDS very often, the ring of C1 in chronic settling,

becomes wider and it becoming wider, the peduncle, which are like, imagine that I am like this and I keep my elbows, my elbows are going to go more out and they’re going to go towards the jugular. By doing that creates a, it is the equivalent to me putting my foot over a garden hose and creates a venous congestion inside the brain and the pressure goes up. So last year I started, you know, shaving.

since I’m there for other reasons, like I’m there for Chiari or I’m there for fusions, I just go a little bit more lateral and I can reach those bones and I cut them. All of a sudden, patients that were problem patients for a while for me, and I put the shunt today and I put another shunt three months later, keeps failing. All of a sudden, the pressure normalizes and I felt stupid. There was one patient, one of the, yeah, again, one of those.

Paolo Bolognese (01:58:06.03)
the sheep who got lost. It was this patient who was suicidal when we first met her in 2001, because she was having extremely loud tinnitus. Actually, she had two tinnitus at the same time. One was high-pitched, and one was sort of a machinery low-pitched. Constant, she couldn’t sleep. It was like...

It was like living in a factory constantly. And she was ready to put an end to life. And she came, we did lumbar puncture. Pressure was very high. The symptoms improved to the point it was tolerable. So we put the shunt. And pseudo tumor, the ventricles are very small. So the failure rate is very high. So we were doing something like one shunt every year. She was, and then we tried different shunt configuration, different shunt valves, the technology got better, but the headache was better, the ringing was better, but was never zero. And then, you know, after she was one of the first patients that I chopped those tubercles in the ring; for the first time in her entire life was gone.

Pradeep Chopra, MD (01:59:29.058)
Wow.

Paolo Bolognese (01:59:29.714)
It took me 24 years, no 23. Now you kind of feel stupid afterwards. So you say like, it was there, like why I didn’t know this before. But again, you do not know what you do not know.

Pradeep Chopra, MD (01:59:44.181)
Exactly. And you thought about it. So that brings me to one more question. And I don’t know, Dr. Bluestein, do we still have time?

Linda Bluestein, MD (01:59:53.297)
So why don’t you ask your question, then I have a question and then we’ll wrap up.

Pradeep Chopra, MD (01:59:57.453)
Okay, we have a subset of patients with EDS that start to have dizziness when they turn their head to one side.

Pradeep Chopra, MD (02:00:14.208)
What is that?

Paolo Bolognese (02:00:16.206)
All right. And that’s another good thing because there were some things I did not know either. One of the... One is easy to find and one is called the bow hunter’s syndrome. Bow hunter because you’re a hunter, use the bow. And the two vertebral arteries pass inside C1 and they’re passing inside the hole. And then...

Pradeep Chopra, MD (02:00:34.711)
Don’t you it?

Paolo Bolognese (02:00:44.002)
they also pass inside a hole at the level of C2. The bow hunter’s syndrome, because of the hypermobility and/or the configuration of C2, because it all depends how much redundant, you know, if C2 in between the two holes has a lot of slack and you rotate a lot, the slack can tolerate for a while. But if you don’t have a lot of slack and you overturn,

At that point, you’re going to create a kink in the tube. Let me just show you something like this. It’s like this. You rotate, and all of a sudden, the rotating gets like this. Or imagine that it’s super tense. The moment it gets super tense, at the level of the two holes, create a kink, create a stenosis. And that causes hypoperfusion from the vertebral artery. What’s the correction? The only thing you have to do is just to tell.

You know, doctor, it hurts when I do this, don’t do it anymore. So you just fuse C1 and C2 so the relative architecture of the two preserves the integrity of the vertebra. So that is one. Then there are some other patients that remember the styloid et cetera, that in their three-dimensional arrangement can actually kink.

Pradeep Chopra, MD (02:01:55.717)
That’s the end of it.

Paolo Bolognese (02:02:11.306)
most of the times the styloids when they’re hypertrophied, bilateral, they can actually choke both jugular. At that point, choking both jugular create an intermittent sedatour. Okay? Then there are some other patients that when they turn,

they can have a more complex structure in which some different cranial nerves are involved. And at that point, you can have the 10th or the 10th and 9th, they’re functionally connected. So you can use one as a kind of a vector for the other, but that’s a different story. But you can have not the artery, not the vein, but the cranial nerves being affected.

Pradeep Chopra, MD (02:02:58.057)
nerve okay got it

Paolo Bolognese (02:03:00.31)
But at the end, it’s a stupid thing. It is something mechanic that is affecting something else.

Linda Bluestein, MD (02:03:09.569)
And that leads me to my question, which was.

Hopefully people by now are realizing that there’s a lot that happens in the neck, the base of the brain, and so these workups are very complex and I think in the beginning when you were explaining about patient selection and invasive cervical traction and how important that is I think most people don’t realize that you don’t want to have a surgery that you don’t need -- like I think some people think oh that seems like an easy fix or if that’s the definitive fix for CCI for example

want that? Don’t I want the definitive fix? But as you’re describing all of these other things that can look kind of similar, a lot of people I imagine are thinking, oh my gosh, I don’t think anyone's ever worked me up for.

you know, Eagle’s syndrome or bow hunter’s, you know, any of these other things. Do you have any suggestions for people who, again, as Dr. Chopra pointed out, people that are listening to this are literally all over the world actually. And they might be listening and saying, gosh, I wish I could schedule an appointment with Dr. Bolognese or to get a screening of some sort with some of his team. But for people that can’t even do that, do you have any suggestions for how they can try to get an appropriate workup? You were describing multiple different ways

people can have something that looks like Chiari, but actually only one of them actually is Chiari, right? So those details are so important.

Paolo Bolognese (02:04:36.05)
Yeah, the key is to find somebody locally like you, you know, like the quote unquote, you know, EDS specialist or concierge specialist or aficionado or the EDS bodega, whatever you want to call it. It doesn’t matter because it doesn’t really matter if you are a neurosurgeon, a pain specialist

a pediatric cardiologist or what have so many of ours that, or physiatrists, it doesn’t really matter because then at the end we kind of have a major overlap of diagnosis. So the important thing is to find somebody local. So the, in the United States is absolutely not a problem because

If it is true that 20 years ago, there were not so much. Right now, there is a, not only a raised awareness, but a lot of people going into the field and that’s very good. The risk for some of my neurosurgical colleagues, if there is a neurosurgeon looking and whatever, I will tell them, think twice about getting into EDS because it’s not that easy. And if you get into it,

go slow in the beginning for a while. Before going, don’t make the mistake of pushing the pedal from the beginning. Otherwise you are going to, you know, at least in the beginning we didn't tell anybody to ask, but at least ask somebody who has more experience before you commit with the large volume of patients, because then otherwise you’re gonna have eggs on the face and then you’re gonna just leave and quit. So it’s...

Pradeep Chopra, MD (02:06:15.153)
Thank you.

Paolo Bolognese (02:06:27.498)
It’s a better strategy for a neurosurgeon who has more things at stake and forms of treatment, which are more impactful to go slow, to have long periods of adolescence. The real problem is in the other countries. But the good news is that in the other countries, things are changing. The United Kingdom has decided in March of this year to

create three multidisciplinary centers for neuro-EDS. That was one of the best chance of skepticism worldwide. Australia has one, Italy has a couple of centers of neuro-EDS diagnostic and they’re starting exploring into that. Spain has a couple of specialists who are dealing with that. Holland, they started and they stopped and probably they’re gonna restart again.

So you cannot put everybody, you know, you cannot expect that somebody was just starting is going to have the same kind of results or somebody has been 10 or 20 years in the field or whatever. But we’re not anymore the way we were 20 years ago. So I’m very, very optimistic. But finding somebody with a good reputation is very, very important. And I would always start.

Right now there is the advantage of people like you guys around. I will always start from a primary care neuro-EDS physician before committing it. Because people like you guys are going to be like the quarterback. Like a neuro-EDS is going to need, at a certain point, is going to need an SMAS [superior mesenteric artery syndrome] procedure. It’s going to need a kidney transposition because of a

May-Thurner [syndrome] or is going to need, you know, other, you know, an orthopedic repair done at Harvard by my friend, does ligament reconstruction, or they need a TMJ procedure done in Indiana. So people like you know who the players are, where to send them, and they are the playback. We are just, you know, what’s his name?

Paolo Bolognese (02:08:46.768)
the quarterback, you know, I’m just a wide receiver, you know, one of the many.

Linda Bluestein, MD (02:08:53.361)
Okay, very good. And I always like to end with hypermobility hacks. This is something that started with Dr. Chopra. He was coming up with all of these different hacks and I thought that’s really great. So do you have any hypermobility hacks to share with our listeners?

Paolo Bolognese (02:08:54.59)
Too skinny to be as good as you.

Pradeep Chopra, MD (02:08:57.009)
Hahaha

Paolo Bolognese (02:09:20.886)
You know, going to fundamental is, okay, the first act is, it’s a negative one, not a positive one. And it is actually a warning. I understand the enthusiasm, and actually I’m probably partially responsible, because in the past we did a lot of educational videos and, you know, big.

Linda Bluestein, MD (02:09:31.493)
That’s okay.

Paolo Bolognese (02:09:48.17)
make the patients more educated. But sometimes things get out of hand. So I see a lot of people here, a lot of patients, playing morphometrics on each other, playing doctor on each other, or rendering opinion on the grounds of this and that. I’m not saying that I’m super smart and whatever, nobody else to deal with that. No, that would be stupid. Especially because if I look back,

things that I, things I did not know 10 years ago, I’m smiling at now. But, um, it’s very dangerous to play. If it is very dangerous to play doctor for, for other, for any kind of pathology, this is a kind of a field that is very complicated. So even it’s difficult for us to do, uh, in one stack, that was under certain you could then.

for sure the patients are not, even if they know more about neuro EDS than the average paramedic and physician they find at home. Indeed, out of all the time that they have, while the EDS from the standard training is a footnote to one page of the internal medicine manual, but that said, they should not do it because they’re just giving each other the service. Second thing is, why is it a major no-no? Right now, we are finally...

coming off to the peak or being able to convince a larger doctor community that these patients are legit and this sub-discipline is legit. So if we have a bunch of patients playing loose, they’re going to give the entire cause a bad name to either critics who are critics in good faith or critics who are just looking for some...

easy target to shoot to say, you see, I told you, this is a bunch of bullshit. So this is my negative thing. About the positive hack, I would recommend is the principle of kicking the tire. If you have a suspicion about something, just kick the tire. So if you think that you have an instability,

Paolo Bolognese (02:12:15.486)
Put yourself in traction, put yourself in a collar, even if the two things are not equivalent. And the important thing is not to do it once because you could have something like, there is the placebo effect. Placebo effect, you do it and you really want to do something and to see this. It is like marrying somebody and you’re infatuated. Guess what? After 20 years, if that person is really not right, the placebo effect goes away.

Linda Bluestein, MD (02:12:30.801)
Mm-hmm.

Paolo Bolognese (02:12:45.146)
The 20s is no longer there. So before jumping to conclusion after one test or non-invasive test self-administered, just check if the results are consistent. And then at that point, get the yellow flag up and look for, otherwise, you know, right now, yes, there are people dealing with new ideas like us, but we’re not that many. So we don’t, it is against the public interest to flood.

uh, to flood the resources which are around with, with things that if just the patient had tested three more times, at least, you know, would have found that maybe it was not that hot, that result. So, provocative testing and repetition, there are a lot of the things  
Pradeep Chopra, MD (02:13:58.865)
Okay.

Paolo Bolognese (02:14:10.198)
That guy’s just neurotic. We just look at this. The history is long like this. How can it be? Even if he’s legit, even if he especially would appreciate it once. Other negative hack is do not use the term migraine. Migraine is a specific diagnosis, which means that you just have visual flushing followed by headache. Just we

Linda Bluestein, MD (02:14:27.377)
Mm.

Pradeep Chopra, MD (02:14:30.575)
Yes.

Paolo Bolognese (02:14:37.998)
head pain just in one side of the head, and yet go in a dark room for three days and react just with specific set of medications. That’s it. That’s migraine. Don’t call migraine everything else. Otherwise, number one, you create confusion on a doctor because the doctor is writing, my primary care physician has five minutes to see you. He’s just going to write in your medical report that you have migraine while that is not a diagnosis.

Telling you have a headache and where the headache is and how many times it happens, what makes it worse and what makes it better. Those are probably the most important things. Because that gives the diagnosis away before you even see the MRI. Like one of the exercises I do with my team is we read the questionnaire, the history and the provocative test without seeing

Pradeep Chopra, MD (02:15:15.291)
Yes.

Paolo Bolognese (02:15:35.342)
any MRI or any former diagnosis as a specialist and on the ground to three things only, which is old school medicine, 19th century, we call the diagnosis. And so no more damn migraine diagnosis, otherwise they’re just going to confuse people. And then also to have a healthy sense of skepticism, not to fall in love with things, but to

Pradeep Chopra, MD (02:15:37.616)
Yes.

Pradeep Chopra, MD (02:15:45.681)
That is great.

Pradeep Chopra, MD (02:15:54.445)
Yes. So.

Paolo Bolognese (02:16:03.918)
keep a balance or say, okay, doubt yourself before you just go zero to 100. But because if it is true, it’s gonna be true anyway at the end. Just repeat the test, repeat the test, repeat the test and don’t jump to conclusion after one. If after 10, repeat the following day and then after 10. If it is the right thing, guess what? 19 out of 20 are gonna be positive.

Pradeep Chopra, MD (02:16:32.593)
So as we try to wind up, I have a tiny little hack. I’m sorry, I’m addicted to hacks. So it is a very small hack. A lot of times patients with craniocervical instability have difficulty sleeping because when they sleep and they turn sides, then it wakes them up. And so they can’t sleep.

Linda Bluestein, MD (02:16:41.029)
Hahaha!

Pradeep Chopra, MD (02:17:01.389)
What I recommend is using one of the inflatable airline pillows that you put around your neck. And you can sleep with that on. And so that prevents your head from rolling when you go to sleep.

Paolo Bolognese (02:17:17.07)
Good, so I'm gonna add one, which is a positive hack since I’ve been so negative so far. People with headache, high pressure headache, which is gets worse when you cough, sneeze or strain. Very often they sleep on three or four pillows, which is good in the beginning of the night. And then they wake up and they’re like this because the pile of pillows falls down. So the best thing to do is not to use pillows, but to use a wedge.

Pradeep Chopra, MD (02:17:31.479)
Oh, yes.

Pradeep Chopra, MD (02:17:37.049)
but not.

Paolo Bolognese (02:17:46.254)
underneath, not so many people can afford the bed doing like this, but just to get the big wedge, which can be like, you know, can be just wood underneath the mattress, or it can be a bunch of softer material, call it a wedge, put it underneath the head of the mattress, and that’s going to give you a good thing, which is going to be more effective than a bunch of pillows that

Pradeep Chopra, MD (02:18:12.909)
All right, so you get the award for the hack of the day, Dr. Bolognese. And with this, we are so appreciative that this has been one of the best podcasts I’ve ever seen and it was so valuable. And we thank you. I know you had a long day in the operating room today and

Paolo Bolognese (02:18:21.307)
No, yours was better.

Pradeep Chopra, MD (02:19:59.729)
So I know it is late for all of us, Dr. Bolognese, and I can’t tell you how appreciative we all are. And I’m sure our learners, our listeners, are going to be very, very... Because I always say, knowledge is power. And in this field, where there are so few physicians who understand this, this information is gold. So...

Thank you for coming. Thank you very much for coming on the Bendy Bodies podcast and sharing your knowledge with us. We are very appreciative of that.

Paolo Bolognese (02:20:36.738)
Thank you for having me.

Linda Bluestein, MD (02:20:40.121)
Yes, yes, we are so grateful. This is such an important conversation and one that I think so many people are gonna benefit from, so we really appreciate it. And thank you so much to Dr. Chopra for being the most amazing guest co-host. So really appreciate it. Ha ha ha.

Paolo Bolognese (02:20:49.998)
You're gonna add subtitles.

Paolo Bolognese (02:20:59.022)
Hehehe

Pradeep Chopra, MD (02:20:59.782)
Oh, thank you.

Paolo Bolognese (02:21:02.834)
Dr. Chopra, when I met him, I couldn’t believe it because there was years that I was trying to explain to my fellow neurosurgeons about all this. And all of a sudden I met him and he had understood it by that time, which was several years ago, better than all the guys that I was preaching to for years. And after a little while, he was better than them. And I was like, how is it possible? He’s not a neurosurgeon.

Linda Bluestein, MD (02:21:22.149)
Wow.

Linda Bluestein, MD (02:21:26.053)
Wow.

Paolo Bolognese (02:21:32.615)
I was elitist.  And then I realized that he was just unique.

Linda Bluestein, MD (02:21:36.805)
Yeah, wow, that’s incredible. Because a lot of people probably don’t realize that the typical neurosurgical residency in the United States is seven years, right? And that’s after four years of medical school and college, four years of medical school, and then a seven-year residency. Is that still true? And you did two. Yeah. Wow.

Pradeep Chopra, MD (02:21:38.15)
Me?

Paolo Bolognese (02:21:53.378)
I did twice so I finished at 41. Yeah. At age 41 I finished. Somebody says I did it twice because I was not that smart.

Linda Bluestein, MD (02:22:07.197)
Oh my goodness. Well, thank you so much to both of you. I’m so incredibly grateful that we got to have this conversation, and I know so many people will appreciate it. And yes, I will also put this in the show notes, but definitely I will encourage people to watch this on YouTube where they can see the subtitles. We will be having the transcript also available so they can read along and be able to search for the specific things they want, and they can look at that specific timeline and everything.

Alright.

Paolo Bolognese (02:22:38.71)
Since we’re talking about a questionnaire, etc. and since my questionnaire is an open source, I’m going to send you the form that we use, not only for the patients to see, but also very important for any practitioner who wants to use it or copy paste or copy paste modified. This is for everybody to benefit from what we have learned over 20 plus years.

Linda Bluestein, MD (02:22:49.293)
Oh, that would be amazing.

Paolo Bolognese (02:23:07.35)
It’s kind of, they start from what, where we ended, which is where we have people have to go. Okay.

Pradeep Chopra, MD (02:23:07.737)
Yes.

Linda Bluestein, MD (02:23:10.961)
That is incredibly generous. Thank you so much. That is really, really generous. And we will link that so people can access it. And that’s amazing. That is amazing. So you’re sharing your knowledge and resources, which is phenomenal. So thank you again Thank you so much and we’ll see you next time on the Bendy Bodies Podcast.

Pradeep Chopra, MD (02:23:48.913)
Thank you. Goodbye.

Paolo Bolognese (02:23:49.292)
Bye.

Linda Bluestein, MD (02:23:50.02)
Bye bye.