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

[00:35] Dr. Linda Bluestein: 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 is not a substitute for personalized medical advice. Before I introduce your guest, I would like to reintroduce you to my colleague and friend, Dr. Pradeep Chopra, who will be your guest co-host 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.

[01:31] Pradeep Chopra: Hello, Dr. Bluestein and Dr. Bolognese. My pleasure to be here once again.

[01:41] Dr. Linda Bluestein: Wonderful. And could you introduce our special guest for us?

[01:46] Pradeep Chopra: Yes, our very special guest for us today is the wonderful Dr. Paolo Bolognese. He is a neurosurgeon and founder of the Chiari Neurosurgical Center in New York. Dr. Bolognese is also on 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 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 1 Malformation, syringomyelia, craniocervical instability, tethered cord, Eagle Syndrome, idiopathic intracranial hypertension, and intracranial hypotension. That's a lot of work you do.

[02:51] Paolo Bolognese, MD: I know. I'm tired already.

[02:54] Pradeep Chopra: Dr. Bolognese's surgical experience includes more than 1,600 Chiari decompressions and 900 craniocervical fusions, 300 of which with condylar screws. He is also on the board of the main 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 it sounds really fantastic.

[03:36] Paolo Bolognese, MD: Thank you very much.

[03:38] Dr. Linda Bluestein: Dr. Bolognese, hello and welcome to Bendy Bodies.

[03:43] Paolo Bolognese, MD: Thank you for having me here. I'm very, very glad to be here.

[03:46] Dr. Linda Bluestein: Oh, wonderful. We are thrilled to chat with you. Could you start out by telling us briefly how you became interested in EDS and related conditions?

[03:55] Paolo Bolognese, MD: It was a total accident. 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. He wanted to start the Chiari 1 Malformation Center, and at that point there were just a few cases done nationwide. So the fact of having two neurosurgeons building a center together was kind of like building Yankee Stadium when you just have a couple of kids from the neighborhood going to play. 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.
[04:50] So long story short, we're having this Chiari Center, and we're operating together. I was seeing the outpatients — and several for Chiari surgery means 6 in a day. All of a sudden I see that 2 of them had Ehlers-Danlos syndrome. And then the following time I was seeing patients, I saw another 2. And then the following week, I found another 4. What were the chances for these things to be associated by sheer chance? So I went to my chairman and I said, I think that there is something strange here. I found it too many times. He gave me a dose of healthy skepticism, because he was the older guy and I was the younger guy. One month later, I went back to him and I said, listen, I keep seeing it, so it is not an accident. He was again skeptical. And by the 89th patient over a few months, I made an Excel file for him and showed him: we have patients who are younger, the ratio between women and men is instead of 3:1, it's 9:1. They have a different phenotype, they're younger, they have different other comorbidities. Ehlers-Danlos Syndrome was not really a main thing in medical school education. So at that point, he picked up the phone and called the geneticist from Duke, who was the international leader in the field of genetics of Chiari. And she said, I've never heard of it myself, but statistically, you have seen this many Chiari, this many EDS — the chances for this to be chance alone were several millions to one.
[07:05] And so he picks up the phone and calls the person in charge of connective tissue disorders. That person was Claire Francomano. Claire Francomano was the director of the Department of Aging, which covered connective tissue disorders and related things. All of a sudden, it was a bizarre phone call, because all of us — 4 of us — were giddy because we were completing each other's sentences. There was this chemistry instantaneously, because they had pieces that we were missing and vice versa. They could not explain why there were so many neurological problems in their EDS patients. On our end, we were simply ignorant — we probably should have opened a textbook before talking to them.
[08:01] So at that point, it was natural for us to start cooperating. We couldn't call anybody, and we started doing surgeries and learned the hard way — eggs on the face — what was working and especially what was not working technically by applying the standard technique we knew. We started going to national meetings, we started talking about Ehlers-Danlos syndrome, and nobody bought into it. For years, we were told, I never seen that. The classic reaction of other colleagues was obvious: I've never seen a patient with Ehlers-Danlos syndrome my entire career among the Chiari population.
So the next person coming on the scene was Dr. Henderson. Dr. Henderson had, like me, a kind of a strange career. He was coming from Australia, went into the military as part of the regular draft, and before coming to the United States, he made a stop in London in a place called Queen Square. Queen Square was at that point the center of the world for everything related to craniocervical instability — not because of EDS, but because the old British ladies were developing this strange disease called rheumatoid arthritis, which is a sort of inflammatory reaction that attacks the joint. Instead of just making the ligaments weak, it creates an inflammatory mass called the pannus, which compresses and pushes things around. He was trained by the god of craniocervical instability, a fellow called Crockard. So when he came to the United States shortly afterwards, he had the same reaction. He said, all right, I studied craniocervical instability. I know how to do craniocervical fusion much better than an average neurosurgeon in the States. I'm going to start going ahead.
[10:52] All of a sudden we were two hospitals starting to work on that, and more and more patients starting coming in. At the beginning it was Chiari 1 Malformation, with EDS looking like a comorbidity — what we call now complex Chiari, meaning that the surgical management is rendered more complicated by the fact that the joint is defective.
[11:18] The very first time that we met a patient who was pure EDS was in 2002, after the first time we observed the Chiari-EDS link. There was this girl who now would check all the boxes for the 3 of us — very debilitated, age 17, 3 or 4 years of progressive debilitation, unable to get out of the wheelchair. Nobody saw anything. Nobody — it's all in your head. The usual routine. And the mother, who had gone anywhere and everywhere, came to us because she thought that maybe it was a form of Chiari 1 Malformation. She checked everything else. I look at the MRI, and it was kind of in the shades of gray between full Chiari and normal, more toward the left side of the spectrum than clearly normal. But I was seeing that this patient was really sick. I got up, went around the table, grabbed her head, and pulled it up — and all of a sudden she woke up and perked up. She was able to move around. She was able to swallow. Then I let it go and she reverted. We did it 5 or 6 times. The mother was all excited because she hadn't seen her daughter like that, even for a few seconds, for 4 or 5 years.
[12:56] So I went back to Milorad, my mentor. I told him, this is a kind of a black box scenario. I do not know what's inside, and neither do you, because right now the MRI looks normal-ish. There is no Chiari 1 Malformation, there is no retroflexed odontoid, there is nothing. But every time I pull her up, she feels better. Every time I let her go, she feels worse. So it looks like some kind of craniocervical pathology resembling cranial settling — like settling of a house on the foundation. I don't understand how it is, but if making it like this makes her feel better, we can do a fusion.
[13:38] So at that point, there was already a jump from complex Chiari — in which the MRI was self-evident — to a scenario we now call pure neuro-EDS with instability, in which the patient had a normal MRI, but a much bigger clinical change than a Chiari malformation by itself. Milorad had the professional courage to say yes. He could have said, the heck we're going to do it — I'm going to have half of the neurosurgical community after me. Instead he said, I see the same thing. So we had her come back, we did a fusion, and it was not a surprise — but back then it was the hell of a surprise — to see that she went well. Not only did she go well, but her story was a Cinderella story. She ended up becoming a supermodel for the Ford agency in New York City. She was a high-end international supermodel for 4 or 5 years. Then she got married, and now I'm an uncle for the third time. Every time a baby is born, she sends me pictures.
So those were two different paths. The second patient was kind of the Rosetta Stone of neuro-EDS. It was the step forward from just the Chiari-EDS connection. And then all the problems start: diagnostic problems and technical surgery problems. When do you call it? How do you grade it? What kind of workup do you do rather than just shooting from the hip? Science is always something that keeps going, sometimes accelerating, sometimes stagnating. And when there is no book to read, you just find out.

[16:00] Pradeep Chopra: You write the book. In that case, you write the book.

[16:09] Paolo Bolognese, MD: Some chapters you discover afterwards that you wrote them, and then in retrospect, the test of time says, okay, you have to do these other things instead. You're always in the fog of war. You're always as smart as the last lesson you learned.
[16:34] What we did not understand at that point was that the problem was way more complex than we were anticipating. The neuro-EDS model had so many additional pieces that we're still finding out. The diagnostic challenge — how to assemble a workup which makes sense — requires a balance. You cannot be so zealous that you say, it is what it is, 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. Flexible about the idea strategically — the criteria you have today could evolve over time — but rigid tactically. So version 3.1, the set of criteria you have now, that's the line in the sand. You do not cross it, because otherwise you're not going to have hard ground to stand on when you look back and evaluate. When you discover there are some limits, then you do version 4.0.
[18:01] And then there were all the problems which are purely technical about the surgery — you have a technique that works for other conditions, and then you discover what works and especially what doesn't work, and you move forward.

[18:29] Pradeep Chopra: That was a great story, because we wanted to know how you began. It looks like you and Dr. Henderson were the pioneers in this field. Even today, after all these years, we're still learning and understanding the nuances of EDS. And you were in the middle of it all — I mean, the spine itself, even without EDS, is such a complex structure, and even within the spine, the cervical spine is probably the most complex structure. It balances a heavy head. So the question I have is: if I suspect somebody with EDS has craniocervical instability or Chiari malformation, and I say, I want you to see Dr. Bolognese — what is your process of evaluation? How do you start? Our listeners want to know, like, how do you evaluate them?

[19:47] Paolo Bolognese, MD: Again, what we have now is the current permutation of all the things we have done over the years. I had 3 mentors, which is extremely lucky. The one who had the biggest influence over me was Milorad. Milorad was OCD to the core. He was never focused on — it's 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 struggling. Every time I would say, okay, textbook says the complication rate for this is good and we're better, so we're fine. He would say, no, it's not good enough.
[20:59] The Chiari 1 Malformation surgery back in the '80s had a solid 50% complication rate. When we went down to around 10%, it was an enormous improvement. It's not about being a genius — it's like tying your shoes, you do it over and over and you push yourself and it's inevitable you're going to get to better numbers. But he was always pushing. The most important complication was cerebrospinal fluid leakage. We went down to 3% and it was still not good enough. Then we went down to 0.3%. At that point, statistically, it's going to be difficult to go lower.
So going in the other direction — which is not only diagnostic — the issue is not so much how many times you have it correct, but how many false negatives and false positives you have. False negatives you realize later on when somebody else operates or you change your criteria. But the false positives are the most important in terms of overall damage to an evolving discipline, because a false positive is going to potentially translate into a surgery. So you have a surgery that has not worked — it can be because of poor selection, poor execution, or simply because the surgery was indicated and correctly executed, but that technique is not enough and you had to resort to something different that is not in the book yet.
[23:31] So after the first 5 or 6 years, we already had something like 300 cases and we were all excited. Craniocervical instability linked to EDS to that degree and with those connotations was something new. We were going to wait some years before reporting because you cannot just do the surgery and observe for 3 months and say we're fine. You have to have a good long-term post-op. When we looked at it, the complication rate was higher than the standard for trauma or other pathologies like congenital in children. Not enormously higher, but higher enough that if we published, it was not going to look great. And so the pathology was not going to get a good reputation either, because a reviewer would say, yeah, right, and pass. So we did not publish intentionally the first hundreds of cases we did, because in order for something to be credible, you had to have at least the same outcome and complication rate as a conventional technique for conventional pathology, and then you can present the new pathology for what it is.
Anyway, going back — what is the situation now? We have an approach which is multi-legged, like the pieces of a puzzle. The patient comes to be evaluated already pre-screened by somebody else, and we raise a level of suspicion before we send them to trial. It's like you are the DA and you listen to the police and look at the body of evidence to see if somebody qualifies to be indicted and sent to trial. We look at clinical history, provocative testing, radiology, and a test trial.
[26:42] The clinical is simple. There is a series of symptoms typical of every anatomical region. If you have a broken ankle, you're going to be limping around. If you have a bad stomach, you're going to have problems digesting. There are a lot of cables and structures passing through the craniocervical junction. So there is a long list of signs and symptoms. Those are not exclusive to craniocervical instability, but they are linked to the region. If you have a trauma, a tumor, a congenital malformation, or neuro-EDS affecting that area, you're going to have the same symptoms with different kinds of severity, different kinds of grouping. If you look at the list, you're going to see that that list becomes hot — in the sense of how many positives you're going to have. That's number one.
[27:42] Number 2 is provocative testing. If I have a broken arm and I do like this, even if the two stumps are aligned, the patient is going to complain. It's like kicking a tire. You play with the joint. If the chief complaints and the symptoms of this area get worse or better respectively, that means you are onto something. That's number 2.
[28:12] Number 3 is the history. If somebody is already hypermobile, one time falls on their head and twists the neck and all the symptoms start afterwards — that's one of the many parameters. Recently, for example, we're seeing a lot of patients with deteriorating EDS or a sort of acquired form of EDS in long COVID patients, with long COVID kind of pushing people over the edge. We've seen it with a lot of tropical diseases. So the history is the third thing.
[28:52] The fourth one is morphometrics. The majority of these patients, when they come in, do not have much in terms of radiology. So there is a big asymmetry between the chief complaints, which are devastating, the quality of life, which is poor, and the neurological exam, which quite often is normal-ish. You don't have the massive hyperreflexia or clonus — you can find some in some patients, but most of the times the signs are less than the symptoms. The quality of life is enormously out of proportion with what the MRI presentation is.
[29:37] So what we use at that point is morphometrics. All the morphometrics which were available at that point were for trauma, and there were about 18 parameters. Notably, the Grabb or pBC2 did not exist yet — it was introduced a few years later — but there were 18 parameters back then. The only principle is: whatever parameter you use, you cannot just use one; you have to use two. It's like looking at the sole of a fish — from one angle it looks gigantic, from another it looks different. 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.
[30:26] We tested a bunch of these during invasive cervical traction and discovered that we were coming with these 18 parameters and working toward an understanding of what was abnormal with the pathophysiology of the joint at extremely sophisticated levels. But it was very counterproductive in terms of screening. So we boiled it down to 3 parameters, which then became 4 after the addition of the Grabb. Certain things we noticed were not really that helpful, like the power ratio, the SDS, or the Chamberlain line. Others were more helpful than usual. So what we're doing now, we're using these 4 param— go ahead.

[31:20] Pradeep Chopra: 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?

[31:34] Paolo Bolognese, MD: Yeah, it's almost correct in the sense that the morphometrics are measurements of distances, angles, areas, or volumes that you can perform on different neuroimaging media.
So we get an MRI supine, high definition, and we start doing the first set of 4 parameters. Then the 5th one is the diagnostic trials. Diagnostic trials means we have somebody doing repeated trials of traction at home, or wearing a collar for a certain amount of days at home. And then if they have it, it is like turning a switch on and off. If you have consistent improvement every time you do certain maneuvers and you go back to square one when you remove the collar or the traction, fine. At that point we have 5 points. Sometimes all 5 are positive, sometimes 4 are positive and 1 is negative, but you raise a body of evidence to establish a level of suspicion.
[32:39] But at that point, we don't finalize the diagnosis at the surgical level. If somebody is a neurologist or a pain specialist, 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 is referred to a surgeon, it's the responsibility of the surgeon to go to the Supreme Court before labeling somebody, because as a neurosurgeon, the diagnosis has a heavier connotation.
[33:23] So at that point, we decided to have the test of invasive cervical traction. The beginning was just, all right, if the patient does the traction at home, who tells us they're doing it the right way? Sometimes they have a bull neck. Sometimes they have a short mandible. We just want to do something in the last minute before doing the surgery that confirms what we can see with our own eyes. And then we started adding the morphometrics to that, and we reached the conclusion of giving scores. So again, first we have the pre-screening by the local physician or the EDS specialist. We put those 5 points together and say, okay, now you qualify for verification. Our standard for giving the diagnosis is you have to pass this test.
[34:28] They come, they do the test, and with the test we give scores. The scores are two: one is clinical and one is radiological. The clinical — it does not matter that we already know 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're coming to a neurosurgeon, is: do you pass a certain threshold of severity? We consider the patient as 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 the chief complaints improved by 80%, at that point they have severe craniocervical instability — at least they have a positive clinical score.
[35:35] Then, in order to have a positive test, they also have to have a positive radiological score. The reason is this: there are a lot of people with EDS out there who have no symptoms. They are excellent ballerinas, phenomenal track and fielders, they get the gold for gymnastics for the U.S. Olympic team. That's a demonstration that EDS is also an evolutionary mutation which has brought advantages but becomes a medical problem when somebody falls off the cliff. The same things which make these people amazing — skin which makes them look younger than they are until a late age, phenomenally attractive skeletal features, athletic performances which are superior because of the way their tissues are — these are the captain of the football team and the homecoming queen, the people who are the idols. But if things start going in the wrong direction, they become our patients.
[37:03] So if you have somebody who's hypermobile but you only have a very positive morphometric change of certain parameters, that doesn't mean you're unstable. That means you are hypermobile, unless you also have a positive clinical score. Hypermobility plus symptoms equals instability. So if you have a positive clinical score above 75% improvement and you have movements which are above normal — defined normal for normal people, not normal for EDS — and you have the two together, at that point the invasive cervical traction is positive, and then we move on.
Another thing: when the patient at 35 pounds feels great and they're happy — more recently, for the last year or so — when they're really happy, since they're going to go back down and be unhappy and have brain fog when we remove the traction, I give them my phone and have them calling their relatives. We take pictures and videos. Whatever happens, they describe it from the inside, and they're always very touching.

[38:22] Pradeep Chopra: Is that what is called the Jesus moment?

[38:26] Paolo Bolognese, MD: Yeah, because you can also have the patient who kind of wants to have it. They come in and they tell you the song and the dance. They can be hypermobile, yes, whatever, but they're convinced they have craniocervical instability. You go up and they say, how's the improvement? And they say, yeah, it is 80%, because they heard the thing. But you see on their face — the people who really have the 80% say, oh my God, it was not like this for the last 5 years.
[39:10] There was this mother who was having phonophobia, so she could not tolerate sound. She was going around with noise-canceling headphones. She's in traction at 35 pounds. We removed everything — the sunglasses and the noise-canceling. She looked into the surgical lights and we had a boombox on at the max and she could tolerate it. All of a sudden she starts crying and says, I'm so happy now I can listen to music with my daughter. You cannot fabricate that. On the other hand, the person who's really not convinced is going to tell you, yeah, I think it's 80%. You can tell that they don't have it even if they want to have it.

[40:02] Dr. Linda Bluestein: You gave a couple of examples of clinical signs and symptoms that people are presenting with. And I think people are probably listening to this and chomping at the bit 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?

[40:28] Paolo Bolognese, MD: Yeah, it's actually one of those things — you start with it like eating cherries, and then you discover that things are way more complex than you were anticipating. For example, we were having patients who were coming in and having improvement in their symptoms. And at that point you ask yourself, okay, how much is coming from the instability and how much is coming from another mechanism? For example, you pull your head up, good, great, and you can affect the instability, but you can also affect, in certain conditions, the jugular outflow coming out of the skull. How do you demonstrate one or the other?
[41:17] So at that point, we added another piece. There are certain neurological signs which come from the lower part of the brainstem. One is the coordination of swallowing, which comes from the lower part of the brainstem and involves a number of cranial nerves. It takes about 14 different movements coordinated in a specific order for stuff to go from the mouth to the esophagus. The things which are a little bit counterintuitive are that the swallowing of solids and the swallowing of liquids are not the same. Swallowing solids tends to be pathologic mostly for mechanical problems — like you have a cancer in the neck, or a hematoma, or simply somebody is doing this to you, you cannot really swallow a piece of bread. Pushing down a piece of solid food is actually a relatively easy task for all the muscles and nerves.
[42:37] On the other hand, swallowing liquids, which looks like you just pour it down and it's easy, is actually the most complicated. One of my grandfathers was an intellectual, the other was a farmer, so I'm going to go with the farmer right now. It is like you have to go through a gate with 1 cow or with 25 sheep — 25 cats would probably be better. The cow, you just beat him on the rear and it's going to go in that direction. It's just 1. But if you had 25 little animals, it's going to be a thing. The liquid is more like the 25 cats that really need to be directed specifically.
[43:28] So dysphagia for liquids is a typical neurological problem that can be pinpointed, because if I cut the glossopharyngeal nerve — everything converges to the lower brainstem. The second thing is breathing. Automatic breathing and all the regulation thereof are integrated in the brainstem as well, in the lower part. Then there is a third one, which is vision — not so much visual acuity, but vision coordination. There is the medial longitudinal fasciculus, which extends throughout a big section of the brainstem and allows us, 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 — even if we are in a car and the target is walking on the sidewalk in the opposite direction — to stay focused. When I want to be a buffoon, I tell the patients, okay, imagine you are in the car driving this direction. All of a sudden there is Brad Pitt walking toward you. You want to look at him very, very well, and for that image to be burnt inside your brain. You don't want a blurry or jumpy picture. The medial longitudinal fasciculus works in overdrive.
[45:00] So we put these 3 symptoms at the bottom of our list of chief complaints. Now we have 2 different scenarios: one in which the patient improves in everything, and another in which the chief complaints do not improve but the brainstem symptoms do — and the vice versa.
[45:29] The first scenario, in which everything improves, gives you a higher level of confidence. It becomes very difficult to explain that these things are not coming from the brainstem being manipulated by the traction. On the other hand, if the chief complaints improve but the brainstem markers don't, at that point you can have some level of doubt: I'm not that convinced it is coming from there. At that point, you put the brake on before you give the golden ticket for surgery.
[46:53] All these things are observations that you see a number of times, then finally come from unconscious to conscious, and you build a system around it. The major issue is — even when you're enthusiastic about what you're doing and all sold and invested in the disease — you have to be very skeptical for the individual patient. Strategy being optimistic, but at the tactical level for the individual patient, be the opposite.

[48:22] Pradeep Chopra: Dr. Bolognese, one of the questions that we as practitioners get a lot is, oh my God, invasive cervical traction — that sounds invasive. You explained to us why you do it, and that was a very good explanation. But our listeners also worry about whether it is painful or difficult. They want to know a little bit more about that.

[49:09] Paolo Bolognese, MD: Okay, the first thing I'm going to say is a little bit tongue-in-cheek, which is this: the best selection is to put a high bar to discourage the faint-hearted. There is a funnel going towards a neurosurgical specialty. If I and the other people in my field were seeing every single neuro-EDS patient with any degree of instability, we wouldn't have the time to operate. It is like waiting in line, and there are a lot of people who are kind of wasting your time. If you are a patient who really needs surgery and is really sick, and there are 99 patients ahead of them who have a mild degree of the disease, that's a delay of care for the poor person who is genuinely sick. So it makes sense to have scary things outside the door. The people who have a mild degree of the disease will think twice. On the other hand, the people who are really sick are going to tell you, you know, it doesn't matter — I have enough with this, I'm so sick.
[51:08] So I do not intentionally want to downplay the procedure. Oh, it's not really invasive, it's just — no, it's invasive, period. You have two pins which are going through the skin in the outer layers of the scalp. Take it or leave it.
[51:24] That said — other surgeons in the field are not doing the test, which automatically means that either we are right and they are wrong, or this is not the standard. This makes sense for us at this point in our journey for all the things we have done for the last 23 years. Period. In a polite way, it's either this way or the highway. If you do not want to do the traction, it ends there. Don't try to come browbeat me with 500 emails per day asking, can I skip the invasive traction and go straight for the surgery? The answer is no. The test is elective. But without a positive test, the surgery is never going to be an option from our part.
[52:36] So how is the test done? The patient comes into the operating room, and we give them MAC anesthesia and local anesthetic. MAC anesthesia is like going for a colonoscopy.

[52:52] Pradeep Chopra: Twilight sedation.

[52:53] Paolo Bolognese, MD: Right. And the reason we give MAC is that we don't want any kind of other medications — like fentanyl or whatever — to make the patient better or sleepy. We want the patient fully awake when they come back from the protocol. And then we also give local. If you use just lidocaine, it does not work as well. But if you use a mix of lidocaine and Marcaine for any kind of procedure, including at the dentist, it works much better. Certain patients, you put lidocaine and it is like you're not giving anything at all. We also use a little touch of epinephrine unless there are contraindications, because that prolongs the local anesthetic.
[53:47] By that time, the patient is comfortable with local anesthetic. The secret of any kind of hardware in the body — wherever you put it — is that once you put something attached to a bone and that piece of hardware is fixed and stable and doesn't move, you can pull the patient up like a pail of water and it's nothing. The problem starts if you don't put it tight enough and it becomes loose. At that point, it hurts. Then we wake them up, wait for the MAC anesthesia to wear off, and then we start.
About the traction itself: for cases like a broken facet or any post-traumatic case, you go up to 50 or 60 pounds. 35 pounds is a safe level for our patient population to do the things we need to do.
[55:07] Now, there is the issue that this is not a therapeutic procedure. So if you feel better during the traction, when we remove the traction, you go back to square one. If the patient says, traction doesn't work because once I remove it the symptoms come back — first, you should read the instructions we sent you, but they're expecting too much from a diagnostic test. It's like, I tried this test but the cancer came back afterwards. That's not therapy. That's just doing a biopsy to make a test. Doesn't cure your cancer. Same reasoning here.
[55:51] The issue is that some patients can have rebounds. If somebody tells me, I tried non-invasive traction at home and I had a rebound, I already know that 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. If they are in pain, anesthesia is there and can give them something, which they cannot have in the comfort of their own home.
As for overall complications: we do something like 2 to 4 invasive tractions per week, we've been doing it for several years, and so far nobody has had complications at the pin site. Nobody has had a broken neck as a result. There were two patients who were already scheduled for surgery two weeks later whose rebounds were particularly intense and lasted more than a few hours, going into the following day. So instead of doing the surgery two weeks later, we anticipated it to the same hospital stay. Per se, that was not a complication — just a rebound in somebody who was severe. It would have happened even with non-invasive traction.
[57:43] But again, this is an elective test. If somebody is squeamish about it, I'm actually happy, because you're probably not that sick after all. If you are ready to commit to a surgery but emotionally uncomfortable about an invasive test used for selection, that doesn't make sense.
Which brings me to the next issue we developed over the years. Back in the days before MRI was used routinely, there was a 5 to 10 year delay between the onset of symptoms and corrective surgery for severe cases. EDS patients have paranoia even worse because of the years of being told, there's nothing wrong with you, the MRI is normal. But their personality is different. These are type A personalities that all of a sudden get frozen, and then once they're corrected, they bounce back and you practically have to put the brake on them. They're patients who give a lot of satisfaction because you know you're giving something back and they're going to use it. But at the same time, we're having a different mix of psychiatric disorders.
[59:15] All right, I'm going to go somewhere uncomfortable now, but first the point. At the beginning, we were asking for a psychiatric screen only on patients who, in our early encounters or on the way to surgery, had a heavy diagnosis on their history — like attempted suicide, heavy-duty psychiatric drugs on board, or some heavy-duty prior diagnosis. So we would say, okay, we need a psych clearance.
[59:53] But then I had a few patients who tricked us — patients who withheld information, or other patients who acted the part but then decompensated at the time of surgery. These surgeries are big, so you need full cooperation. You cannot have somebody sabotaging their own recovery, because they're going to be exposed to more risk of complication and squeeze less out of the entire adventure. So we decided we need a psych clearance on everybody.
[1:00:37] The current parameter 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 nothing about what EDS and the surgery is about. What they need to tell us is: is the patient optimized? Is the patient reliable in reporting their symptoms and their intensities? Does the patient have a support system at home? Is the patient resilient enough?
[1:01:09] And then there is a second, independent psych screening with our neuropsychiatrist. Our neuropsych 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 lies ahead. So he is going to be the person who pushes back and pokes them a little just to see — you're really sure? It's not that it's a game. It's just to give the patient one last real moment of insight. Because sometimes you're so invested — I'm going to see a neurosurgeon, I'm going to have the surgery, I'm going to be good — and that little shaking that somebody needs is important.
[1:02:18] And we don't do it before the surgery. We do it at the time of the invasive cervical traction. Because if we did it, as we used to do several years ago, two days prior to surgery — at that point it's a loaded circumstance. The patient is already involved, the insurance is approved, the machinery is going, it's a different kind of psychological pressure. On the other end, when they come for the invasive traction, they don't have a confirmed diagnosis yet and don't even know if they're going to be a surgical candidate, because they don't know what the score is going to be.

[1:03:07] Pradeep Chopra: I had another question, and I'm sorry to interrupt. Your neuropsychiatrist is amazing. A lot of the patients I've sent who have seen him actually want to keep him as their psychiatrist. He's great, and like you said, he helps them understand exactly what they are going to go through. A lot of them feel like, yeah, this is the first time they have met somebody who really understands.
[1:03:49] My question was: we are spread apart — 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.

[1:04:08] Paolo Bolognese, MD: Yeah.

[1:04:09] Pradeep Chopra: 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 about it?

[1:04:23] Paolo Bolognese, MD: I'll tell you a secret. Some of the things for the report, how I structure it — I actually copied from you. That's why I like it so much.

[1:04:35] Pradeep Chopra: 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.

[1:05:00] Paolo Bolognese, MD: Yes. Can you tell us a bit more about that? It's actually coming from something related to COVID and legislation. Telemedicine 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 to come the first time, then do the testing, come a second time, and then finally get a diagnosis. Before you know it, it is at least 2 trips. And 2 trips around the country by somebody who's sick, needing to come with a companion who has to take time off — it gets complicated.
[1:05:51] So in 2004 we were looking into telemedicine with our health system, and there was no legislation nationwide. Actually, one state — Texas — was at that time prohibiting any doctor from calling a patient on the phone from outside Texas because they didn't want their patients being poached. Our health system said, good, you guys want to do telemedicine — you cannot do it. So it took several years, and then we were allowed. Then COVID came and legislation became more precise. We had a decision to make: do we go back to the model before, in which patients come multiple times, or do we do something similar?
[1:07:08] By that point, some institutions — Stanford, I believe, was one of the first — had done the second opinion model. Second opinion model means you send in your clinical information in a structured way. It gets reviewed by a committee, then a junior attending has to become the scribe and sends it back to the patient. That's it.
[1:07:42] We saw some value in that, and the value is this: instead of seeing you in the office and then telling you what the workup is, we front load everything. We say, these are the things you have to do before coming. Once you fill out our long, multi-page clinical essay, once you enter the history and all of that — and if you've seen other specialists, add their letters too. I know that when I have one of your letters, I can just kick my feet up, because you've already done everything. The only thing I had to say is, the following diagnoses have been suspected by Dr. Chopra and I fully agree. Easy day.
[1:08:35] But anyway, at the end of that letter, what we can say is: we have suspicions about this, and the clinical history, provocative testing, and radiological data raise a critical mass of suspicion for the following diagnoses. For which we need additional testing. This is the additional testing. And if you want us to confirm it, you come here for invasive testing. At that point, the first encounter is done and we're already 3 or 4 steps ahead.
In the past, they'd reach that point after 6 or 9 months and 2 or 3 visits, with the patient going back and forth. The second opinion avoids all that. But then we figured out that a lot of patients — since we didn't make it so expensive — were just curious and not particularly sick. They were writing in. So we needed somebody to do a screening to separate the patients who were not very severe and should go to the conservative component of our team, not directly to a surgeon. Obviously don't throw surgery at everybody. But at least to prevent patients who were truly sick from having a long line, 50% of which was made up of patients who were only mildly sick.
[1:10:26] We're still refining the process, but so far it's working pretty well. Because at the end of all that, when the patient comes into the hospital, all the background is already in the bank, already worked out. When they arrive, it's, okay, all this is already digested — now we just need to check on these 3 or 4 items. So the encounter doesn't become a meandering adventure with a lot of loose ends that can never be tied up. Everything becomes more effective.

[1:11:13] Pradeep Chopra: So I'm just summarizing for our listeners: the first thing you do is ask them to fill out some forms, upload their radiological images, and then based on that, you come up with a suspicion of the following diagnoses.

[1:11:20] Paolo Bolognese, MD: Yeah.

[1:11:51] Pradeep Chopra: And then, if the patient has Chiari malformation, or whatever the case may be —

[1:11:52] Paolo Bolognese, MD: If the patient has mild symptoms, for example — you can have a monster Chiari 1 Malformation on the radiology, but they have mild symptoms and a very good quality of life. At that point, there is no need for them to come here for further invasive testing. It ends there and we say, okay, in 2 years do another MRI and touch base with me.
[1:12:26] But on the other hand, you have somebody with severe craniocervical instability with compromised quality of life. At that point, the plan is: if you're interested to go further, come here, we're going to do the invasive test.
[1:12:47] So once they get that second opinion report and you feel they need to come in because they are clinically very symptomatic, their data is going to point directly to invasive testing in a hospital setting. It's not going to be like, I see you in the office and you come back. You just come here, we do it right. And it's not only the basic traction. Some people have a suspicion of a disorder of intracranial pressure — going to do an intracranial pressure monitor with positional testing. Or there is a problem with the glossopharyngeal nerve — going to do a glossopharyngeal block. Or there is a suspicion of neurogenic bladder — going to do urodynamics. Since we are more aggressive with our toolkit, the ownership of the responsibility of the diagnosis has to go to a stronger and more objective level. Invasive testing is the highest level of diagnostic standard we can apply.

[1:14:06] Pradeep Chopra: So that reminds me about one condition that you treat very well called Eagle Syndrome. Can you tell our listeners what the symptoms of Eagle Syndrome are?

[1:14:23] Paolo Bolognese, MD: There is a bone at the base of the skull which is converging from below the ears toward the voice box, and it is called the styloid process. Usually it is a small and thin piece of bone, and it is the attachment for about 3 tendons which are attached to muscles that go to the voice box. They're helpful with a number of movements that we routinely do.
[1:14:55] Eagle Syndrome is when this bone becomes very big, very cumbersome, very long, and in doing that creates a number of different problems. In the past, it was called Eagle Syndrome only when it was poking the trachea and/or the esophagus. Then they found out that sometimes other vessels passing by — like the jugular or the carotid — can be impinged. So that was called the vascular form of Eagle. Then it was found that a number of cranial nerves passing by get stretched, pushed, or nudged by these bones simply because they are in the right place and this bone, being bigger, is kind of pushing and shoving.
[1:16:05] Now, like everything related to EDS, they're like the princess and the pea — super sensitive for everything. The majority of people really don't care that they have a styloid that is a little bit bigger or a little bit thicker. The majority of the longest series about Eagle are actually coming from the Indian subcontinent, because for some reasons over there, the styloids are enormous when they're pathologic.

[1:16:40] Pradeep Chopra: Indian, like from India?

[1:16:42] Paolo Bolognese, MD: Yes. The largest series come from there.

[1:16:47] Pradeep Chopra: Oh, the largest.

[1:16:49] Paolo Bolognese, MD: Yes. Also because you have 1.3 billion people, but they are really phenomenally big. So there is also a kind of genetic component to that.
In EDS, the clinical presentation mostly affects the function of the glossopharyngeal nerve. If the styloid becomes thicker and just passes by, that's a little extra stretch. In our very touchy-feely EDS patients, it's enough to create trouble. Now comes the issue, because the glossopharyngeal nerve is very complex. If I tell somebody, I go to the doctor and say, I have palpitations and a ringing in the ear and a sensation of metallic taste in my mouth — this doctor is going to say you're crazy. And instead, those things come from 3 branches of the same nerve. The glossopharyngeal nerve. If you remember medical school and neuroanatomy, that's easy. Apparently my ENT colleagues took a while to figure that out.
[1:18:12] So what you do is this — same principle as when you go to the dentist and you have a big problem with your tooth and the dentist gives local anesthetic and you feel better. So if the glossopharyngeal nerve is crying uncle because it's pushed by the styloid, you 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 the glossopharyngeal being kicked around by this bone.
[1:18:55] Now comes the tricky part. We figured out the association between Chiari and hypertrophic styloids, and EDS and hypertrophic styloids, a long time ago. Since we were not trained as neurosurgeons to do these cases, we were sending these patients to ENTs. I remember one time I sent a patient to an ENT in my health system, and that patient came back with a tracheostomy and a gastrostomy. I know. And obviously at that point we became more prudent about referring, because I thought, okay, this is a dangerous surgery. 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 there were two patients at the same time who were really having big styloids and severe EDS and needed craniocervical fusions. They knew each other and were calling themselves Thing 1 and Thing 2. They were having craniocervical fusions, and that was raising some problems about intubation. So I said, all right, I'm going to do it myself. I went to the library the days before and went through textbooks of head and neck surgery. I took my notes. In the operating room that day: Step 1, put the patient on the table. Step 2, turn the — you can't turn anything because the patient was fused. So I threw the notes away and realized the surgery had to be done completely differently.
[1:21:22] A large patient population with Eagle Syndrome has EDS and former fusions. That creates an enormous technical problem. It's like trying to change an engine in a car but you can only open the hood like this. And most of the dissection, instead of doing with tools, I do blind underneath the mandible with my finger, and then I dislocate the thing and carry on.
[1:21:59] The other thing I realized is that by that point I already had years of experience with EDS patients, so I knew they were not tolerating a lot of dissection. I realized what had happened to that ENT in the past — the ENT did the surgery using the Bovie knife, which is the electrocautery.

Pradeep Chopra: Right.

[1:22:24] Paolo Bolognese, MD: The fire stick, we call it in jargon. And you cannot do that around nerves in general, but especially around nerves in EDS patients. So once I pass the skin, I don't use any cautery at all. It's all blunt dissection and nothing else.

[1:22:50] Pradeep Chopra: Makes sense.

[1:22:51] Paolo Bolognese, MD: So far it has worked. That doesn't mean complications will never happen. But that's an example of how to adapt the technique to a specific subset of patients that are strange and different.
[1:23:12] About the diagnostics: you know the problem now. It's obvious when everything is normal, and it's obvious when everything is textbook enormous. The finesse is where in the shades of gray you draw the line. Right now we do a 3D CT angiogram because it shows the structure, length, and thickness of the styloid, and because then we see how the jugular passes by and how the carotid passes by, and how they're affected in 3D. And then we do a glossopharyngeal block.
[1:23:58] But again, before we get to that level, there is clinical. We have a series of all the symptoms linked to the glossopharyngeal nerve — check, check, check. Then there is the provocative test. You push here and the patient winces.

[1:24:15] Pradeep Chopra: Oh my goodness.

[1:24:16] Paolo Bolognese, MD: Or you put your finger inside the mouth and they jump. That means that nerve is hurting, the same way that you would do this to a tooth with decay. The third one is the history — the patient had those symptoms progressively getting worse and so on. Very often it happens after a tooth extraction or other dental work. So there are certain patterns we keep seeing. Then there is the fourth, which is the radiological part — which for once is self-explanatory. And then the fifth, which is the glossopharyngeal block as an invasive provocative test. If whatever symptoms improve, at that point we can link them to the glossopharyngeal nerve, and we can go ahead in removing that bone.
[1:25:18] Now, we do one side at a time, separated by 2 months. So in case there is a problem or a complication, you're not going to have somebody with a tracheostomy and a PEG again like it happened to that colleague of mine.

[1:25:36] Pradeep Chopra: Right. So we've talked about craniocervical instability, we've talked about Eagle Syndrome. Your other area of expertise is Chiari malformation.

[1:25:50] Paolo Bolognese, MD: Yeah.

[1:25:52] Pradeep Chopra: You mentioned cranial settling, and to my understanding, cranial settling is a very big issue in this population. In terms of imaging, what kind of imaging do you want in patients for Chiari malformation? Can we do a supine MRI? Do we need an upright MRI?

[1:26:25] Paolo Bolognese, MD: Okay, first of all, cranial settling is an old-timey term for what, in a simpler way, is the vertical dimension of craniocervical instability. You can have a rotational component, you can have an anterior-posterior component like pure C1-2 instability. The rotational component would be the bowhunter syndrome, in which people do like this and the vertebra gets choked. And then there is the vertical.
[1:26:57] Going back to the morphometrics: the basiodens interval — the normal distance between the base of the skull and the top of C2 — should be between 4 and 5 millimeters. When you go from supine to sitting, you should settle by only a millimeter, millimeter and a half tops. If you extend the head, it should go up 1.5 millimeters tops. That is the normal range.
[1:27:26] 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. You have a sense that something is wrong when in the supine position on a high-definition MRI the BDI is 2 or 3 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 and pull up, if you have a difference between off traction and on traction of 2 millimeters or more, that's vertical instability, cranial settling. That's actually one of the surgical qualification criteria.
[1:28:20] Cranial settling can happen in many different things. Traditionally in neurosurgery it's described in pathologies which destroy the joint — like rheumatoid arthritis, or tumors in the area, or vertical traumas which break down the condyles. But in our patients, the viscosity of the joint is totally altered. It is like you have a piston inside a cylinder — they go up and down, but there is oil and they don't go from one extreme to the other. All of a sudden the viscosity falls. That is craniocervical instability in car terms — it collapses too much and expands too much.
[1:29:39] Different doctors have their own approaches. Generally what I start with is an MRI in supine of 3 Tesla at least. Three Tesla is standard now. 1.5 is acceptable, but 3 Tesla is very easy to find anywhere. Supine position, nothing else, because we have 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 Tesla. The guy who invented the upright MRI was actually one of the very first inventors of the MRI principle and lives near here, and was together with us at our old university. Although an upright MRI is a very good and helpful thing, you don't have a good picture. All of a sudden, all those morphometric measurements — the execution of which relies on crisp definition of bone versus ligament — gets blurred out. That's number one.
[1:30:56] Number two: if you send somebody to do flexion and extension, it is not standardized. If that day Mr. Smith the technician is nervous, he's going to say, okay, move a little bit, move a little bit. But there are two different kinds of flexion. If you flex like this, the stress is on the lower part of the cervical spine. If you flex like this, the stress is on the higher part of the cervical spine all the way to the junction. You can have two people making a major effort but stressing two different parts of the spine, simply because of how the technician does it. The majority of technicians who do MRI flexion-extension are doing it to test degenerative joint disease in the mid-lower cervical spine.
But the most counterintuitive thing — which makes sense when you think about it — is this: if you have somebody who's hypermobile from EDS but has zero symptoms, they're going to give you these scary, contortionist levels of flexion and extension because they have zero symptoms. On the other end, the patient on the opposite side of the spectrum who is super sick would rather get shot than move even just a little bit. So what does this upright flexion-extension MRI give you, even if we get it standardized?
[1:32:53] It can be helpful as a sniper rifle for specific questions — for example, if you want to test whether there is excessive gliding between C1-2 or C2-3. Fine. But as a screening tool, it has all these pitfalls. And realistically, the insurance is not going to authorize multiple MRIs. If they authorize one MRI of the cervical spine, and these patients very often need an MRI of the lumbar as well, and sometimes they have problems inside their head — before you know it, they have lumbar and cervical MRIs for sure. If you also add a flexion-extension upright MRI, they're going to tell you, pick one. And if you pick the upright, you're going to have a poor-quality image of the area you really want to see, with data that are not standardized. 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. If you use it as a screening tool, you shoot yourself in the foot, because then if you really need a good picture, you have to wait until the insurance authorizes it.

[1:34:20] Pradeep Chopra: So for patients with Chiari malformation, what kind of imaging do you want to see?

[1:34:28] Paolo Bolognese, MD: In the past, people were saying, okay, I'm going to get an MRI of the brain because it's a disease of the brain. That's actually not the best MRI to do if you have one shot. The best MRI to do is an MRI supine, high definition, of the cervical spine. Because an MRI of the cervical spine, classically done, shows you the entire posterior fossa and the entire cervical spine. And about 50% of Chiari 1 Malformations have an associated syringomyelia cavity. So that single test puts you ahead of the game.
[1:35:11] Then obviously, once you have that screening, you pass it to a neurosurgeon and they're going to do other things. In the past, when the cine MRI first came out in the late '90s, we were excited — we're going to see the cerebrospinal fluid flow. Fast forward 10 years, and while others were still using it, all the experts dropped it because they were saying the cine MRI, the vast majority of the time, doesn't tell me something I don't know already. If I have a patient with visible herniation and the patient is sick and the symptoms are coming from there, 90-plus percent of the time the cine MRI is going to show me blockage posteriorly. Big surprise — I already knew that. On the other hand, cine MRI can be helpful if you have a herniation that is not particularly deep, with a discrepancy between strong Chiari-like symptoms and an MRI appearance that looks less than what you would expect. At that point, you use the cine MRI as a tiebreaker.

[1:36:30] Pradeep Chopra: So from what I understand, you're looking for a high-definition 3 Tesla supine MRI of the neck.

[1:36:44] Paolo Bolognese, MD: 1.5 is acceptable. Yeah, in cases of Chiari malformation, it's the same image that I need to investigate craniocervical instability.

[1:36:55] Pradeep Chopra: Right.

[1:36:55] Paolo Bolognese, MD: So everything wrong from here to here — MRI cervical supine, 3 Tesla.

[1:37:02] Pradeep Chopra: Now, a lot of these patients with Chiari malformation also present with intracranial hypertension.

[1:37:11] Paolo Bolognese, MD: That's a classic chicken-and-egg situation. First of all, we have to talk about semantics, and there are actually many people in the field who are very loose with the semantics, and they create confusion for themselves and for the patients.
[1:37:22] A lot of people, wrongly so, see the cerebellar tonsils coming down and out of the skull where they belong inside the skull, and they say, that's a Chiari malformation. That's not correct. The herniation of the tonsil is just the anatomical effect of 18 different mechanisms, which can be summarized in 4. You can push the tonsils out of the skull, you can squeeze the tonsils out of the skull, you can pull them down from below, or they can dangle — like the cheeks of a bulldog.
[1:38:22] The classic Chiari 1 Malformation is the purest form: tonsillar herniation caused by a volumetrically small posterior fossa. It's like squeezing a zit. The posterior fossa is a craniosynostosis of the posterior fossa, and the Chiari 1 Malformation is the herniation of the tonsils as its effect. It's a disorder of the skull which is squeezing out the brain. So squeeze is Chiari. Then you can have syndromic Chiari, other forms of craniosynostosis, and the herniation is going to come down.
[1:39:04] Then you can push. If you have a tumor in the posterior fossa, it's going to push everything down, including the tonsils, for the path of least resistance outside of the hole at the bottom of the skull — the foramen magnum, which is Latin for big hole. The posterior fossa skull is normal in size, but there is a big fat mass pushing you around.
Then you can pull down. If you have severe forms of tethered cord — Chiari with tethering, lipomyelomeningocele — it can actually pull the spinal cord down to the pelvis, to the sacrum, at a very young age, and then not only the tonsils but a part of the cerebellum leave the skull and go into the spine. Other times, with a tight filum terminale, people can actually have minimal displacement.
[1:40:14] Now comes the confusion. Some of my colleagues call it Chiari 0, Chiari 0.5, Chiari 0.75. For goodness' sake. Then there is the 5mm rule, which was decided by neuroradiologists who never saw patients and didn't know if the patient had symptoms or not. They decided Chiari malformation was defined by tonsils being 5mm or more below the foramen magnum, but if 4.75mm — no Chiari. What kind of logic is that?
[1:40:43] So practically, you can pull the tonsils down because if you pull down the spinal cord, then the brainstem, the brainstem is attached with 3 prongs. The cerebellum is attached to 3 prongs to the brainstem, and it comes down. The first piece of the cerebellum coming down is the tonsils. So we have push, squeeze, and pull.
[1:41:04] And there is the dangle — people with EDS, saggy things, saggy here, saggy there, the uterine prolapse. It's not a surprise that something can also sag. Now, the gravity of the nervous system is not gravity for all the rest because the nervous system is bathing in cerebrospinal fluid. So there is this buoyant thing — more like a boat that's tied to a dock. You're not a boat in the middle of the ocean. With EDS, you're expecting some degree of sagging. The typical tonsil should be 3 millimeters above the foramen magnum. But you have one nose, I have a bigger nose, she has a cuter nose — same for the tonsils. There is a spectrum, and unfortunately, this spectrum of normal overlaps slightly with the spectrum of abnormal. That's why there are experts out there. But a lot of the confusion comes from the fact that when you start calling everything Chiari regardless of the cause, you're creating confusion.
Chiari was actually first described by a man who was a pathologist. Geographically he was Italian, but at the time he was living in the Austro-Hungarian Empire and was the pathologist in Prague. Back then, what was the major pathology at the end of the 19th century? TB. Tuberculosis. So he was doing autopsies on people with TB. These people were having hydrocephalus secondary to TB infection, and the first herniation he saw was actually the push variety, the squeeze, because the hydrocephalus was pushing things down. He was making a pathology-based, anatomically-based description. Then it became an MRI-based description, but the best ground to stand on is a pathophysiology-based, mechanism-based description — because if you understand the mechanism, understanding what you have to do to fix it is easy. If somebody has a Chiari malformation because of the squeeze, you have to do Chiari surgery. But if it is coming from a push, at that point you're barking up the wrong tree.
[1:44:03] Which brings me to one of the questions I didn't answer before — the intracranial hypertension. That's a chicken-and-egg situation. In some cases, you can have the squeeze-level herniation going down — typical Chiari with mild elevated pressure, which normalizes after the decompression. 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. The pseudotumor is causing the herniation. And then there are situations in which you have both. You have the Chiari, you do the decompression, the patient still has high pressure on intracranial pressure monitoring. The patient has a 1-and-3 situation. In order to — again, pathophysiology — you need to treat both for the patient to have a full recovery.
[1:45:24] In the past, what was called pseudotumor was so named because they didn't have CT scans. People were opening the skull looking for a tumor, and there was none. Then it was thought to be a cerebrospinal fluid disorder because you do a lumbar puncture and the CSF was spraying out of the needle. Then it was understood that the problem was a swollen brain — but swollen from what? Fast forward several years: it was a swollen brain because the venous circulation was choked up. It's easy to understand: if I choke myself like this, my face is going to become purple. The brain is going to bleed in, so to speak. There are only two ways out for blood leaving the brain, which are the two jugular veins.
[1:46:18] Fast forward to a few years ago: pretty much at the same time, Dr. Higgins from Cambridge and myself and another researcher, we each stumbled on a number of patients from different directions. I was looking at styloids, and next to the styloid, there was the tubercle of C1. I was very often seeing the jugular sandwiched between the two. I was removing the styloid, and then sometimes I was seeing the jugular re-expanding and the pressure normalizing. I said, wow, that's great. On the other hand, Higgins at Cambridge was looking at C1. He was seeing C1 like a starfish around a rock, wrapping around the pedicle and the tubercle.
[1:47:13] So long story short, coming from different directions, we hit the same thing — 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 with cranial settling — imagine you have a burger and you push it like this. What does the burger do? Patients with EDS, their C1 ring in chronic settling often becomes wider. And in becoming wider, the pedicles — like elbows going out — go towards the jugular. By doing that, it's the equivalent of putting your foot over a garden hose, and it creates venous congestion inside the brain and the pressure goes up.
So starting last year, when I'm already there for other reasons — for Chiari or for fusions — I just go a little bit more lateral and I can reach those bones and cut them. All of a sudden, patients that had been problem patients for a while — I put a shunt today, then another shunt 3 months later, keeps failing — all of a sudden the pressure normalizes and I felt stupid.
[1:48:36] There was one patient — one of those lost sheep. She was suicidal when we first met her in 2001 because she was having extremely loud tinnitus. Actually, she had two tinniti at the same time. One was high pitch, one was a sort of a low-pitched machinery sound. Constant. She couldn't sleep. It was like living in a factory constantly. She was ready to put an end to her life. She came in, we did a lumbar puncture, pressure was very high. The symptoms improved to the point they were tolerable. So we put in a shunt. And with pseudotumor, the ventricles are very small, so the failure rate is very high. We were doing something like one shunt every year. We tried different shunt configurations, different shunt valves. Technology got better. The headache was better, the ringing was better, but was never zero.
[1:49:50] And then, she was one of the first patients I worked on those tubercles of the C1 ring, and for the first time in her entire life it was gone. It took me 22 years. You kind of feel stupid afterwards. You say, it was there the whole time — why didn't I know this before? But again, you do not know what you do not know.

[1:50:14] Pradeep Chopra: Exactly, and you thought about it. So that brings me to one more question. Dr. Bluestein, do we still have time?

[1:50:23] Dr. Linda Bluestein: Why don't you ask your question, then I have a question, and then we'll wrap up.

[1:50:27] Pradeep Chopra: Okay. We have a subset of patients with EDS that start to have dizziness when they turn their head to one side. What is that?

[1:50:50] Paolo Bolognese, MD: One is easy to find and is called bowhunter syndrome. Bowhunter because you are a hunter using a bow. The two vertebral arteries pass inside C1 and also inside a hole at the level of C2. Because of the hypermobility or the configuration of C2 — it all depends on how much redundancy exists — if C2 between the two holes has a lot of slack and you rotate a lot, the slack can tolerate it for a while. But if you don't have a lot of slack and you overturn, at that point you create a kink in the tubing. If it gets super tense at the level of the two holes, it creates a kink, creates a stenosis, and that causes hypoperfusion from the vertebral artery.
[1:52:05] What's the correction? The only thing you have to do is, as the doctor says, it hurts when I do this — don't do it anymore. So you fuse C1 and C2 so the relative architecture of the two preserves the integrity of the vessel.
[1:52:23] Then there are some other patients with hypertrophic styloids, bilateral, that in their three-dimensional arrangement can actually kink and choke both jugulars. At that point, choking both jugulars creates an intermittent pseudotumor.
And then there are some other patients where, when they turn, they can have a more complex structure in which different cranial nerves are involved. You can have the 10th cranial nerve, or the 10th and 9th, which are functionally connected — you can use one as a backdoor for the other. But at the end of the day it is this stupid thing: it is something mechanical that is affecting something else.

[1:53:36] Dr. Linda Bluestein: And that leads me to my question, which is: hopefully people by now are realizing that there is a lot that happens in the neck and the base of the brain, and these workups are very, very complex. I think most people don't realize that you don't want to have a surgery that you don't need. Some people think, oh, that seems like an easy fix — if that's the definitive fix for CCI, don't I want that? 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 has ever worked me up for Eagle Syndrome or bowhunter syndrome or any of these other things.
[1:54:26] Do you have any suggestions for people who — as Dr. Chopra pointed out — are literally all over the world, listening to this and saying, gosh, I wish I could schedule an appointment with Dr. Bolognese or get some kind of screening. But for people who 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 that people can have something that looks like Chiari but actually only one of them actually is Chiari. Those details are so important.

[1:55:02] Paolo Bolognese, MD: Yeah, the key is to find somebody locally — like you — the EDS specialist, the aficionado, the EDS concierge physician, whatever you want to call it. It doesn't really matter if you're a neurosurgeon, a pain specialist, a pediatric cardiologist, a physiatrist, et cetera. There are so many of us with a major overlap of diagnoses. The important thing is to find somebody local.
[1:55:49] In the United States, it's absolutely not a problem because right now there is not only raised awareness, but a lot of people going into the field. That's very good. The risk for neurosurgical colleagues — if there's a neurosurgeon watching this — I would 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. Don't make the mistake of pushing the pedal from the start, otherwise you're going to have eggs on your face. At least in the beginning we didn't have anybody to ask, but at least ask somebody who has more experience before you commit to a large volume of patients. Otherwise you're going to quit. It's a better strategy for a neurosurgeon to go slow and have a long period of learning. The real problem is in other countries, but the good news is that things are changing there too.
[1:57:09] The United Kingdom decided in March of this year to create 3 multidisciplinary centers for neuro-EDS. That was one of the bastions of skepticism worldwide. Australia has one, Italy has a couple of centers for neuro-EDS diagnosis, and they're starting to explore into that. Spain has a couple of specialists dealing with that. Holland started and stopped and probably will restart again. You cannot expect that somebody who's just starting is going to have the same kind of results as somebody who's been 10 or 20 years in the field. But we are not anymore the way we were 20 years ago. So I'm very, very optimistic.
[1:58:09] Finding somebody with a good reputation is very, very important. I would always start from a primary care neuro-EDS physician before committing, because people like you guys are going to be the quarterback. Neuro-EDS is going to need, at a certain point, a SMAS procedure, a kidney transposition because of a May-Thurner, or an orthopedic repair done at Harvard by my friend who does ligament reconstruction, or a TMJ procedure done in Indiana. People like you know who the players are and where to send them. You are the playmakers. We are just the wide receivers — one of many.

[1:59:17] Dr. Linda Bluestein: Very good. 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?

[1:59:45] Paolo Bolognese, MD: The first one is actually a negative one, not a positive one.

[1:59:50] Dr. Linda Bluestein: That's okay.

[1:59:52] Paolo Bolognese, MD: 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 to make patients more educated. But sometimes things get out of hand. I see a lot of patients out there playing morphometrics with each other, playing doctor on each other, or rendering opinions based on this and that. I'm not saying that I'm super smart and nobody else can deal with this. But it's very dangerous to play doctor, and for any kind of pathology this field is very complicated. Even it's difficult for us to do a nuanced diagnosis. For sure, patients should not do it, even if they know more about neuro-EDS than the average primary care physician at home. EDS from standard training is a footnote of one page in the internal medicine manual. They're just giving each other a disservice.
[2:01:29] Second thing: right now we are finally coming to the peak of being able to convince a larger physician community that these patients are legitimate and this subdiscipline is legitimate. If we have a bunch of patients playing loose with diagnoses, they're going to give the entire cause a bad name — to critics who are critics in good faith, and to critics who are just looking for an easy target to shoot and say, see, I told you this is a bunch of nonsense. So that is my negative thing.
[2:02:13] About the positive hack: I would recommend the principle of kicking the tire. If you have a suspicion about something, just kick the tire. If you think you have an instability, put yourself in traction, put yourself in a cervical collar, even if the two things are not equivalent. And the important thing is not to do it just once, because you could have a placebo effect. Placebo effect: you do it and you really want to see something. It is like marrying somebody when you're infatuated — after 20 years, if that person is really not right, the placebo effect is gone. So before jumping to a conclusion after one non-invasive self-administered test, just check if the results are consistent. And then at that point, get the yellow flag up and look for help.
[2:03:21] Right now there are people dealing with neuro-EDS, but we're not that many. It is against the public interest to flood the available resources with patients who, if they had just tested themselves 2 or 3 more times, might have found that the result was not that consistent. Provocative testing and repetition go a long way.
[2:04:05] Another negative hack: do not use the term migraine. Migraine is a specific diagnosis. It means visual aura followed by headache, with head pain only on one side of the head, and you had to go into a dark room for 3 days and it responds only to a specific set of medications. That's it. That's migraine. Don't call migraine everything else. Otherwise, number one, you create confusion for a doctor — a primary care physician with 5 minutes to see you is just going to write in your medical record that you have migraine, while that is not the diagnosis. Telling someone where your headache is, how many times it happens, what makes it worse, and what makes it better — those are probably the two most important things.

[2:05:00] Pradeep Chopra: Yes.

[2:05:01] Paolo Bolognese, MD: Because that gives the diagnosis away before you even see the MRI. One of the exercises I do with my team is we read the questionnaire, the history, and the provocative test results without seeing any MRI or any former diagnosis from a specialist. Three things only — old school medicine, 19th century — and we call the diagnosis.

[2:05:31] Pradeep Chopra: That is good.

[2:05:32] Paolo Bolognese, MD: So no more migraine as a catch-all diagnosis — it's just going to confuse people.

[2:05:39] Dr. Linda Bluestein: Right. Yes.

[2:05:41] Paolo Bolognese, MD: And also, to have a healthy sense of skepticism. Don't fall in love with a diagnosis, but keep a balance. Doubt yourself before you go from 0 to 100.

[2:05:55] Pradeep Chopra: Okay.

[2:05:56] Paolo Bolognese, MD: Because if it is true, it's going to be true anyway at the end. Repeat the test, repeat the test, repeat the test. Don't jump to a conclusion after 1. If after 10 repetitions it is consistently the right thing, guess what? 19 out of 20 are going to be positive.

[2:06:18] Pradeep Chopra: So as we try to wind up, I have a tiny little hack. I'm addicted to hacks. A lot of times patients with craniocervical instability have difficulty sleeping because when they turn sides, it wakes them up. What I recommend is using one of the inflatable airline pillows that you put around your neck and you can sleep with that on. It prevents your head from rolling when you go to sleep.

[2:07:02] Paolo Bolognese, MD: Good. And I'm going to add one, which is a positive hack since I've been so negative so far. People with high-pressure headache — which gets worse when you cough, sneeze, or strain —

[2:07:17] Pradeep Chopra: Yes.

[2:07:17] Paolo Bolognese, MD: Very often they sleep on 3 or 4 pillows, which is good in the beginning of the night. And then they wake up and they've slipped off the pile because the pillows fall down. So the best thing to do is not to use pillows, but to use a wedge underneath. Not everyone can afford an adjustable bed, but just get a big wedge — it can be wood underneath the mattress, or just some firmer material cut into a wedge — and put it underneath the head of the mattress. That's going to be more effective and more consistent than a bunch of pillows.

[2:07:55] Pradeep Chopra: All right. So you get the award for the hack of the day. With this, we are so appreciative. This has been one of the best podcasts I've ever been part of, and it was so valuable. We thank you. I know you had a long day in the operating room today, and it is late for all of us. I can't tell you how appreciative we all are. I'm sure our listeners are going to feel the same, 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 on the Bendy Bodies Podcast and sharing your knowledge with us.

[2:08:57] Paolo Bolognese, MD: Thank you for having me.

[2:08:57] Pradeep Chopra: Very appreciative.

[2:09:00] Dr. Linda Bluestein: Yes, we are so grateful. This is such an important conversation and one that I think so many people are going to benefit from. So we really appreciate it.

[2:09:10] Paolo Bolognese, MD: You're going to add subtitles, obviously.

[2:09:12] Dr. Linda Bluestein: And thank you so much to Dr. Chopra for being the most amazing guest co-host. Really appreciate it.

[2:09:20] Pradeep Chopra: Oh, thank you.

[2:09:22] Paolo Bolognese, MD: Dr. Chopra — when I met him, I couldn't believe it, because for years 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 I had been preaching to for years and years. After a little while, he was better than them. And I was like, how is it possible? He's not a neurosurgeon — I was being very elitist. And then I realized that he was just unique.

[2:09:58] Dr. Linda Bluestein: That's incredible. Because a lot of people probably don't realize that a typical neurosurgical residency in the United States is 7 years — and that's after 4 years of college and 4 years of medical school, and then a 7-year residency. Is that right?

[2:10:13] Paolo Bolognese, MD: I did it twice, so I finished at 41. Yeah, age 41, I finished. Somebody says they did it twice because he was not that smart.

[2:10:24] Dr. Linda Bluestein: 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. I will also put this in the show notes, and I will definitely encourage people to watch this on YouTube where they can see the subtitles. We will have the transcript available as well so they can read along and search for the specific things they want.

[2:10:55] Paolo Bolognese, MD: Since we're talking about a questionnaire — and since my questionnaire is open source — I'm going to send you the form that we use. Not only for the patients to see, but also very importantly for any practitioner who wants to use it or copy-paste or modify. This is for everybody to benefit from what we have learned over 20-plus years. They start from where we ended.

[2:11:28] Dr. Linda Bluestein: That is incredibly generous. Thank you so much. We will link that so people can access it. You're sharing your knowledge and resources, which is phenomenal. So thank you so much. And we'll see you next time on the Bendy Bodies Podcast.

[2:11:43] Paolo Bolognese, MD: Thank you.

Pradeep Chopra: Bye-bye.

[2:11:48] Dr. Linda Bluestein: Thank you for listening to this week's episode of the Bendy Bodies with the Hypermobility MD Podcast. Visit our new website at bendybodiespodcast.com where you can now view guest profiles and show notes with links to products and journal articles. Leave me a comment, sign up for updates, leave a review or a voicemail, and access the podcast on your favorite player, all directly from our website. You may hear your voicemail in a future episode where we answer your question or dive into your gracious feedback. Follow us on Instagram at bendy_bodies. We love seeing your posts and stories, so be a buddy and engage our community by using the hashtag #BendyBuddy. That's hashtag B-E-N-D-Y-B-U-D-D-Y. You can also find me, Dr. Linda Bluestein, on Instagram, Facebook, Twitter, or LinkedIn at @HypermobilityMD. Visit hypermobilitymd.com for information about medical services and one-on-one coaching. This podcast is for general informational purposes only and does not constitute the practice of medicine or other professional healthcare services, including the giving of medical advice. No doctor-patient relationship is formed. Do not disregard or delay obtaining medical advice for any medical condition you have. Opinions shared are that of the guest and do not necessarily represent the views of the host or any particular organization. Thank you for being a part of our community, and we'll catch you next time on The Bendy Bodies Podcast.