Examining Head, Spine, and Chest Pain with Pradeep Chopra, MD

[00:12] Jennifer Milner: I think all EDS patients have pain, but at times this pain will go up to a point where they become non-functional, and that's when they start seeking treatments. But they have pain all the time. It's just that they've grown to accepting it as, hey, this is normal.

[00:42] Jennifer Milner: Welcome back to the Bendy Bodies Podcast, bringing you state-of-the-art information to optimize your health. This is co-host Jennifer Milner, a former professional ballet and Broadway dancer who struggled for years with hypermobility-related problems. Now I train dancers to ensure the next generation of hypermobile artists are better equipped to work to their fullest potential.

[00:59] Dr. Linda Bluestein: I am Dr. Linda Bluestein, the Hypermobility MD. I started Bendy Bodies to provide accessible information for everyone on the hypermobility spectrum. Combining my medical education and personal experiences enables me to treat and coach patients and clients to optimize their quality of life. This information is for educational purposes only and is not a substitute for medical advice.

[01:16] Jennifer Milner: Today we wanted to talk about the problems that can occur in the head and spine and neck and chest, which sounds like we're just kind of throwing a dart at different parts of the body. But they all are kind of connected. And this series that we're doing with Dr. Chopra is really trying to break it down into parts of the body rather than specialists, because there's so much overlap between issues within one certain area of the body that they're very difficult to parse apart on their own. So we thought this would be a helpful way to look at it.
[01:38] So when we're looking at the head, the spine, the neck, the chest — Dr. Chopra, why are these topics so important when we're talking about EDS?

[01:47] Pradeep Chopra, MD: I call this connecting the dots, Jennifer, in the sense that you have to look at the human body as a whole. I can't have a patient come into my office with one complaint and then not relate it to other things that are causing it. And so it's important to understand that.
[02:07] Just to make sure that we don't miss anything, the way I like to do it — and I think the way most doctors do — is they start from the head and then they walk all the way down to the toes. That's how we teach our medical students. We'll say, look, there are two ways to go about it. One is you can go physically from head to toe thinking of all the possible causes of the condition and then rule them out, or you can go by systems. And over the years, I've found that going anatomically from the head down to the toes is the best approach. And patients understand that easily.
[02:40] Hopefully I didn't miss any organ by the time I finish. I kind of jumped out of my usual norm of going from head to toe when we had our first podcast on abdominal pain, because I was hoping it would be just one abdominal pain podcast. But here's Dr. Bluestein and Jennifer Milner who grabbed onto me and the rest of it, which is fine, because I want people to understand on their own and not be solely dependent on doctors to diagnose them, or at least know what they have — and understand whether it's a serious thing or not. For example, brain fog can be a little scary, but once they understand why they have brain fog, it's a little reassuring. So that's why we are going from head to toe. And on this podcast, we'll go from the head to the neck and possibly even the thoracic spine.

[03:38] Jennifer Milner: Yeah, that sounds great. So how about we dig in?

[03:40] Pradeep Chopra, MD: So I'm going to start with headaches. Headaches are really very common — again, common in the non-EDS population as well as the EDS population. I've sort of teased out what's more common in the EDS population, and it turned out to be about 25 different reasons for having headaches. I teased out the very rare ones down to the more common reasons. I'm going to list them and then discuss all of them later.
[04:08] The common causes of headaches — the first one is migraines. I don't know if there's any relationship between migraines and EDS, but we do see a lot of migraines. The second one is Chiari malformation. Then we look at cervicogenic headaches, headaches from what are called craniofacial pain or TMJ or temporomandibular joint dysfunction, headaches from POTS — postural orthostatic tachycardia syndrome — headaches caused by tethered cord syndrome. Then you have the two culprits: high pressure headaches, also known as intracranial hypertension, and low pressure headaches, usually because of spontaneous CSF leak. And then you also get headaches from craniocervical instability. As we go along, I'll discuss the symptoms of each one. I cannot go into treatment in depth on these because a lot of that is based on the patient's presentation.
[05:00] So, going to the first symptom: patients complain of pain in the whole head, they have double vision, they feel a throbbing in their ears, and their headache increases when they laugh or cough. Just to remind you — this is really important. They may be having a headache and if they laugh or cough or sneeze, their headache increases significantly and then drops down to a very baseline severe headache. They also have this pulsing ringing in their ears. And sometimes, not always, they may have double vision. These are symptoms of increased pressure inside the head.
[05:37] Now, the thing with the head is that it's a closed structure — it's a box, a round box, and it's closed. It doesn't expand. And so if the pressure changes either way, whether it increases or drops down, it becomes very symptomatic. Raised pressure inside the head can be from many different reasons. The big one being the headache increases with coughing or sneezing. The headache also feels different — like the head is going to explode from inside. It's a very intense pressure feeling inside the head.
[06:14] Among the common reasons for this kind of headache from increased intracranial pressure is narrowing of a blood vessel inside the brain. It's called venous sinus stenosis, and usually it's the transverse venous sinus stenosis. What it means is that one of the veins is narrowed. The theory behind why you would have a headache from a narrowing of a blood vessel inside the head is that the amount of blood that goes inside the head should be exactly the same that comes out. If there's plenty of blood going inside the head but not the same amount coming out, that adds to increased pressure. And that can be from a narrowing of a blood vessel.
[06:55] The second reason is Chiari malformation, and we'll discuss that when we get to it.
[07:02] How do you diagnose this? Let me tell you how you don't diagnose this, and that is a spinal tap. Absolutely do not get a spinal tap, and I'll explain why. The fluid inside the head is called CSF. This CSF is produced inside the brain, then flows out of the skull and down into the spine, all the way down, and then goes back up into the skull — it keeps circulating within a closed system. Now, a spinal tap is a lumbar puncture. They stick a needle into your back and look at what's called the opening pressure, and with that they decide whether the pressure inside the head is high or not.
[07:45] There are two problems with that. One is: what if there is an obstruction of fluid at the level of the neck? For example, in Chiari malformation, the pressure inside the head is very high, but that high pressure is not transmitted down to the spine. So if you do a spinal tap in these patients, the pressure is going to show up as normal. The second problem is that in patients with EDS, the covering — the bag that holds this fluid — is very elastic. It's like measuring pressure inside a thin-walled balloon. You're not going to get the right pressure because the covering in which the fluid stays, called the dura mater, is very thin. You're not going to get a good pressure reading. The third reason, of course, is that if you do a spinal tap, the hole does not close up, and you wind up with what's called a post-dural puncture headache, which is even worse than the regular headache. But that's besides the point. The point is that the pressure inside the head may not match the pressure in the spine.
[08:50] So how do you solve this problem? The best way is to measure the pressure inside the head directly. That's done with a very small procedure called putting in a subarachnoid bolt. It sounds gruesome — it's not really a bolt, but it's a little transducer that measures pressure. The surgeon makes a tiny hole and puts it in there, and that goes to a computer which records the pressure continuously. The patient stays in the ICU overnight and they look at the pressure readings over time inside the skull. That is a far more accurate way to test pressures inside the head.
[09:26] So, doing a spinal tap is a bad idea — or I should say not a good idea. You can do what's called an MR venography: they inject some dye into your vein and then look at the map of that dye in the brain to see if there's any narrowing, which I think is a really safe and valuable technique. If you suspect the cause is Chiari malformation, you can get an upright MRI.
[09:56] Now, I've had neurologists say, well, this cannot be high pressure inside the head because there are no eye changes. If the pressure inside the head stays very high for a long time, then you start to see eye changes. Obviously, because everything is under high pressure — but you don't want to wait for that. That's a complication. You want to fix this before any eye changes happen. So it's not a good idea to wait for a change in your eyes before looking for high pressure inside the head.
[10:32] How do you treat this? In medicine, there is only one rule: you treat the cause of the problem. Everything else is a Band-Aid. So if there's high pressure in the head and it's because of a narrowing of a blood vessel, they sneak a little stent in there to open up the blood vessel. They don't open up the skull — they do it through the arm or through the leg and get into the brain and place a little stent. If it's Chiari malformation, then they do a decompression surgery.
[11:02] In some cases, you never find the cause of high pressure, which is called idiopathic. In that case, they do what is called a shunt. They put a little device through the skull, and it drains the CSF through a little tube hidden under the skin and into the belly, where it keeps draining. And believe it or not, it's computer-driven — they can use a little remote to control the amount of pressure being lowered. They don't want it lowered too much, so they can do that.
[11:41] So these are the reasons why patients with EDS can have headaches from raised intracranial pressure. Just to recap: the most common cause is a narrowing of a blood vessel or Chiari malformation. Doing a spinal tap is a bad idea. MR venography is good. And if you have symptoms of Chiari malformation, which we will discuss a little later, treating it makes a big difference. I forgot to mention there is a pill you can take, but from practical experience I really haven't found it to be very useful. It does help in the initial stages and then doesn't really help. But just be careful — you don't want the pressure inside the head to be too high.

[12:21] Jennifer Milner: What's really interesting to me about this conversation is that you started out saying there are about 25 different causes of headaches, and you sort of narrowed those down. I think the average person would say there are like 3 types of headaches: a sinus headache, a tension headache, and migraines — that's just kind of what we've heard from over-the-counter drugs. So just hearing that there are so many types and they can be caused by so many different things — it has to be comforting.
[12:44] Because a lot of times we go to the doctor with a headache and they're like, does it go over the head or the side of the head? Okay, then it's this. And I know some people with EDS with chronic headaches where it's pretty much daily. Being told you're not crazy, there are a bunch of different things it could be, let's dive a little deeper and see what the cause could be — as you said, everything else is a Band-Aid. So trying to get to the cause of it rather than just, have you tried some over-the-counter ibuprofen, is hugely comforting. So that's really interesting to hear. Thank you for that.

[13:14] Pradeep Chopra, MD: Actually, you said most people are told there are 3 types of headaches. I can shorten it down to 2 types of headaches: either your headache is from inside your head or it's from outside your head. And that's important because if it's inside your head, it's most likely a migraine, and the treatment is completely different. If it's from outside the head, then there are other reasons. But in any case, moving on to headaches — so that our listeners don't get a headache from listening to me.

[13:43] Dr. Linda Bluestein: Before you move on, I was curious about Eagle syndrome — would that be included in that first section?

[13:49] Pradeep Chopra, MD: Yes, ma'am. Eagle syndrome is right inside this.

[13:52] Dr. Linda Bluestein: Okay, great.

[13:54] Pradeep Chopra, MD: There are a couple of other reasons for headaches which I forgot to include in my list. TMJ, temporomandibular joint, can cause headaches. And sinus headaches — frontal sinus, that's a headache in your forehead — are also very common, and the reason for that is mast cell activation syndrome. So when we get to mast cell activation syndrome in the 300th episode of our podcast, we'll talk about that.
[14:21] So, one of the headaches I always ask patients about is: does your headache get worse when you stand? And a lot of them will say yes. But then the critical question is: when you lie down, does it get better? They'll think about it and say yes. And that's when I ask them: does it only get a little better, or does it go away? There's a big difference. So we're going to talk about the one that goes away. The minute they lie down, it goes away, and the minute they stand up, it comes back. These are symptoms of a low-pressure headache.
[18:44] When the pressure inside the head is low, the brain kind of settles down — the term for that is boggy brain. That's actually what they look for on MRIs of the head in these cases: a boggy brain, which sounds a little gruesome, but it's literally the brain sort of falling down because the pressure isn't enough to hold it up. It's a very intense headache when they stand up, and the best part is it resolves fully when they lie flat.
This is often because cerebrospinal fluid inside the head is leaking out for whatever reason. One of the reasons it would leak out is if somebody went in there and did a spinal tap to look for a high-pressure headache, and now you have a leak which adds on to the headache you already had. What's happening in these cases is there's a hole in the dura mater — remember, the covering in which the fluid is held — and it's slowly starting to leak out. When you stand, it leaks out more. When you lie down, it doesn't leak as much.
The treatment for this is fairly simple. They do what is called an epidural blood patch, or they might do an epidural fibrin — basically glue that closes up the hole. If it is because of surgery, like somebody's had spinal surgery and they wind up with a post-dural puncture headache or a low-pressure headache, and none of these methods are working, then they go in and do a surgical repair.
But things that you can do at home — didn't we talk about hacks in our last episode?

Jennifer Milner: Yes. And people love them.

Pradeep Chopra, MD: We're going to have the hacks. So this is a hack.

Jennifer Milner: Give us a hack.

Pradeep Chopra, MD: So the trick for this is that you drink a boatload of caffeine. That helps. If you can't drink a boatload of caffeine, then you can get caffeine tablets — the most common one being Excedrin Migraine, and that has a lot of caffeine in it. That might help. The other one is to wear a corset around your belly. That puts pressure on the fluid. It doesn't stop the leak, but what it does is increase the pressure inside the CSF. They do say drink a lot of fluids, but I don't think that really works because the headache is so intense that it causes nausea, and that doesn't work. But we have our hacks.

Jennifer Milner: Well, and this is something that you mentioned in the abdominal pain episode — it sort of links to something you discussed there. You talked about how with connective tissue, things can start to just fall into gravity, and if they shift down just a little bit it can cause significant issues. And it's replaying itself in another part of the body here as well. You're talking about boggy brain — if things just settle a little bit, they can cause significant issues. And so I think it's yet another example of how loose or different connective tissue is not just about the joints, and that it has such ramifications. As you were saying, the dura mater is so thin. And if there's low pressure for whatever reason, there's going to be issues, as we've seen with some pretty stellar headaches. So it's really not just about the joints — it affects absolutely every single part of the body.

Pradeep Chopra, MD: Exactly. But I'm so excited that we got our two hacks in.

Dr. Linda Bluestein: And I have a question before you move on — and maybe you're already going to get to this — do CSF leaks ever spontaneously close? Is there a chance it would spontaneously close?

Pradeep Chopra, MD: Dr. Bluestein, I know you've seen lots of CSF leak headaches, and it does spontaneously close in some patients. But not in EDS. These don't let go easily.
There's one small point I wanted to bring up, which is not a phenomenon where a person can have a high-pressure headache and a low-pressure headache at the same time. What happens is when the pressure inside the brain starts to increase so much, a leak develops in any part of the dura mater — it can happen in the spine, it can happen in the brain. A leak develops and CSF starts to leak out from there, and that lowers the pressure. So it's a kind of self-sustaining mechanism where the pressure goes up, a leak develops, and the pressure now drops down to normal.
[19:09] Sometimes patients will complain of a very clear fluid draining from their nose. Up in the nose there's a bone where a leak can develop, and this fluid is very different — literally like water, even thinner than water. It has a slightly salty taste. It's different from mucus. Patients will tell you it's like water dripping down a faucet, and then it stops, and then it comes back again. And that fluid can be tested for CSF. There's a test called the beta-2 transferrin test. You just bend down, let some of it drip into a little bottle, and send it off to the lab. So just because someone has symptoms of a high-pressure headache or symptoms of a low-pressure headache, you really have to look at who's the culprit. Does this patient really have a high-pressure headache which is ending up in a low-pressure headache?
[20:15] So the next one I want to talk about is Chiari malformation. In this case, again, the headache gets worse when they cough or sneeze — remember how I mentioned that in raised intracranial pressure headaches? Here with Chiari, the headache also increases when they cough or sneeze. They have tingling in their hands and feet, and difficulty swallowing. And this is just the basic head-related stuff — I'll get into a lot more about the other symptoms of Chiari malformation.
[20:47] So for those who have not Googled it yet, here is a little education on what Chiari malformation is. Your brain lives inside a skull, and under the bottom of the skull is a hole, and through that hole the brain comes out. At that point it is now called the spinal cord, and the part at the hole is called the brainstem. So the brainstem is at the hole, and then it comes out through the hole, and that becomes the spinal cord.
[21:14] This hole is really important because in Chiari malformation, for whatever reason — mostly because the skull is a little deformed, though you can't see it — the brain starts to push down and it pushes through the hole. When it pushes down, the brainstem, which is at the beginning of the hole, plugs that hole. And along with that, there is an organ at the back of the head called the cerebellum that also gets pushed down and squeezed. So in Chiari malformation, not only do you have the brainstem plugging the hole, the cerebellum also plugs the hole. Once the hole gets plugged, the CSF cannot leave the skull and go into the spine. It starts to collect inside the head and the pressure inside the head starts to increase. And this is the point I was trying to make before: if you do a spinal tap in this patient, it will be normal. But when you do a BOLT in these patients, it's going to show us high pressure.
We also need to know what happens when the brainstem is compressed and when the cerebellum is compressed. These patients obviously present with neck pain. But one of the most important things is they present with balance problems. The cerebellum is responsible for balance, so these patients have really poor balance. They often complain of a pressure headache in the back of the head. It's associated with POTS-like symptoms.
[22:40] When we get to POTS, there are 3 reasons for patients having POTS. One is, of course, blood pooling down their legs. The second reason is your brainstem being compressed, which causes symptoms of POTS. The third is an autoimmune dysfunction. So this is one of the reasons why they have POTS. And in my office I have to differentiate, because if they have POTS because of their brainstem being compressed, then no amount of salt or fluid is going to fix it — you've got to fix the Chiari malformation.
[23:09] They do have difficulty swallowing — the way to ask patients is, does it feel like your food is getting stuck in your throat? They have poor hand coordination. So these are some of the main symptoms of Chiari malformation. There are many others, but these are the key ones: pressure in the back of the head, balance problems, high-pressure headaches, POTS-like symptoms especially dizziness, and difficulty swallowing.

[23:36] Jennifer Milner: For people who are looking at this and trying to diagnose it, I know that Chiari malformation is best diagnosed with an MRI, but it's a certain position for the MRI, right? Isn't it seated, or am I thinking of something else?

[23:47] Pradeep Chopra, MD: Oh yes, that's an excellent point. I forgot to mention that.
[23:51] So I have to back up a little bit. One of the phenomena that happens in patients with EDS is the head sits on the spine, balanced on the spine. The analogy — and this is a true analogy — is that balancing a bowling ball on the end of a pin. That's exactly how much the head weighs, about 11 pounds, same as a bowling ball balanced on the tip of a pin. That's how delicate this part of the anatomy is. And this is all held down by ligaments. Ligaments in most people are strong, so when I sit, I'm not really using my muscles, I'm using my ligaments. But in EDS, because the ligaments are lax, the bowling ball — the head — settles down. It descends a little bit. And that's called cranial settling.
[24:56] As the skull settles down, it presses on the cervical spine — the neck bones. And when it presses down, that's when you get a lot of the symptoms. You don't see this cranial settling when you're lying down. It shows up only when you're upright. Hence, getting an upright MRI in these patients is important, because you want to see the cranial settling. You want to see what happens when these patients sit up. So the diagnosis depends a lot on upright MRIs — it may not show up on a supine MRI. Upright MRI centers are not that common, but they are around the country. It's kind of a sitting MRI — you sit and they take a picture.

[25:46] Jennifer Milner: Yeah, that makes sense, because that was also something we discussed — I think Dr. Bluestein, when we talked about cervical instability and the importance of having the upright MRI for that. So again, it makes sense that if your ligaments don't do as good a job supporting you against gravity, having to fight gravity would be crucial for people to get a true picture of what's going on.

[26:03] Pradeep Chopra, MD: Right. And thank you for bringing that up, because I completely forgot about that. So getting an upright MRI for the diagnosis of Chiari malformation is sort of critical.
[26:09] The treatment is, of course, surgical decompression. What they do is they widen the hole a little bit — that's basically what they do.
[26:21] The other type of headaches you get are from clenching your teeth a lot. They call it temporomandibular joint dysfunction, but this is more of a habit. I don't think this is very particular to patients with EDS — we do see it in our non-EDS patients also. You clench a lot. And clenching can actually be helpful in some ways: when you lift a heavy item, you sort of clench your teeth and get that extra strength. And also when something hurts, you clench your teeth. So there's a lot of clenching going on.
[27:02] The clenching is done predominantly by the chewing muscles called the masseter and the temporalis muscles. The masseter muscle lives at the back of your jaw, just below the ear — you can feel it expand or contract if you clench your teeth. And the temporalis muscle is on the side of the head. These muscles get tired of constantly clenching and they start to hurt, and that adds to another headache.
[27:27] The treatment for that — there are many ways, from the simplest to the most complicated. And there are very few practitioners who actually know how to treat it. A mouthguard is not a good option because you're still going to be clenching on the piece of plastic in your mouth. If anything, if the mouthguard is ill-fitting, you're likely to clench even more. So we don't really recommend a mouthguard. It does protect your teeth, but that's about it.
[27:52] What I like to do is shoot some Botox into the masseter muscles and the temporalis muscle. A little bit of Botox goes a long way in these patients. And if you really want to get fancy, there are what are called oral appliances where when you clench, you're clenching on air rather than on teeth.

[28:08] Jennifer Milner: And I think, Dr. Bluestein, when we talked to Dr. Russak about jaw issues, she said that sometimes in her experience, people with hypermobility clench their jaws out of proprioceptive issues — just so their jaw knows where they are in space. And the physical therapy work that could be done to get out of that and relieve some chronic headaches was really fascinating to think about.

[28:29] Dr. Linda Bluestein: Yeah, I actually started to chuckle when Dr. Chopra mentioned the jaw clenching because that has been a huge issue for me. My biggest problem over the past few years has been jaw pain, as Jennifer knows. So talking and smiling and all of that can be a problem. And my oral surgeon — a dentist who is now a maxillofacial pain specialist — has really tried to educate me about the clenching. I can't tell when I'm doing it. But apparently when you clench, you transmit forces into the jaw. We shouldn't be touching our teeth more than — I forget the number — but yeah, this whole clenching thing, I think probably a lot of us do. And we're looking for stability and that proprioceptive input.

[29:12] Pradeep Chopra, MD: So here we come to our hack section of the podcast. Yay!

[29:16] Jennifer Milner: Hack alert!

[29:16] Pradeep Chopra, MD: You guys should have a drum roll.

[29:18] Dr. Linda Bluestein: We do.

[29:18] Pradeep Chopra, MD: We need it.

[29:18] Jennifer Milner: Yeah.

[29:20] Pradeep Chopra, MD: So hacks for this. One is that when you feel yourself clenching — firstly, take it out of your subconscious mind and put it into your conscious mind, which means you've got to always keep thinking about it: I'm not going to clench. And if you do find yourself clenching, open your mouth a little bit, sort of just like a bird. Or you can stick your tongue between your teeth, and then in 2 minutes it'll be gone.
[29:51] Sometimes I've noticed this when people are walking up the stairs and their knee hurts — they tend to clench. That's when I tell them, start by opening your mouth a little bit.
[29:56] And I think there should be a double drum roll for this hack: you know how when you have to take a flight early in the morning, like at 5 AM, and you set your alarm for 3 AM, and then you wake up exactly 10 minutes before the alarm goes off? That happens — at least with me, it happens a lot. The reason is you've been thinking about it all day long: I've got a flight at 5 o'clock, I really need to get up at 3 o'clock. And so when you go to bed thinking about that, the brain beats the alarm clock by 10 minutes. It's a very common phenomenon. It doesn't happen on a daily routine basis — if you have to wake up for work, it doesn't happen because you're not thinking about it. But when there's a singular episode, there's something important you need to do at 4 o'clock, you wake up 10 minutes before.
So the hack is: start thinking about it — I should not clench when I sleep, I should not grind my teeth, I should not clench. Keep thinking about it, and eventually you'll stop clenching your teeth. They have to drag it out of their subconscious mind and bring it into their conscious mind — make it the most important thing in their life: I am not going to clench anymore. When you go to bed, think about it a few hours before. And if you're thinking about it during the day, then again, if you clench, open your mouth a little bit or stick your tongue between your teeth. Hence the double hack.

[31:55] Dr. Linda Bluestein: Those are excellent double hacks. What about medications like SSRIs — can't that influence clenching also?

[32:02] Pradeep Chopra, MD: I haven't heard of that. SSRIs in general don't help pain. They do help with anxiety and depression. And when you're anxious, we do tend to clench, so in some form it might help. But that is a whole different issue — if you have tremendous amounts of anxiety, you need to be on an SSRI. In that case, of course, you can go on to that.

[32:25] Dr. Linda Bluestein: Yeah, I think — from this doctor that I've seen — she was saying that there's some data showing that SSRIs actually can increase clenching.

[32:33] Pradeep Chopra, MD: Oh, okay. I had no idea.

[32:34] Dr. Linda Bluestein: But of course, we don't want anyone to stop their medication based on what I just said.

[32:39] Jennifer Milner: Right.

[32:39] Dr. Linda Bluestein: A lot of these things are multifactorial. But those are great hacks, because anything we can do with a less risky intervention like that — there's no downside to bringing it into our consciousness. Those are fabulous hacks and double hacks.

[32:55] Pradeep Chopra, MD: Listen, I am a cheap guy. I like to do cheap things. Dragging something out of your subconscious mind and bringing it into your conscious mind is extremely cheap.

Dr. Linda Bluestein: Yeah.

[33:02] Pradeep Chopra, MD: And keeping your beak open when you think you're going to be clenching is pretty easy. It may look silly walking around with your mouth open, but it's only for a few minutes.
[33:15] So let's talk about craniocervical instability, otherwise briefly known as CCI. Cranial is the head, cervical is the cervical part of the spine — the neck — and there's an instability there. This is fairly common in EDS.
[33:34] Remember I told you how it's the equivalent of a bowling ball balancing on the end of a pin? All of this is a marvel of anatomy where everything is so well-balanced and held together that we can walk around with a bowling ball on our spine and still turn and look around and nothing falls off. But if something goes wrong in this intricate mechanism, everything falls apart. The problem is that the ligaments that hold the head and the spine together may become loose and lax. And when they become loose and lax, you're depending solely on your muscles. Muscles are not very good at working too hard — they let go after some time, and that's when you get the instability.
[34:20] In terms of CCI, there's a spectrum. You can have severe instability where you don't even want the patient to drive home and you call the surgeon right away, all the way to situations where you can do some other things and that'll be fine. I just want to clarify that there's a whole spectrum.
[34:39] So just to help you understand the concept of cranial settling again: the skull weighs about 11 pounds — and for people listening in Europe, that's 6 kilograms.

[34:58] Dr. Linda Bluestein: No, that's great.

[34:58] Jennifer Milner: Helpful. We have people listening all over the world, so that's great.

[35:00] Pradeep Chopra, MD: Right. So it's 11 pounds, or 6 kilograms. When you stand, and because the ligaments are loose and lax, that bowling ball — the head — settles down. It drops down. And as it drops down, it puts the cervical spine out of place. The vertebrae in there go out of place. It's like when you put something heavy on something and it deforms — it sort of deforms the cervical spine. And that is why we need an upright MRI.
[35:44] The neurosurgeons and neuroradiologists draw all kinds of lines with the computer and measure these angles and say, huh, this angle seems to be off — hence there is craniocervical instability. And on some of those pictures you can literally see that the vertebrae have shifted significantly when they're upright. That would not show up if they are lying down. You can actually see the shifting even on an upright X-ray.
[36:13] So I just want to touch a little bit on imaging in EDS. Imaging in EDS is different from imaging in other people — it's a dynamic situation. You need to get dynamic pictures. The analogy is: let's say you have a loose wheel on your car. One of the wheels is loose and it's parked in your driveway. You will never have any idea which wheel is loose. It's only when you drive that you know which wheel is loose. And it's the same thing here — you have to take these pictures in different positions to see how things shift at different angles.
[36:53] So for example, when you do an upright MRI, you look to your left and take a picture, you look to your right and take a picture, you look up and take a picture, you look down and take a picture. And then the neuroradiologists put this on a computer screen, draw lines and angles, and can say whether there's been shifting or not.
[37:10] Besides an MRI or an X-ray, there's something called a DMX — digital motion X-ray. These are far and few between, but they are done here in the US. The people who do them can actually measure these changes in angles. A lot of them are done by chiropractors who understand mobility of the spine. In the United States, at least, it's not mainstream — you won't find it in a hospital. It'll be more in a private practice setting.
[37:43] So that's all about cranial settling. Now I've talked a lot about cranial settling, and I want to segue into why I'm so focused on that: when patients go in for surgery — cervical spine surgery or cervical stabilization surgery — if they are lying on the operating room table and their spine is all crooked, the surgeon may go in and fuse it in that crooked position. And the results are never good.
[38:13] The other way to do it is what is called invasive cervical traction. In this case, they put a pair of tongs on the side of your head, a rope goes over a pulley, and they put some weights and literally pull up the head a little bit. They start at usually 10 pounds and go to 20, then 30 pounds — not more than 40 pounds. And at one of these points, all of a sudden things change. Everything improves significantly: their tolerance to light improves, their tolerance to sound improves, their breathing improves, their brain fog improves, their lightheadedness improves. The surgeon who does this calls it the Jesus moment. I kind of told him that sounds a little religious and somebody might not like that. He said, we've tried changing the name, but everybody has come back to the Jesus moment. And I've seen videos of this — these patients are thrilled by the experience of invasive cervical traction.
[39:28] Now let's say somebody has this so-called Jesus moment. Numerically, they look for an 80% response across a few factors like dizziness, brain fog, breathing, exposure to light, exposure to loud sounds. If there's an 80% improvement, then the patient is a candidate for surgery.
[39:54] Let's say I have EDS and I do the invasive cervical traction and my response is at 25 pounds. That is the traction that will be on my head when I go in for surgery. When you get on the operating room table, they put that same traction of 25 pounds because that's your best position — the position where the spine is the most straightened out. And that's the position they fuse it.

[40:23] Jennifer Milner: That's so interesting. I'm sorry, I'm still amazed that somebody thought of that and that it works. That's so cool. Sorry, go ahead.

[40:32] Pradeep Chopra, MD: I tried to think of an analogy and I could. The analogy would be: if you're trying to repair a little stick — it's a broken stick and you put super glue — you really want that stick to be very straight and aligned before you put the super glue. And this is the situation where you want the neck to be in the most optimum position, with the weights being your guide, before you fuse it.

[40:57] Dr. Linda Bluestein: Yeah, and I think doing that when the patient is awake and can give that feedback — so then they have that additional data — is very, very helpful. I'm wondering, some people listening to this might think they've seen a neurosurgeon who did not propose that technique. I know some neurosurgeons do that and some don't. Do you have any thoughts?

[41:17] Pradeep Chopra, MD: So here comes another hack.

[41:20] Dr. Linda Bluestein: Okay, here comes a hack.

[41:23] Pradeep Chopra, MD: So if you have a surgeon that says, oh no, no, no, I'm just going to fuse you — I can see where the problem is, I'm just going to fuse you — you're essentially telling me that you're going to fuse my neck in the worst position you're seeing on the MRI. If you come across that surgeon, just politely say thank you and run.
[41:42] Now it is kind of sad because we literally have millions of neurosurgeons in the country and they don't follow that. The only neurosurgeon that I know who follows that is Dr. Bolognese in New York. And as a third person living in Rhode Island and seeing these patients, looking at the results of surgeries from different surgeons — I have never seen a complication, or I shouldn't say complication, but I've never seen a patient come back after 5 years of the first surgery saying they now have to extend the fusion.

[43:16] Jennifer Milner: I have not seen that.

[43:17] Dr. Linda Bluestein: That's actually amazing, because that's a very common problem — having to extend a fusion. So if you have not seen that, and I haven't either — I've had a lot of patients who have had surgery with him. I think a lot of people might not realize that's a very important piece of data, because whenever you fuse a part of the spine, that transmits forces to the other parts of the spine. So that series of events is not that rare. Very, very good information.

[43:43] Jennifer Milner: Well, and what a great hack — it's something we talk about, but it's so wonderful to hear a doctor say—

[43:47] Pradeep Chopra, MD: You mean the hack about running away?

[43:49] Jennifer Milner: Yes. It's really empowering to hear another doctor say, hey, if this doesn't seem right, if this is not lining up with what you think should be happening, just leave. It's okay not to do that. Because a lot of times those of us who are not medical professionals feel like, well, they said this would work and I went to them, so I guess I should, even though it doesn't sound like it will, and the data I've read doesn't say — it's empowering to know you don't have to continue with that if it's not right.

[44:15] Pradeep Chopra, MD: You know the term "your neck is on the line"? This is exactly where it came from.

[44:20] Jennifer Milner: This is true.

[44:21] Pradeep Chopra, MD: Your neck is on the line. When you buy a car, you talk to 20 different people: hey, what's a good car? You do research, you do all sorts of things. But when it comes to having surgery on your neck, you go to the first guy and he makes all these bizarre promises: oh, you're going to be in the circus next week, everything's going to be fine and great. But the fact is — I have no motivation here. Dr. Bolognese refuses to pay me. But I just look at this and say, firstly, does the science make sense or not?

[44:58] Dr. Linda Bluestein: Right.

[44:59] Pradeep Chopra, MD: For me, it makes total sense. And you compare the results of other surgeons to this surgeon and say, well, yes, there is a big difference. And anybody can understand that if you fuse something that's already in a very displaced position — if the spine is in a very displaced position and you haven't corrected that, you haven't aligned that little broken stick — then fusing it is always going to leave you with a crooked stick. And once you fuse that neck in a crooked position, in a few years you're going to start having more problems.
[45:37] Now, I do get some pushback from patients who say they hate the idea of having traction put in their head. But number one, this is done under anesthesia. When they put the pins for the traction, they give you a light sedation, MAC anesthesia. You're not aware of it, you don't feel it. And considering that it's a tiny little hole which will eventually close up, it's worth it. I have asked patients: you had a cervical traction — how was it? And they said it was fine. They didn't worry about the pin being put in their head. So it really is important to get the data from this cervical traction. If your response to a cervical traction is only 20%, getting surgery is not going to help because the cervical instability is not severe enough that surgery would benefit.

[46:35] Dr. Linda Bluestein: Yeah, that's a good hack.

[46:37] Pradeep Chopra, MD: Yes — when not to do surgery.

[46:40] Dr. Linda Bluestein: Yeah.

[46:40] Pradeep Chopra, MD: And in my view, it's always been — Dr. Bluestein, you worked in operating rooms, I worked in operating rooms — and sometimes you just look at the surgeon and say, where did he get his training from?

[46:53] Dr. Linda Bluestein: Unfortunately, yes.

[46:53] Pradeep Chopra, MD: Yes. And then you see these brilliant surgeons doing brilliant work.

[46:54] Dr. Linda Bluestein: Yes.

[47:00] Pradeep Chopra, MD: So in my theory, a good surgeon should know when not to do surgery. That's the key thing. And they know when this is not going to help. So if you flunk the invasive cervical traction, then surgery is not going to help.

[47:19] Dr. Linda Bluestein: I'm actually so glad you just brought that up. I've had patients who keep looking for an opinion until they get someone who will operate on them. If you've seen a surgeon and they're saying surgery is not indicated — surgeons like to do surgery, they train to do surgery — so if you've seen one and they say surgery is not indicated, getting a second opinion may be a very good idea. But if you've seen 2 or 3 people saying surgery is not indicated, I would strongly suggest people not keep going until they find someone who will operate, because you will eventually find that person.
[48:07] So patient selection — if you're a surgeon, patient selection is everything. Who they're turning away for surgery is everything. Good surgeons are very careful about that patient selection, and they know who will benefit from surgery and who won't.

[48:19] Pradeep Chopra, MD: And that's what I tell patients. Even my non-EDS patients — I'll send you to a surgeon that I think is good, and if he says surgery is not an option, then stop.

[48:21] Dr. Linda Bluestein: Yep.

[48:33] Pradeep Chopra, MD: Not an option. If he says it is an option, then you can get another opinion from another surgeon as to what kind of surgery needs to be done — that's a different story. But don't go doctor shopping.

[48:48] Dr. Linda Bluestein: Yep. All right. So that's another hack. All right, Dr. Bluestein, here comes Eagle syndrome.
[48:58] This is one of those conditions — after 30 years of seeing EDS patients, you think you know everything, and then all of a sudden another condition starts showing up and I'm like, where was this? I remember mast cell activation syndrome. Do you know how old mast cell activation syndrome is? About 12 years. 12 years ago was when the first paper was published, and I read it and said, yeah, I'm not going to see one of these patients. And now it's like, you have mast cell everywhere — not only in EDS patients, but now in long COVID patients. So bizarre.
[49:41] Eagle syndrome is one of those. For the longest time, this is more of a facial pain. The pain is in the face — and I guess the face is part of the head. These patients will present with sharp shooting pain in the jaw on one side, or it could be both. They also have it in the back of their throat, and it feels like there's something stuck back there. They have pain in the base of their tongue, in the ears, into the neck and going into the face. They have difficulty swallowing, and it feels like there's a foreign object stuck in the throat. The pain gets worse with chewing and swallowing, and also gets really worse with turning their neck. It also gets worse with touching the back of the throat, which I do — and this is a doctor hack. Never stick a finger into a patient's mouth with your dominant hand. Don't put your right hand in their mouth.

[50:40] Dr. Linda Bluestein: Or into an elevator door.

[50:40] Pradeep Chopra, MD: Or into an elevator door. Always use your left hand. So you put your finger into the back of their throat, press to the side, and they're going to swear at you — which is good, because that confirms your diagnosis. So what is Eagle syndrome? It's got nothing to do with the bird. This is Dr. Eagle who discovered it.
[51:06] There's a little bone in the corner of the jaw. It has a purpose — some ligaments and muscles attach to it — but for some reason it starts to elongate. And when it elongates, it presses on a nerve called the glossopharyngeal nerve. Glosso means tongue, pharyngeal means throat. So the pain is in the tongue and throat.
[51:30] Very briefly: when someone comes in with pain on one or both sides of the face that goes into the throat and something feels like it's stuck in the throat, think of Eagle syndrome. The way it's diagnosed is just what I told you — the doctor puts a finger into the throat, presses on the styloid process, and the patient can immediately feel the pain. The scan you need is a 3D CT scan — they take a CT scan and reformat it into a 3-dimensional view. That's the gold standard. And the good news is surgery has a cure rate of 80%.
[52:07] I've struggled with this — why would people with EDS have Eagle syndrome? I have no solution to that. I don't know why. I've not seen it in my non-EDS patients. It is often misdiagnosed as trigeminal neuralgia: oh, you have pain in the face, it must be trigeminal neuralgia. So if it relates to the throat, you should start thinking of Eagle syndrome right away. Facial pain with throat problems is Eagle syndrome. The cure rate with surgery is fantastic — they shorten the bone that sticks out, called the styloid process.

[52:46] Dr. Linda Bluestein: You said you've never seen it in a non-EDS patient. How often have you seen it in EDS patients?

[53:00] Pradeep Chopra, MD: Obviously I don't have the figures, but I would say 1 in 10 EDS patients have it. It's fairly common.

[53:05] Dr. Linda Bluestein: Really?

[53:06] Pradeep Chopra, MD: That's the puzzling part.

[53:08] Dr. Linda Bluestein: Wow, that's a lot.

[53:08] Pradeep Chopra, MD: That's a lot. And that's the puzzling part — up until a few years ago, I didn't even know Eagle syndrome existed. I was reading about facial pains and trying to figure them out, and then I came across Eagle syndrome. I said, oh, so easy — you stick a finger in somebody's throat and if it hurts and you still have a finger, they haven't bitten it off, then you're good. So with your left hand — the pinky finger, because that's expendable. You don't need your index finger, right?

[53:09] Dr. Linda Bluestein: Right.

[53:42] Pradeep Chopra, MD: The most useful fingers are not the pinky. Stick that in there.

[53:44] Jennifer Milner: Is there a physical — like, do you see a shifting of the jaw or sort of a misalignment that goes with it, or is it purely just the lengthening of that bone in the back? Like, is it just visual assessment?

[54:00] Pradeep Chopra, MD: You don't see it. There's nothing — you cannot see it from the outside. There's no change in the jaw. It's a really tiny bone, barely a few centimeters long, maybe about half an inch. And it's behind the jawbone. Sometimes I'll try and push from the outside and they'll feel the pain. But the best test is to push it from inside.
[54:24] So the point I'm trying to make is: if you have facial pain that you may have been told is trigeminal neuralgia, but it has throat symptoms — something stuck in your throat, or your tongue hurts, the base of the tongue hurts — think of Eagle syndrome.
[54:41] With this, we can move on to the thoracic spine. Moving down — we've talked a lot about the neck, now moving down to the thorax. The thoracic portion of the spine is relatively a stable spine because it's part of the rib cage, so it doesn't really show up as a problem in the non-EDS population. But of course, EDS patients don't make life easy for us — they have to have something you've never even paid attention to. But jokes apart, they do have thoracic pain. They don't often complain about it. But if you push on the thoracic spine, they'll say, oh yeah, that really hurts. They may complain of pain in the upper back in what is called a coat hanger distribution, and they might also complain of pain between the shoulder blades.
[55:39] And again, the spine is made up of little bones like a 3-dimensional jigsaw puzzle, stacked together and connecting — each one connects to the one above and the one below with ligaments. The problem is that the ligaments are elastic, so it shifts. And when it shifts, it hurts.
[55:57] For a physician, the way to diagnose that is to go to the thoracic spine, go to the midline, then go a little to the left or right and push with your thumb — it will be tender. Now the issue here is rib subluxations. Each rib connects to the thoracic spine with 3 teeny tiny little joints. The reason there are joints there is because each rib has to constantly move up and down — as you breathe in, it moves up, and as you breathe out, it moves down. So your ribs are constantly moving and they are tethered down to the thoracic spine with these 3 little joints.
Two things happen here. First, people with EDS tend to slouch — and I'll get into that a little later, but it's not a habit thing. It's a condition. And when they slouch, the rib pops out of its joint at the junction between the rib and the spine. And that's called a rib subluxation. It is an excruciatingly painful condition. It feels like you're having a heart attack.
[57:17] So the rib subluxes out — how do you treat that? I'm going to talk about how to treat it when it subluxes, and then how to prevent it from subluxing. When it subluxes out, you can lie on a roller — one of those exercise rollers.

[57:36] Jennifer Milner: Yeah, like the foam rollers.

[57:37] Pradeep Chopra, MD: Not the foam ones, but the harder rubber ones — they're rubberized but they're hard. And you lie on it and roll back and forth, and that pops the rib back in place.

[57:49] Dr. Linda Bluestein: Rolling your thoracic spine back and forth on it?

[57:51] Pradeep Chopra, MD: Yeah. So you put the roller on the floor, you lie facing the ceiling, and then you move back and forth. And when it moves over that subluxed rib, it pops the rib back in place.

[58:04] Dr. Linda Bluestein: That's a double hack.

[58:04] Pradeep Chopra, MD: That's the — oh wow. Look at that. We're on a roll with our hacks. Literally on a roll. Roll to put the rib in.
[58:13] All right, so we're on the floor rolling back and forth and pushing this rib back in place. You've got that done. But the thing is, you can't be walking around — if you're in the mall and suddenly your rib pops, you don't want to pull out the roller in the middle of the mall and start pushing it back. So to prevent it, there are a few things. One is that you want to stabilize the thoracic spine. You don't want to slouch. And like I told you, it's not a habit. The thoracic portion, the chest portion of the spine, is held together by ligaments, and these ligaments are lax. Because most of the stuff like the lungs, the heart, and in women the breasts are in front, you tend to lean forward. And then you just automatically start to slouch forward. To sit back straight up is an effort. When it's an effort, you get tired eventually and just let go.
[59:12] So some of the techniques for keeping it stabilized: one, you can try a compression garment. There's a company called DM Orthotics — it's a UK-based company, but they do sell in the United States — and they make these pretty cool shirt-like garments that can help keep your posture correct. There's another company called AlignMed — A-L-I-G-N-M-E-D — and they produce something more like a vest called the Spinal Q. But these are big and bulky, and you are never going to convince an 18-year-old to wear that. That's been my thing.
[1:00:06] But there's another one I wanted to talk about: the BodyBraid. The BodyBraid looks like a series of tapes connected together — not sticky tapes, but tapes designed to cover the joints, mostly the joints and the spine. What it does is keep your spine in alignment — actually keeping everything in alignment, starting from the shoulders, the spine, the pelvis, the knees. It's a phenomenal piece of wear.
[1:00:41] Just to give you a little background, it was invented by a physician in Toronto, Canada. I think he spent many, many years researching it and went through many different designs before finally coming up with this one. On a personal basis, I tried it — I don't have EDS — and I loved it. I felt so much better. My posture was better. Patients love it. If you go on their website, you can see it — it's not even that expensive. There are videos on how to use it, and you can also set up an appointment with the physician and he can demonstrate it to you.
[1:01:24] There's a tiny learning curve because there are so many tapes. For me, the learning curve was 3 wears, 3 times. And EDSers — they're extremely smart, it's one of the strangest things I've seen. They're extremely smart with all their fatigue and brain fog. They ace their exams. So even though it looks like a lot of tapes around, it's really just a learning curve. Wearing it once or twice and you know the trick. It's discreet — you can wear it under your clothes. And it's very effective.

[1:02:10] Dr. Linda Bluestein: It seems like another advantage of the BodyBraid — we're recording this during the summer, and people will listen at all different times of the year — but another advantage is that it's a lot cooler than some of these other things that cover a lot more of your skin. We know people with EDS are often temperature intolerant, and the heat can increase their mast cell activation. Do you find that it's more comfortable for people because they're not adding another layer?

[1:02:31] Pradeep Chopra, MD: Absolutely. That's been my problem before I came across BodyBraid — recommending patients, especially young patients, to wear a compressive garment on their torso. Frankly, even for me it's uncomfortable to wear. No one likes to have a tight shirt unless you have a six-pack.

[1:02:52] Jennifer Milner: But the benefits of it — you realize you feel better when you wear it.

[1:02:56] Pradeep Chopra, MD: Right. That's the thing. You feel great when you wear one of these compressive garments. And it depends a lot on the patient's wishes. That's why I mentioned all of them, because they all work. And of all these, the lightest one is the BodyBraid. It is also very discreet — you put it under your shirt, it takes 2 seconds to wear, and it's not choking your lungs. So this is the thoracic part of the spine with the rib subluxations.

[1:03:28] Dr. Linda Bluestein: So I was really curious to ask you, Dr. Chopra, if you think that some of these differences we see in people with EDS versus people who don't have EDS could be because we have greater levels of interoception at the same time that we have less good proprioception — which is an interesting phenomenon in and of itself. People refer to us as like canaries in the coal mine. So do you think some of these phenomena that are harder to explain on the basis of differences in connective tissue could be on the basis of differences in sensation and that kind of thing?

[1:04:02] Pradeep Chopra, MD: So I know you had previously referred to this in terms of Eagle syndrome being more common in EDS, and we were puzzled about why. In that context, I don't think it is, because in Eagle syndrome there is a definitive increase in the length of this bone. There's no gray area — you can literally see this little bone. It looks like a dagger and it's protruding out through the jaw.
[1:04:31] So the question you were really asking was: are people with EDS more aware of their body and sensations? I would think it's actually the opposite. One of the toughest parts of seeing an EDS patient is getting them to talk. I'll ask a teenage girl, does your right wrist hurt? And she'll say no. But then when you start diving a little deeper, she'll say, oh yeah, it hurts when I write, and I can't write for more than 2 minutes. They don't really — they've grown up accepting that pain is something every human being feels. Lightheadedness, it's something every human being feels. So in some parts, yes, they do have more awareness of their issues because those issues are affecting their functioning. But in some parts, they grow up thinking, okay, everybody gets lightheaded when they stand. So when they get lightheaded, they don't care.

[1:05:24] Jennifer Milner: I think that's an interesting point that we have seen played out through so many people we are in contact with who have EDS. Everything hurts, or they're so aware of everything, that it's hard for them to tell what is quote-unquote normal and what is something they should bring up. Should I tell them my wrist clicks? Oh, everybody's wrist probably clicks. Should I tell them my fingers hurt when I pull the comforter up because it subluxates? Probably happens to everybody.
[1:05:47] So I think what you're saying, Dr. Bluestein, is perhaps something like Eagle syndrome — not necessarily Eagle syndrome specifically, but perhaps something like that — is not necessarily more common in people with EDS, but people with EDS are more aware of such pain and issues, and so they point it out more. And I think it's that fine line: my experience is that people with EDS do have much more awareness in their body, and at the same time there's so much everyday low-level pain. It's hard for them to turn down the static and figure out which ones they're supposed to be paying attention to. Does that make sense?

[1:06:23] Pradeep Chopra, MD: Yes. I like the word static. It is a lot of static. There's a lot of noise that they live with. And it's only when it starts to get worse — and we'll talk about this when we talk about mast cell activation syndrome — how this noise increases to a point where it becomes unbearable. I think all EDS patients have pain, but at times this pain will go up to a point where they become nonfunctional, and that's when they start seeking treatments. But they have pain all the time. It's just that they've grown to accepting it as, hey, this is normal.

[1:07:01] Dr. Linda Bluestein: Yeah, no, I think that's true. And your point about sometimes having to extract the information — I feel that way too sometimes. I think what happens is, if we report our symptoms to a number of people and they're not seemingly interested, then we stop reporting them. Or let's say we do report them, they do a couple of tests, and everything comes back unremarkable. We don't want to be in that position of being told, oh, you're fine, you're fine, just kind of go away. So I think that's a good point you raised.

[1:07:29] Jennifer Milner: Hey, can we make sure that we cover the lower back as we're looking at the spine from head all the way to tail? What have you got for us in the lower back?

[1:07:37] Pradeep Chopra, MD: Yes, ma'am. So the lower back, like the rest of the spine, does have its issues with instability. But oddly enough, I don't see a lot of damaging instability in the lumbar spine. I haven't had patients where I said, okay, your lumbar spine is so unstable that you need surgery. That's a little surprising. But the relevance of lower back pain here is in terms of tethered cord syndrome.
[1:08:11] Tethered cord means your spinal cord is tethered down. The normal spinal cord ends at the L1 level, which in a woman would be at about the bra strap level. So it's pretty high up, not further down. It ends in a conical form, called a conus. And then there's a little string that hangs from it which has no purpose in life — that string is called the filum. It has no nerves in it, nothing in it, it just hangs there. It's a remnant from embryology. And it hangs loose. The reason it hangs loose is because the spinal cord and the brain shift as we move around — as children grow taller, the bony part grows faster than the spinal cord itself. So the spinal cord and brain are under no tension, and that's why the filum hangs loose.
[1:09:22] In tethered cord, what happens is the filum — that little useless piece of thread hanging at the end of the spinal cord — is tethered there. It's stuck. And when it gets stuck, it puts a tension on the spinal cord. Remember I told you the conus, the conical end of the spinal cord, ends at L1? It can get pulled down to L2 or even L3. So essentially there's tension on the spinal cord from tethered cord syndrome.
It presents differently at different ages. I'm not going to go into babies because that diagnosis is pretty obvious, but in the teenage to adult group, how they present: one of the things they present with is lower back pain. Now, I don't want you to think that if you have lower back pain you have tethered cord — you can have lower back pain for 16 million other reasons.

[1:10:22] Jennifer Milner: Absolutely.

[1:10:25] Pradeep Chopra, MD: They also have what is called a neurogenic bladder — essentially where the bladder does not talk to the brain. I'll explain that in more detail. The key thing I look for is that their legs get heavier and weaker as they walk. That is a key point for me. They'll always tell you that when they walk a short distance, it feels like their leg is getting weaker — and painful. They have diffuse pain in their legs, and the reason I say diffuse is because it's not along a specific nerve distribution. The whole leg may hurt. And it may be either the left leg or the right leg — today the right leg, tomorrow the left leg. It's not in a very specific area of the leg. It's unrelated to the joints. The legs get heavier as they walk. They have bladder issues known as neurogenic bladder. And they also have this back pain that cannot be explained by anything else.
[1:11:20] So what is a neurogenic bladder? They go often to the bathroom. And that's a difficult question for a lot of people to answer, because EDSers don't know what is normal — they think going to the bathroom 20 times a day is normal. There's no set number, but going a lot is considered increased frequency. Sometimes they can have urinary hesitancy. And sometimes it's urgency — okay, I can't wait, I need to go right now, we can't wait for the next McDonald's. Then they have a sense of incomplete emptying of the bladder — they just peed but it feels like there's some left, not satisfied, and they may go back again. In some cases they can have incontinence. To clarify: incontinence in women can come with stress — like when you cough, sneeze, or laugh, a little bit of urine comes out. That's not what I'm talking about. I'm talking about a lot of urine coming out with no control.
[1:12:35] The incontinence is not as common here. These are the symptoms of tethered cord.
[1:12:39] The one point I wanted to make was about the incidence: 66% — roughly 70% — of patients who have Chiari malformation will also have tethered cord syndrome. The significance of that is that when you get diagnosed with Chiari malformation, start looking for tethered cord symptoms. There's a high incidence of tethered cord in patients with Chiari malformation.
[1:13:14] The problem with tethered cord syndrome is that MRI is not a very useful tool. Hence the term occult tethered cord. I think all tethered cords are occult, because it's not often that you see the filum or the tethering, or that you see the conus has come down a little bit. You cannot depend on an MRI.
[1:13:38] And this is a big deal, because the world of neurosurgeons is very strongly divided on this. There is a major group that has said: if it's not on MRI, it doesn't exist. And then there's this smaller group that understands the situation and says you really don't have to have it on MRI — you can base it on clinical diagnosis. These surgeons will look at the clinical history, do an exam, and that's what they base it on. They will do what is called a urodynamics study — a UDS. The urologist does it. They look at how much urine you retain after you've finished, how strong the sphincter is, all of those studies. And that gives them another piece of information.
[1:14:22] So before a surgeon goes into the operating room for tethered cord, they have all this data, and they don't solely depend on an MRI. The problem I run into is that some centers will absolutely not accept that. In Boston, for example, they will not — they do not believe you can have tethered cord without it showing on MRI. That's the problem.
[1:14:53] So just to recap on tethered cord syndrome: the filum is kind of stuck down at the bottom. When I see it in teenagers and go back and talk to the mom, there will often have been a growth spurt about 6 months before — and now they have all this pain in their leg, often attributed to growing pains, but it's not. Growing pain is pain below the knee, in the front, at night. It's not diffuse pain in the leg. It's not weakness in the leg.
[1:15:32] Patients with tethered cord present with back pain, bladder symptoms, legs getting weaker as they walk, and diffuse pain in the legs that doesn't match up with any nerve distribution. Bladder issues include increased frequency and a sense of incomplete emptying. These are the common symptoms, often associated with Chiari malformation. MRI is not a dependable tool — the diagnosis mostly depends on clinical history and examination.
[1:16:02] I have to say, Dr. Klinge, who does a lot of these surgeries, is about a mile from my office. I've often sent patients to her and always asked for feedback: hey, I diagnosed this patient with tethered cord, what did you find? I don't think I've been wrong. But that doesn't mean I haven't missed somebody.

[1:16:28] Dr. Linda Bluestein: Right.

[1:16:29] Pradeep Chopra, MD: So when it comes to diagnosing tethered cord, I really grill the patient. Are you sure your legs feel heavier? Are you sure you have pain here? Are you sure about your bladder symptoms? Although Dr. Klinge has told me that the bladder symptoms are not that crucial nowadays — she hasn't found them to be as important. So when you do the surgery — the surgery is actually not a scary surgery. They don't remove any bone. They go in there, find the filum, and then snip it and remove a section of it.
[1:17:05] The question is: what benefit will you see? The first benefit these patients see is that their bladder symptoms improve. And this literally happens as soon as they wake up from surgery — that fast. Their bladder symptoms improve within the first 24 hours.

Jennifer Milner: Wow.

[1:17:20] Pradeep Chopra, MD: The leg symptoms take a little longer. I've found it takes about a year to reach a point where everything has stabilized. So those are the expectations.
[1:17:36] We've had some good surprises also. In some cases we've seen GI symptoms improve. In some cases we've seen neck pain improve. And I truly don't have a great explanation for that. The nerves to the GI system don't even go through the spinal cord — they have their own pathway, the sympathetic and parasympathetic nerves. So we've seen some surprisingly good results, but the usual expectation should be improvement in bladder symptoms and leg symptoms. And that I think is our lower back.

[1:18:19] Dr. Linda Bluestein: Would you—

[1:18:22] Pradeep Chopra, MD: Just wanted to touch on SI joint pain.

[1:18:24] Dr. Linda Bluestein: That was on my list. Yep.

[1:18:26] Pradeep Chopra, MD: We'll talk about sacroiliac joint pain when we talk about leg issues.

[1:18:29] Dr. Linda Bluestein: Okay.

[1:18:30] Pradeep Chopra, MD: Because it's closely related to that.

[1:18:32] Jennifer Milner: Okay.

[1:18:32] Dr. Linda Bluestein: That's fine. What about Tarlov cysts?

[1:18:34] Pradeep Chopra, MD: Tarlov cysts are tiny cysts — they can be tiny or big — but let's go to the anatomy quickly. We have the bony spine, and it has holes everywhere through which all kinds of nerves come out. Sometimes a little cyst develops at the junction where the bone meets the hole through which a nerve is coming out. A cyst is basically a teeny tiny balloon. Some of them are filled with water — squishy balloons. And sometimes they're squishy enough that they don't cause any symptoms. You do an MRI for some other reason and you'll see a Tarlov cyst. You talk to the patient: hey, do you have pain in this area? They say no. You leave it alone.
[1:19:34] Sometimes they become bigger, or tighter, or they're at a really bad place and are actually compressing a nerve. That Tarlov cyst needs to be treated. There are two ways. Some physicians will go in and stick a needle into the Tarlov cyst and burst it open. In some cases, surgeons will go in and actually remove the cyst.
[1:19:55] But the point is: if you get an MRI report that says you've got a Tarlov cyst, or you've got 3 of them, don't panic. It may be completely benign and have nothing to do with your pain. Let the surgeon decide whether this is significant enough to be the reason for your pain. Tarlov cysts are common in EDS — it probably happens because of loose connective tissue. You do see them in non-EDS patients, but not as often.

[1:20:27] Dr. Linda Bluestein: Yeah. That's actually what led to my EDS diagnosis. I was having severe sciatica and had multiple MRIs, and they kept saying, no, that's not the problem. And finally somebody said, maybe it is the Tarlov cyst. So I ended up getting other opinions, and was reading, and realized — oh, they're saying this is more common in connective tissue disorders. So then I started reading more about connective tissue disorders, and that's when the light bulb started to go off.

[1:20:49] Pradeep Chopra, MD: That's the problem, Dr. Bluestein. You should not have read that book. You would not have had EDS. So this is another hack: don't read books.

[1:21:06] Jennifer Milner: If you don't know it, then you can't have it, right?

[1:21:08] Pradeep Chopra, MD: Exactly. You could be living your life happily — oh yeah, my knee gives out, no problem.

[1:21:12] Jennifer Milner: No big deal.

[1:21:13] Pradeep Chopra, MD: But obviously we're kidding. It is crucial — very, very crucial — that if you get a diagnosis of EDS, you educate yourself. And please don't think the worst of it. If you're diagnosed with hypermobile EDS, stick to hypermobile EDS. Just because you have some scoliosis, do not walk into the kyphoscoliotic EDS section. There are only 11 known cases of kyphoscoliotic EDS. One of them is my patient. I asked her what her number was and she said, my number is 9. But this is what I'm trying to say: all EDS patients have scoliosis — it's a dynamic scoliosis. Your ligaments are loose and your spine is going to be shifting around all the time. Nothing to worry about. But if you're diagnosed with hypermobile EDS, just read about hypermobile EDS.

[1:22:04] Jennifer Milner: And there again, it's that fine line of wanting enough information to be armed and knowledgeable without getting so much information that it becomes overwhelming or stressful or sends you down rabbit holes you don't really need to go down. And I think part of the time that's why people are afraid to go to a doctor or to look things up — they don't want to know. We joke about it: if Dr. Bluestein just hadn't looked it up, she'd still be happily working in the OR as an anesthesiologist — which is not true, right? Health issues sent her in a different direction, and having that knowledge helped her make wise decisions.

[1:22:34] Dr. Linda Bluestein: And yes, I had tons of issues before that.

[1:22:37] Jennifer Milner: Right, right. So something I tell my dancers — my dancers are notorious for not wanting to go to the doctor, they don't want to know — and I say, going to the doctor isn't going to change what already exists in your body. It's just going to give you more information on what to do with it. You can always choose to ignore the doctor if he says, oh, if you dance you might tear a ligament. You can say, okay, but at least then you know and you know what the odds are. So don't be afraid of getting quality information you can trust. It's going to help you even if it sounds like, oh no, now I know I have this. Well, you always had this, but now you know about it. Maybe there are things you can do about it.

[1:23:07] Pradeep Chopra, MD: So Jennifer, that's an extremely important point you brought up, and we will discuss it more in our pediatric EDS episode. One of the key things when I see pediatric patients: I tell their parents to absolutely make sure they're not hyperextending their joints. They should not be in any activity or sport that's going to get them into hyperextending their joints. I have had national-level athletes — ice skaters, rollerbladers, and all sorts — go from competing at a national level down to being in a wheelchair. Prevention is the key in EDS.

[1:23:56] Jennifer Milner: Yeah, absolutely. Prevention.

[1:23:58] Pradeep Chopra, MD: That's the hack. That's another hack: stop doing foolish things.

[1:24:01] Dr. Linda Bluestein: Yeah, and that is one of the absolute reasons why we do this — to educate that population. That's why I go into dance studios and talk about: don't do party tricks, don't do things that may make your joints more unstable and cause you more pain down the road. And it's great — we really appreciate you coming back to chat again. Obviously we're going to be having more conversations because this is a great way for people to learn. One of the challenges as there has been more awareness about EDS is there are all these charlatans, and clinics that may not have the best intentions. They know the trigger words to put on their website. So it can be really hard for people to sort out where to get information from, where to go for treatment, and what treatments are a good choice versus might not be helpful. It can really be the Wild West, especially for this population of people.

[1:25:00] Pradeep Chopra, MD: And that's why talking to peers is important — because someone may have already gone through, let's say, invasive cervical traction. There's a lot of fear in getting that done. Talk to someone who's already had it done. There's nothing like getting information from a person who has walked through that journey.

[1:25:30] Dr. Linda Bluestein: I think that wording was key — asking them what their experience was. Because what I see sometimes in support groups — and I don't go into them anymore, but for a while I did, especially when I was early on in my own journey while I was still working in the operating room and transitioning out of that — sometimes I would see people say, well, you should— and you have to keep in mind, in the support groups they don't know your circumstances. So ask what their experience was, and then you can use that information. Just be careful about that person giving advice, right? This podcast is information, not specific advice, because there are a lot of people listening and they're all dealing with different symptoms and different situations. We're giving information to help them make more informed choices, but we can't give any one person advice because we don't know what their circumstances are.

[1:26:15] Pradeep Chopra, MD: Right. And don't forget how important the hacks are. Our hack section is by far the best.

[1:26:23] Dr. Linda Bluestein: Yes.

[1:26:24] Jennifer Milner: Right. And because they're low intervention, they're low risk. We're certainly not advocating that anyone cut and paste someone else's experience into their own medical charts — it's not a cut and paste, it's a read and learn type of thing. Find their own experiences.
[1:26:39] Well, this is a lot to absorb, and we have literally gone from head to tail, which I so appreciate. I love the way we're looking at it — not from one specific system in the body, but from a grouping of things. What's the best place for people to find you? I know people have already found you, but for those listening for the first time, what's the best place?

[1:27:02] Pradeep Chopra, MD: My website, which is in pretty bad shape, painri.com — pain as in pain, and RI for Rhode Island. Or my office email address is [email protected].

[1:27:19] Jennifer Milner: Did you say SNAPPA? S-N-A-P-P-A?

[1:27:22] Pradeep Chopra, MD: Yeah, SNAPPA. Don't ask. It's an acronym.

[1:27:24] Jennifer Milner: SNAPPA?

[1:27:25] Pradeep Chopra, MD: Yeah.

[1:27:29] Jennifer Milner: [email protected].

[1:27:30] Jennifer Milner: Okay, you're gonna be inundated, I'm sure. Now, are you on Twitter?

[1:27:33] Pradeep Chopra, MD: Actually, I got an email from a lady in Peru who had heard your podcast.

[1:27:40] Jennifer Milner: See? So you're gonna get all the love now.
[1:27:46] Well, as always, it is so great to have you on the podcast and to have you contribute your wisdom to everything we are trying to do — continuing the conversation around EDS, getting accurate information out there for people to feel like they can learn from trusted and reputable sources and get information that may actually be helpful on their own health journey and road to discovery. So, thank you so much once again for being here and putting up with all of our questions and synthesizing everything so wonderfully.

[1:28:17] Pradeep Chopra, MD: Thank you, Dr. Bluestein. Thank you, Jennifer. It's a pleasure. I love this podcast — we keep it solid, but we do add some humor to it. I love my hack section.

[1:28:28] Jennifer Milner: I love your hacks. We love your hacks.

[1:28:32] Jennifer Milner: And we're going to have to write up a whole sheet of them.

[1:28:34] Dr. Linda Bluestein: Yes, we will. And I want to add one other thing. When I was in my residency at the Mayo Clinic, one of the things I really liked was that it wasn't the senior faculty teaching the junior faculty teaching the senior resident teaching the junior resident teaching the medical student — we learned directly from the faculty. By bringing someone like Dr. Chopra on, we want to bring experts who can share information directly with our audience so it's not filtered through all these different layers where it gets contorted. It's like the telephone game, right?
[1:29:06] So we really appreciate you, Dr. Chopra, taking that time, because we know you're extremely busy. People being able to hear directly from you is so incredibly beneficial. It's such a great way for people to learn and get much more accurate information than if they get it from a whole bunch of other sources. We bring in experts from all different areas to get the best quality information we possibly can for our listeners.

[1:29:34] Pradeep Chopra, MD: Absolutely.

[1:29:35] Jennifer Milner: Thank you very much, Jennifer. Thank you, Dr. Linda Bluestein. It's such an honor, and thank you for inviting me. The more information we share, the more we try to enlighten our patients, it does help. And we'll work on the #HysterectomyMovement.

[1:29:57] Jennifer Milner: Everybody heard it here first. We are starting that.

[1:29:59] Pradeep Chopra, MD: We're starting that. You heard it here first, right?

[1:30:02] Jennifer Milner: Well, you have been listening to the Bendy Bodies Podcast with the Hypermobility MD. Our guest today has been Dr. Pradeep Chopra, a specialist in chronic complex pain conditions and their associated coexisting conditions. Dr. Chopra, thank you so much for once again coming back and being our guest.

[1:30:13] Pradeep Chopra, MD: Thank you so much.

[1:30:15] Jennifer Milner: And we will see everybody another time.

[1:30:16] Dr. Linda Bluestein: Thank you for listening to this week's episode of Bendy Bodies with the Hypermobility MD podcast. Help us spread the word about joint hypermobility and related disorders by leaving a review and sharing the podcast — this helps raise awareness about these complex conditions. Visit bendybodiespodcast.com and follow us on Instagram at bendy_bodies. We love seeing your posts and stories, so please tag us using #bendybody. You can also find me, Dr. Linda Bluestein, on Instagram, Facebook, Twitter, and LinkedIn, all with the handle HypermobilityMD.
[1:30:58] 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. This is not intended to be a substitute for medical diagnosis or advice. Do not disregard or delay obtaining medical advice for any medical condition you have. The opinions shared are that of the guest and do not necessarily represent the views of the hosts or any particular organization. Thank you for being a part of our community, and we will catch you next time on the Bendy Bodies Podcast.