Bobby Jones CSF: Ask the Expert with Dr. Fraser C. Henderson, Sr.

VIRTUAL MEETING - June 4, 2020

In our second virtual Ask the Expert, Dr. Fraser Henderson Sr. takes real questions from patients and caregivers affected by Chiari, syringomyelia and related disorders. Some of the topics discussed include craniocervical instability, atlantoaxial instability, Ehlers-Danlos syndrome, Chiari malformation, dysautonomia and tethered cord syndrome. Dr. Henderson, a well-known expert in craniocervical instability, emphasizes the importance of understanding the overlap between symptoms of instability, Chiari, EDS, and even mast cell activation syndrome. This presentation was given on Thursday, June 4, 2020 and was presented entirely online. (2020) A note from Bobby Jones CSF: We want to take a moment to sincerely thank all those who donated in support of this virtual presentation. No one at the organization was expecting that much generosity during such a difficult time for us all. From the bottom of the hearts of our boards, staff and volunteers, we want to thank you for your donations. Your kindness continues to motivate us and makes us all the more certain that this work is valuable and helping the people who need it. Thank you. Full transcript: [Kaitlyn] So, with us today we have Dr. Fraser Henderson. He is a neurosurgeon out at-- in Maryland. And he specializes in spine, and he's done a lot of work in cancer, CyberKnife radiosurgery. He's published over 70 peer-reviewed articles and book chapters-- [Dr. Henderson] Well-- 90-- [Kaitlyn] Ninety? Wow! Okay [laughs] And given how many invited lectures? [Dr. Henderson] About 190. [Kaitlyn] Wow! Okay. Um, he's listed among the Top Surgeons by Consumer's Research Council of America one of the Best Doctors in America, Top Doctors according to the Nichols Institute. And he's been a Top Doctor of Northern Virginia, as well. He was also awarded the Mayfield Neurosurgical Research Award in 2008. So, thank you, Dr. Henderson, for being with us today [Dr. Henderson] Yes [Kaitlyn] I really appreciate it. Um, so we're gonna get started. So, I wanted to start with questions about diagnosis [Dr. Henderson] Yes. [Kaitlyn] There was a question that came up— and we're gonna just jump right in, I guess. A patient complaining of stiff neck, eye pain, blurry vision, and edema in the eye. So, the question really was: is this something that a Chiari or a tight cervical medullary junction could cause? And if not, what is this most likely-- condition to be? [Dr. Henderson] Okay-- you said: stiff neck, blurry vision-- [Kaitlyn] Mmhm. Eye pain. [Dr. Henderson] Eye pain. [Kaitlyn] And edema. [Dr. Henderson] Papilledema? [Kaitlyn] Well, edema-- but I'm sure [Dr. Henderson] Edema on fundoscopy? [Kaitlyn] Uh... I have to assume of the eye, so papilledema. But, I'm not sure. [Dr. Henderson] Um.. and this is a question from whom? From what kind of doctor? Or a therapist? [Kaitlyn] Well, this is a question from a patient. And, I think, an adult. [Dr. Henderson] Okay. When you say a stiff neck, you mean a painful neck, or? [Kaitlyn] Yes. I believe so. [Dr. Henderson] Alright well, first... There's so many things that could be a stiff neck, but the papilledema suggests--- oh, is it one-sided? Or bilateral papilledema? [Kaitlyn] It was in both eyes. [Dr. Henderson] Or you don't really know? [Kaitlyn] Both eyes. [Dr. Henderson] Okay, good. So, that would suggest increased pressure, increased cerebrospinal fluid pressure. That could be from intracranial hypertension, meaning, you know, just an increased pressure in the cranium. And that can be due to a um-- one of the large veins that drain the brain could have a blood clot. Or there could be a compression of that vein that was preventing blood from leaving the brain. The stiff neck, you know, suggests some kind of spinal injury. So, we'll assume there's a problem at the craniocervical junction. A stiff neck could be a tumor. And the-- uh—um.. Assuming it's not a tumor, patients with connective tissue disorders often have instability between the cranium and C1, or C1 and C2. That can lead to spinal fluid flow obstruction. It can also result in abnormal venous flow leaving the brain. [cough] [Dr. Henderson] That can cause eye pain, and papilledema, and of course, the neck—neck pain. The appropriate treatment would probably begin with the MRI of the brain. And then, ideally, if that was normal-- that's probably going to be normal-- but it's important to rule out a brain tumor. Or a tumor of the skull base. If that's normal, you would look for a Chiari malformation, you'd look for craniocervical or atlantoaxial instability. Or, a high cervical level-- essentially C3, C4. The only other thing is-- with eye pain, you might also need to rule out glaucoma. Finally, severe temporomandibular joint spasm—spasm of the TMJ typically refers pain to the back of the head, which can be construed as the upper neck. And also, above the eyes. So, it could cause eye pain. So, someone with a severe TMJ spasm could have those-- though, it would not cause the papilledema. So, I suppose, if there's true papilledema, the first thing is to rule out-- find out what's causing the intracranial hypertension. And if the MRI is normal, and the cervical spine is normal, they should have a intracranial pressure monitor, a "bolt", placed in the top of the skull to measure the pressure in the cranium. And if that's elevated, they might need a shunt, or something. [Kaitlyn] Great. That's great. Actually, this flows into the next question-- you'd mentioned about injury... there was a question that in a patient with EDS, who might've sustained a traumatic injury, like whiplash or TBI— is it common for those symptoms to worsen, or for new symptoms to appear? What steps do you recommend patients take to get the right diagnosis? And to-- treat their issues? Particularly pain? [Dr. Henderson] You mean, if they have a diagnosis of Ehlers-Danlos syndrome? [Kaitlyn] Yes. [Dr. Henderson] And if they're in some kind of an accident, and now they're having progressive symptoms? [Kaitlyn] Correct. [Dr. Henderson] Okay. Well, if they have access to a doctor, osteopaths tend to be well-versed, some chiropractors, some doctors... You know, for the kinds of problems I see, I like a flexion/extension MRI to see if there's any instability between the skull and the spine. And that will also show if there's instability in the cervical spine. And I also like a CT scan, with rotational views looking 90 degrees to the left, and the scan from the skull base down to C3, and then the patient turns their head 90 degrees to the right. And that will show instability between C1 and C2. So, they're my standard things, but that's for a fairly severe case. If they don't have access to those things, then simply wearing a good, hard neck brace for 3 or 4 weeks, night and day, day and night and doing isometric exercises of the cervical spine would be the first line of treatment, regardless. So, if they do that and they get better, then great. If they get better, then take the neck brace off and get much worse again, then they should come and see a doctor who knows-- or, a really good physical therapist who understands Ehlers-Danlos syndrome. [Kaitlyn] That's great! Um, actually.. there was a question about cervical traction. What are your thoughts on that before surgery, as-- kind of how you were just explaining. Is it common, also, that new symptoms appear after the traction is applied? How does this help make your surgical decisions? [Dr. Henderson] Well, Dr. Milhorat in Boston, and Dr. Bolognese [clears throat] are strong believers in invasive traction, where they put pins on the head and then apply-- can you see a picture of me? [Kaitlyn] Yes. [Dr. Henderson] Okay! And apply a traction, stretching the head out. And their belief is that if that gives a significant improvement, then that implies that there's a mechanical problem in the upper cervical spine. And I think that that's a reasonable assertion, however, I don't do traction myself because I don't think it's very specific, for one. And two, there are more definitive ways of determining if there's instability. So, I don't do invasive traction. I think, provided that a lot of weight is not put on the traction, for instance, for problems in the craniocervical junction, you should not put more than, five-- or, you know, maybe 7 pounds of traction on the neck. I think it'd be unlikely to get a neurological injury. [overlapping conversation] [Kaitlyn] Okay, so I'm gonna ask a question that came up in the chat because, actually, there are other questions— two people have asked it. If you have craniocervical instability, but also a tethered cord and it's possible that both surgeries are required, is there an order in which those two surgeries should be done? And if so, what order? [Dr. Henderson] Yeah, I generally-- I generally do the thing that's disabling the patient the most. If the tethered cord is much worse than the neck, and disabling them, then I'd probably do the tethered cord first. But, 90% of the time, I'll do the neck first. And that's a general neurosurgical principle: to start at the top, and then go down. Furthermore, I've had some cases where when I fix the neck, their tethered cord symptoms got much better and I didn't have to do the tethered cord. [Kaitlyn] Wow, that's great. So, this is kind of reminiscent of what we just spoke about but it was a question that came up in the chat and one that was asked prior. What's the process for diagnosis of craniocervical instability and atlantaoaxial instability and what would your doctor order, as far as tests for this? And how would it be treated? [Dr. Henderson] We diagnose craniocervical instability based upon the typical symptoms of the cervical medullary syndrome. Headache, neck pain, nausea, visual changes, auditory changes, vertigo, choking, speech changes, weakness and sensory loss. And then, the presence of neurologic deficits like real weakness, maybe hyperreflexia-- increased reflexes-- abnormal gait. [clears throat] And radiologically, flexion/extension MRI is what I use for craniocervical instability. We look at the Harris measurement. And there are some other measurements, as well. And for atlantoaxial instability, we do the CT scan with neck rotation to left and right. And now, sometimes patients can't turn their head far enough to the side, in which case, we obtain an open-mouth odontoid view on fluoroscopy, it's-- it's called a "DMX", a digital dynamic fluoroscopy. The patient will tilt their head, full left and full right. If the C1 slides too far on C2, that demonstrates an instability. [Kaitlyn] There are a couple of questions that have come up about fusion surgeries. How do you make the decision that it's time to have a fusion? And what are some of the outcomes as far as impact on range of motion, or long-term outcomes of that kind of surgery? [Dr. Henderson] If they fail all of the conservative, non-operative regiments-- they have to try those- surgery is the very last resort. Okay? After everything else has failed. And if there is instability, then the fusion removes the instability. We don't have a way of stabilizing the occiput, C1, C2, without doing the fusion. Lower in the neck, [clears throat] now they have methods of you know, doing sort of artificial discs that you can place that will leave a certain amount of movement without a fusion. But, if there's a lot of instability, an artificial disc is not appropriate. But, at the craniocervical junction, if there's instability, and they're symptomatic, then you need to do a fusion. Most patients who have instability at the top end of the neck, they have plenty of movement below, so even if you fuse cranium to C2, they still have a very, almost normal cervical range of motion. And so, none of my patients-- I don't recall any of them complaining about inadequate range of motion. So, that's not an issue. [Kaitlyn] If a patient also has a Chiari and they're getting a fusion as well, for craniocervical instability, are these procedures often done at the same time? Or, again, is this something that's done in a certain order? [Dr. Henderson] Well, tomorrow I'm doing a patient who's got a very bad Chiari, and she also has craniocervical instability. And, I'm actually going-- tomorrow I'm going to take care of the Chiari, and in one month, I'm going to bring her back and take care of the instability. Normally-- 95% of the time-- I'll do it all at the same time. But she has very big Chiari and I was afraid that if I do the appropriate surgery for the Chiari, that there's a high risk for a very small amount of leakage of spinal fluid compromise the fusion and I don't want to take any chances. So, most of the time, we take care of them both at the same time. But when there's a really bad Chiari, then it's very reasonable to split it up. [Kaitlyn] One of the questions that came in was kind of about syringomyelia. Is it true that syrinx will continue to grow? Particularly in a child who has Chiari and syringomyelia, unless the surgery for decompression is done? So, for this particular patient Chiari runs in the family and the child's symptoms are extremely mild, reporting tussive headache at the back of the head, lasting less than a minute and [occurring] after a Valsalva maneuver-- or strain. But the parents are unsure if surgery is the best option because of this-- because it's not really impacting the child's life, but at the same time, there's that syrinx there and so the physicians are a little nervous. They want to take that Chiari down and hopefully get that syrinx to go away. Is it that the syrinx will continue to grow and become more of a problem? Or, what do you say? [Dr. Henderson] I mean, my belief is that the syrinxes grow when there's a mechanical deformation of the brainstem and of the spinal cord. And so, as long as you have that mechanical deformation, there's probably going to be some growth. And the corollary of that is it's not just a matter of taking care-- of decompressing the Chiari-- sometimes, those patients have craniocervical instability, as well, or a very kyphotic clivoaxial angle. And if you don't correct all of that, if you don't treat them like a "complex Chiari', and take care of the clivoaxial angle and the instability at the same time, then the syrinx will remain. And there are a couple of studies that suggest that fact. That said, if the child has no symptoms, I would simply follow and only give the surgery when it's obvious that the syrinx is getting too big. Or, you know, when the child starts developing neurological deficits. [Kaitlyn] Um.. One of the questions that just came up in the chat, [clears throat] is how one would diagnose Chiari malformation when it's not shown on the MRI-- now I don't know if that means the MRI report, or the actual image-- but all the symptoms are present. What would a patient do in that kind of situation? [Dr. Henderson] Well, then... A lot of patients with craniocervical instability have all the same symptoms of-- not quite, but close enough-- similar symptoms to hydrocephalus-- oh, sorry. To Chiari malformation. And so, if there's no Chiari, there's a good chance they have craniocervical instability. [Kaitlyn] So they would have-- they should get worked up for that? Maybe a flexion/extension MRI or something? [Dr. Henderson] Correct. [Kaitlyn] Okay. There was one question that we didn't get to in the last session that I did want to bring back up. Are there orthopedic or chiropractic techniques to reduce cervical medullary junction abnormalities that cause CSF flow blockages? So, CCI or instabilities? Anything like that? [Dr. Henderson] Yes, atlas-orthogonal-- a lot of patients with ligamentous laxity well, there are some where C1-C2 or the cranium-C1, are out of alignment. And if you can define the misalignment, diagnose it, then you can restore appropriate alignment. And that can improve CSF flow. But this is very sophisticated. And it's a very difficult thing to do. But, a very good-- there are some atlas-orthogonists who are able to realign C1, C2. And that's usually effective. The problem is, it's often effective only for a few weeks and then the situation may recur again. [Kaitlyn] Okay. Almost to that end, a patient wrote in-- kind of discussing their trials-- Is pain in the neck, shoulders, and upper back-- so that "cape-like" pain-- is that common, even many years after surgery? Some patients, like this one, have found themselves in PT for many, many years. Is pain like that, that's so persistent-- is that something that should be medically managed, or therapeutically in PT, or should a neurosurgeon address it? [Dr. Henderson] Well, first thing is-- yes they can have pain. And, many patients don't have the appropriate posture. And the head may be too far forward. So, for instance, if the patient has developed a habit, or somehow, they've been more comfortable holding their head an inch forward of where it should be, that adds 10 pounds of force that the poor neck muscles have to withstand. So, every inch forward, the head exerts an extra ten pounds of leverage. And that can cause, you know, burning and muscle pain posteriorly. So, posture is really important, number one. Number two, it often takes patients a year and a half to fully learn how to-- for the muscles to be firing appropriately and working in a coordinated fashion. So, after the surgery, that's the beginning of a you know, say two-year process. And they need to be working on posture, and muscle strengthening, and correct dynamics. And second, there are other causes of neck and shoulder pain, and that may be instability at C3,4 or C4,5 which causes burning over the shoulders, burning between the shoulder blades, muscle spasm, and so on. You can have temporomandibular joint pains that trigger the levatoscapulae muscle spasm. So that causes pain down the back of the neck and into the upper-medial quadrant of the scapula, the shoulder blades. And that's from TMJ. So, you know, there are a few reasons for muscle spasm. [Kaitlyn] So say it's five, ten years down the line-- is there ever a reason where maybe it's getting to the point where a neurosurgeon should look into it? [Dr. Henderson] Well-- if you'll-- one second? [Kaitlyn] [laughs] Okay! No problem. I actually just sat upright when we just talked about that. [Dr. Henderson speaking into phone] [Dr. Henderson] Okay, Kaitlyn? Yep. [Kaitlyn] Yeah, all good! I noticed when you gave that answer, I sat upright, so... [Dr. Henderson] Say again? [Kaitlyn] When you gave that answer, I sat upright and my back pain went away. [Dr. Henderson] Oh, is that right? [Kaitlyn] Yeah! [laughs] [Dr. Henderson] Oh, should they see a neurosurgeon? Well, they ought to make sure they've got their posture right and-- and they're doing all the right physical therapy things. And they ought to make sure that there are not other issues, like TMJ. And then, they might get a flexion/extension MRI of the cervical spine to look for instability. [Kaitlyn] Okay. That's good advice. A couple of questions actually came up in the chat. I guess-- I will start here.. Is flexion/extension X-ray a valuable image to diagnose craniocervical instability, or does it have to be an MRI? [Dr. Henderson] Yeah, you'd think that an X-ray would be okay. And in the most severe cases, you might see it on an X-ray. Most of the time, you cannot see it on the X-ray. And that's because, on an X-ray, you're looking through a lot of thickness and you really need something tomographic, in other words, you need to slice through the middle of the skull-- you don't want to be looking through the whole skull and the whole spine. That tends to average everything-- and X-ray tends to average the full depth of what you're looking at, and most of the time, you won't see craniocervical instability. [Kaitlyn] So, it's more that you have 2D image in an X-ray and you want a 3D look at someone's entire head when you're talking about this? [Dr. Henderson] Uh-- well, you don't really need a 3D... a tomographic is 2D. An X-ray, actually, in a sense, is more 3D. [laughter] Now, you can do a 3D reconstruction, of a CT scan and that-- that might show it in better detail. [Kaitlyn] Okay. If testing shows significant craniocervical instability or atlantoaxial instability, but the patient feels that their symptoms are under control, is monitoring appropriate in that instance? Especially if-- [Dr. Henderson] I'm sorry, could you repeat that question? [Kaitlyn] Oh, sorry, yeah. So testing is showing significant instability, but the symptoms aren't bothering the patient to the point where they think it's necessary to necessarily have surgery maybe-- is monitoring appropriate in that situation? [Dr. Henderson] Yeah. I mean, I have patients I've been following for years they have a horrible looking X-ray, MRIs-- but they're still functioning quite well, so, you know? It's hard to beat that with surgery. [Kaitlyn] Um, for migraine medication: a lot of patients use Botox. Is that something that should be avoided for any reason in instability patients? Or any others? [Dr. Henderson] Well, Botox paralyzes the muscle, so it's going make them a little bit weaker. Sometimes, it helps. Generally speaking, I think it's expensive and it doesn't really get to the root problem. And it's temporary. [Kaitlyn] Are there better medications? For migraine? Or, I guess it depends on the headache, correct? [Dr. Henderson] Well, I don't have any experience with Aimovig AIMIG but that's meant to be good. I think Lamotrigine might be helpful. A lot of what's called "migraine" is actually "cervicogenic headache", meaning the pain begins in the upper cervical spine. And then, some migraines are the result of tiny little blood clots that pass through the heart and go up to the brain. And those little blood clots can cause some migrainous type headaches. And so, plain old Aspirin might be helpful! Um, but in my experience, neurologists call everything a migraine. [laughs] And the real number of migraine-- I think some neurologists and found that 60% of diagnoses for headaches are incorrect, and that migraine diagnosis is one of them. But, well... [Kaitlyn] Um, there's a call back-- you spoke about atlas-orthogonal workups. Is that something that's recommended for patients with CCI, or constant neck pain, headaches, that are not explained by-- [Dr. Henderson] Not CCI, but AAI-- if you have a diagnosis of AAI, it might be good. It can helpful yes. [Kaitlyn] What about TMJ? [Dr. Henderson] What about it? [Kaitlyn] Is that something that you would use atlas-orthogonal workup for? No? [Dr. Henderson] For TMJ? No, you'd see an oral surgeon for that! [Kaitlyn] Yeah, that's true! What are the symptoms of basilar invagination? And what would suggest that it's severe and requires a consult to neurosurgery? [Dr. Henderson] Well, severe pain and disability, neurological disability would be a reason to see a neurosurgeon. And the symptoms should include weakness, and usually, difficulty speaking, or swallowing, or choking, or sleep apnea, and gait changes, you know, abnormal walking, and then maybe vertigo, nausea, memory, balance changes. And the straightforward basilar invagination on regular MRI will show that. [Kaitlyn] Okay. There's actually a very interesting question that came up about first responders, emergency workers. Is there anything that someone with instability who experiences head pressure, neck pain, headaches, even without wearing PPE, can some of those tight-fitting respirators and the face masks that are pretty hard, or hard hats-- is anything like that going to make symptoms worse? [Dr. Henderson] Yeah, does COVID-- I haven't heard of any EDS patients having COVID yet. I mean, I would think it would have gotten back to me, because I have so many patients. That would be good, if somehow, EDS confers some kind of immunity. I think COVID could be tough because if there's coughing, that could make things worse. Or proning, if you're lying prone on a mattress,-- which is one of the mainstays of severe COVID infection for the lungs-- you'd need to make sure that your head, your neck was straight when you're positioned face down. [Kaitlyn] Would a mask or even, say for a firefighter if they had a hard hat on, is something like that going to give them additional problems? Regardless of COVID? [Dr. Henderson] You mean, you say if you're a firefighter that had cervical instability? [Kaitlyn] Yeah. And you have some sort of EDS, Chiari, and-- if there's some sort of pressure being put onto the back of that head? Or front? [Dr. Henderson] Um.. [Kaitlyn] Could that exacerbate symptoms? [Dr. Henderson] Well.. I don't think wearing any hats and masks would necessarily exacerbate a symptom. The kinds of activity that exacerbate problems are flexion/extension, and bumpy roads, and a lot of neck rotation. [Kaitlyn] Mmhm. [Dr. Henderson] So, it's the activity more than what's put on the head. [Kaitlyn] Okay. There's an interesting question that had come in before we got on the call, asking about long-term issues with degenerative disc disease and other degenerative issues as far as: should patients be concerned if they had EDS or Chiari with age, as they get older with degenerative problems? With neurological issues? [Dr. Henderson] So, can you say that again? [Kaitlyn] What are-- I didn't ask it very well. What are the long-term issues that patients with EDS, or Chiari or instability should be worried about with neurodegenerative, neurodegenerative diseases? [Dr. Henderson] Well, first thing: it's not a neurodegenerative disorder. You know, the nerves are more susceptible to injury, given the instability. But the brain, brainstem, spinal cord, is not more-- it's not degenerative per say. It's the spine. The discs, as you mentioned earlier, all of the EDS patients have degenerative disc disease. But over time, degenerative disc disease leads to more stability. So, the neck becomes more stable as they get older. So, it's a young--it's the period between you know, 16 and 30 where I see most of my patients. And I see some, you know, in their 50s. On the whole, I don't think this is something where you dread getting old. On the contrary, the real problem's when you're young. [Kaitlyn] Interesting! Because the follow-up question was would there be a need for additional fusion surgeries and, based on what you're saying, the answer would be 'no'. If degenerative disc disease were to progress. [Dr. Henderson] Yeah, degenerative disc disease-- everyone's got it eventually. Only 1 or 2% of people need surgery. That's not a long-term problem. [Kaitlyn] Okay! Is adhesive arachnoiditis a major contraindication for surgery? That's very specific. [laughs] [Dr. Henderson] Well, actually that's something that's overdiagnosed. We used to see bad arachnoiditis back in the 70s from some of the agents put in, like Pantopaque. But there are very few cases of bad arachnoiditis now. Now, some people-- you can get arachnoiditis from surgery or from trauma. Sometimes, that tethers the cord, and it may need to be dealt with. But it's not a contraindication to do anything. [Kaitlyn] Okay. Is there any specific radiology practices that you know of that really do 3D CT... Because I know that's not a very common... [Dr. Henderson] Any radiology department can do a 3D CT-- well, it's not a-- it's been around a long time it's a fairly basic thing. You can call up and say, "Can I get a 3D CT?" And it should not be a big issue. I know at Doctor's they do them more routinely. At Doctor's Hospital. Yeah. [Kaitlyn] Um.. is there any way you can speak to dysautonomia a little bit. So, I know that's a problem for a lot of patients who have Chiari, and I know you're doing work to research the treatment of dysautonomia, so I don't know if there's any way you can speak to that. [Dr. Henderson] Well, we have a-- two manuscripts coming out on AAI-- atlantoaxial instability. And the surgical one touches on the autonomic nervous system, and the one which is-- that'll be in Neurosurgical Review and hopefully available soon. But that's more for surgeons. And the second paper's probably a little bit more for clinicians and/or patients. But there are many causes of autonomic dysfunction. The autonomic fibers begin in the hypothalamus and go down through the brainstem and the spinal cord. And then the sympathetic fibers exit through the thoracic spine from T1, down to about L2, the second lumbar vertebrae. They go to all parts of the body. So, in the spinal cord, they're susceptible to mechanical deformation or compression, or injury and they result in dysautonomia. But dysautonomia can also be the result of peripheral neuropathy like diabetes. Or it can be a central neurodegenerative disorder like Parkinsonism or [inaudible] syndrome. As far as the brainstem and spinal cord type of dysautonomia, often we can improve that say, with reduction-- in other words, with realigning the spine craniocervical junction and the stabilization and fusion. The other thing about dysautonomia, is it seems to trigger mast cells all over the body. So, mast cell activation syndrome often goes with dysautonomia. [Kaitlyn] That's interesting. No one's asked about that, but do you want to quickly describe how mast cells activate different symptoms that might be, either mistaken for Chiari symptoms or dysautonomia, or causing them? And how treatment by neurosurgery may or may not fix those things? [Dr. Henderson] Okay—well, we didn't learn anything about mast cell in medical school. But, the mast cells are the most primitive immune system in the body. And they respond to environmental triggers-- anything from environment to bacteria, parasites, viruses, even emotional stress or mechanical stress. And when they're triggered, they can release any of at least fifty known types of chemicals, causing the brain fog, headache, congestion behind the eyes and nose, difficulty swallowing, you know, "dysphagia". Gastric reflux. They can cause abdominal swelling, you know, loss of peristalsis. Most common thing they cause is flushing-- you know, the skin turning red like a rash. So they get rashes, eczema. If you scratch the skin, they may have-- a welt will come up. That's called dermatographism. [clears throat] They often have allergies to food, or even anaphylaxis to drugs. There may be joint pains, muscle aches. Interstitial cystitis can be due to mast cell. Food intolerances, I said. And "dyspnea", breathing problems. And these can be made worse by a faulty autonomic nervous system. And conversely, we've seen that sometimes, when we fix the spine and improve the autonomic function the mast cell improves. [Kaitlyn] Hm! [clears throat] That's interesting. How do you prevent something like arachnoiditis, or adhesions that require repeat revisions and different surgeries following that? [Dr. Henderson] If you get bad adhesions and arachnoiditis, often, you know, every 5 years it needs to be redone again. Could you excuse me one second? Someone [inaudible] I'll be right back! [Kaitlyn] This is the problem-- Dr. Henderson wasn't supposed to speak today, so he's doing us a big favor. So we have a couple more minutes. I'm going to actually mute him so it doesn't-- Well, that's fine. If I speak quietly enough... Um if you have any further questions, we have a couple of minutes left in our hour. I know we got started a little bit late, so if questions do pile up, we can add up a couple more. Does anyone have any specific questions regarding I guess-- maybe tethered cord, or other-- there have been a lot of questions today about instability. So, if there's specific questions about instability that maybe you either feel that we haven't answered or maybe we didn't understand the question when it was asked, feel free to ask us again! I see that there are one or two that have been added. I'll ask him when he comes in. I have some interest in vascular issues, as well. So, if anyone has any specifically about those, as well... [Dr. Henderson] Okay! [Kaitlyn] Oh! Okay, hello! [laughs] Um, so we got a couple more questions—we have a couple more minutes left in the hour that we were going to put together. Is it appropriate to wear a hard collar, when symptoms are calling for it, more full-time? Is it okay to wear a collar a lot of the time if it's improving your symptoms, I guess is the question. [Dr. Henderson] Yeah, well.. the standard doctrine with neurosurgeons and spine surgeons is: if you wear a neck brace, you're going to get muscle atrophy and make your neck worse. However, with Ehlers-Danlos syndrome, suddenly, sometimes you have no choice; the pain is too great without a brace. But if you do the isometric exercises, six times a day-- and that's pushing against your hand forward and backward, and rotation, and lateral tilt, left and right. If you do those six times a day, you know, religiously, then you can preserve your muscle strength so I routinely get patients to wear the neck brace for 2-3 weeks, night and day provided they do the isometrics to get them into a better place. [Kaitlyn] Yeah, actually-- I'd been having some TMJ pain the past week and I found great videos on YouTube that helped so you can just go on YouTube, I promise! [laughs] So, there was a question about facet joint injections. About how many weeks or months of relief does something like that give? And what are the pros and cons of something like that? [Dr. Henderson] Well, facet injections are useful for diagnosis. If you suspect that there's "facet arthropathy", meaning a specific degenerative fact joint that's causing pain. Frankly, I don't see many of those, despite the thousands of patients I've seen. We see it in the lumbar spine. I don't see it much in the neck. More often, we see unstable facet joints and injecting those-- especially if you put steroid there-- you'll just make it more unstable. Moreover, the steroid often has a preservative in it that is inflammatory to the nerves and can increase the pain. So, when I inject [clears throat] so, it's usually I-- I prefer a type of long-acting type of [inaudible] like Exparel. And you're looking at a benefit of a few hours or a day or two... But I don't think-- it's a little hopeful to expect long-term benefit from a facet injection. [Kaitlyn] There was a question based on your explanation of mast cell. How do you decide which symptoms are resulting from mast cell activation, versus craniocervical instability, or the like? And, I guess, when you correct that instability, do those symptoms get better/worse? [Dr. Henderson] Um, well it can be a little difficult to separate them. And they tend to run together [clears throat] a little bit. But you don't want to have surgery for mast cell symptoms. [Kaitlyn] Yeah. [Dr. Henderson] And um.. If you have really severe mast cell, there's a drug called Xolair-- X-O-L-A-I-R-- that is very expensive and effective. You take it once a month for treating the severe mast cell. But it's worth seeing a really good allergist and there's a good guy in Washington, Talal Nsouli, N-S-[O]-U-L-I, Nsouli, at Georgetown-- Allergy... what is it? Georgetown Allergy Clinic? Something like that... And uh, Anne Maitland, of course, is one of the best in the country. And Lawrence Afrin is one of the best in the country. For a few examples... And then, Castells up at Brigham. But uh, you want to get your mast cell, treated, ideally, before a neurosurgical procedure. [Kaitlyn] Right. Sort of to that end, there's a question that came up that a patient who has a lot of different issues, so EDS, Klippel-Feil, dysautonomia, a moderate scoliosis, some basilar invagination, but also has autism— [Dr. Henderson] I'm sorry, what? [Kaitlyn] Autism. [Dr. Henderson] Yes. [Kaitlyn] And I know you've looked into that a little bit, as well.. the patient themselves doesn't want surgery, but is it dangerous to wait to get neurosurgical intervention for certain symptoms or conditions? [Dr. Henderson] Well, first I'll— there are a lot of people with autism who have craniocervical instability and I think-- I mean, I've operated on a number where some behaviors went away. I followed them a long time before I operated on them, but they had all the findings... Second, when you clearly make a diagnosis-- if someone's really symptomatic, then it's probably better to proceed sooner. I think the longer you have the problem, the longer it takes to get over it. More things have to repair. [Kaitlyn] Mmhm. Um, there's a really technical question that came up... about when-- so, when an EDS patient, for example, is wearing that hard collar, there's obvious discomfort at the back of the skull, or even the skin. Is there anything that the patients can do to kind of ease that discomfort in the process? [Dr. Henderson] Ah... Try a different neck brace. [laughs] Miami J is better-- if you've got bad TMJ problems, then a Miami J is more comfortable for TMJ. The Vista brace tends to push up on the jaw and exacerbate the TMJ. However, generally, ninety percent of the time, I use the Vista-- I think that's the best brace. [Kaitlyn] Yeah, and then-- I guess I'll close out on a project— that I know you're working on, because I'm working on it with you— is some tethered cord stuff. What is the proper course of monitoring, versus surgical intervention for a tethered cord patient? Maybe they may or may not have any kind of fatty filum visible on their MRI. What would a patient do to advocate for themselves if they're experiencing tethered cord symptoms and not getting any relief from management? [Dr. Henderson] Well, first off, the primary symptoms are severe back pain, leg weakness, numbness in the legs, and bladder difficulties like urinary incontinence, urgency, frequency, incomplete bladder emptying, nocturia-- going at nighttime-- or if they're children, enuresis, you know, bed wetting. There can be sexual dysfunction, there can be rectal incontinence. And uh, typically, if they sit on the floor, and stretch their legs in front of them, that stretches the nerves, and that should exacerbate the pain. And they typically have a lot more pain walking up an incline, up steps. Anytime they're bending forward, stretching the nerves, it increases the pull on the spinal cord. Those are the symptoms. If they have all of that, then, especially if they have a foot deformity-- flat feet or club feet or hammer toes-- or if they have a mark on their low back a little cafe au lait spot, or a hairy patch or a tiny little hole there, those external findings we see— The MRI can show the end of the spinal— Traditionally, [in tethered cord] the spinal cord will be down at the third lumbar level. But in '94, one of the top neurosurgeons showed that about 20% of tethered cord exist with a "conus medullaris", or the bottom of the spinal cord, ending at a normal level, like L1. So, the spinal cord can be at a normal level. There are a few other radiological tips: fatty filum-- a little white spot in the filum. Or a spina bifida occulta. Or scoliosis. Or a very straight spine. Or a, um, a little syrinx in the lower part of the thoracic or lumbar spinal cord. There are other tips, but the clinical findings are the most important and if they have them, a urodynamic evaluation by someone who knows what's going on-- and I use a group in Baltimore who work with Donlin Long, the former Chairman of Neurosurgery at Johns Hopkins--that's David Gordon and Rupinder Singh-- and they do very detailed urodynamics. And at the end of that [testing] we're pretty sure, one way or the other, if they have tethered cord, or not. They should really have a urodynamic test--urodynamics-- to confirm the diagnosis of tethered cord syndrome. [Kaitlyn] Okay. And then, I promise this is the last question now! [laughs] There was just a follow-up to something you had said about the autism symptoms sort of correcting after the corrective surgery... Were those patients high-functioning, or low-functioning autism? [Dr. Henderson] High. [Kaitlyn] High? Okay. Yeah. Well! Then that's all. [laughs] So, um-- I want to thank Dr. Henderson for joining us tonight because it was not in his original plan. I really appreciate that. There was an emergency. Thank you all for coming. I know tonight was a little bit spastic because a lot of stuff was going on, [Dr. Henderson] I think... I don't think I've ever used the GoToMeeting before... My Zoom works better. But I'm not sure (Oh) what was wrong with this... But that was clever, Dorothy, to use the cell phone. [laughs] [Kaitlyn] Yeah. Sometimes you gotta go low-tech. Alright. Thank you all! [Dr. Henderson] Hasta la vista! [Mary] Thank you, Dr. Henderson!

Revised: 6/2020