Ask the Expert: Craniocervical Instability with Dr. Fraser C. Henderson

VIRTUAL MEETING - JUNE 18, 2020

The craniocervical junction is the area at the back of the skull which houses the brainstem, the cerebellum and the top of the spinal column. Abnormalities in this region can cause a collection of symptoms that are common in Chiari malformation, craniocervical instability, Ehlers-Danlos syndrome and related disorders. These symptoms also can overlap with those of other conditions like tethered cord syndrome or mast cell activation disorder. In this Ask the Expert, Dr. Fraser Henderson answers questions about craniocervical instability, Chiari malformation, tethered cord and Ehlers-Danlos syndrome from the perspective of a neurosurgeon. All questions were sent in by real people impacted by Chiari malformation, syringomyelia and related disorders. This presentation was given on Thursday, June 18, 2020 and was presented entirely online. (2020) 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] Okay. There we go. Hello, welcome everyone. Thank you all for coming tonight. I know tonight we have Dr. Fraser Henderson with us, so I'm just gonna briefly introduce him and then we can get started. Dr. Henderson received his Bachelors and Medical Degree from the University of Virginia at Charlottesville. After graduation, Dr. Henderson served for the multi-national peace-keeping force in Beirut, Lebanon. He earned the Navy Commendation Medal for his service. He then returned to the U.S. to complete his residency at MUSC before going back to active duty as commander at the National Navy—Naval Medical Center in Bethesda where he was the brigade neurosurgeon. He then completed his fellowship in cranial-- craniospinal surgery at the National Hospital for Neurology and Neurosurgery in London, England. And he became the Director of Neurosurgery of the spine and craniocervical junction at Georgetown. He currently serves as the Director of Neurosurgery at Doctors Community Hospital and serves patients at Metropolitan Neurosurgery Group in Silver Spring, Maryland. He's invented numerous devices related to the brainstem and spinal cord and he's authored-- I checked the last video-- over 90 peer reviewed articles and books. And as part of a mission to continue education about craniocervical disorders and Ehlers-Danlos syndrome, different spinal abnormalities, he's given over 190 invited presentations. And in his spare time, when he's done doing all of that [laughs] he serves on our SEA Board, our medical board. And he's arranged countless in-person and now two virtual presentations to educate health care providers, patients and their families. So, we are really, really grateful that Dr. Henderson could be with us today and with that, I'm gonna see if Dr. Henderson wants to say anything? [Dr. Henderson] Nope! All ready to go! [Kaitlyn] Cool. Alrighty then. So, let's get started. I'm gonna start with some symptomatic questions. There were two questions kind of related to vertigo. Do you know what percent of Chiari patients have vertigo spells as a number one issue? And is vertigo more typical of craniocervical instability? Or, is it typical of Chiari? [Dr. Henderson] Yeah, I think that vertigo would be more typical of craniocervical instability. I would say dizziness is extremely common with Chiari and craniocervical instability. Vertigo is specifically a type of dizziness where the room is spinning around the patient. So, it's a more specific type of dizziness. And I'd say it's a little bit more common with craniocervical instability. [Kaitlyn] Do you know if there’s a percentage of Chiari patients who typically have vertigo? Or, is it just prohibitively small? [Dr. Henderson] No, I-- I'd say-- it's a common-- Milhorat's paper mentions that number and it's been a while since I've read it, but I think it's in the order of about 35%. [Kaitlyn] And we can try and locate that also and share it with the group, too. What are some symptoms of occult tethered cord in Ehlers-Danlos patients? [Dr. Henderson] Occult tethered cord. Alright. I actually like the term "tethered cord syndrome" as opposed to "occult tethered cord". Tethered cord syndrome refers to the symptoms and signs that arise from pulling down of the spinal cord by a thickened remnant of what's called a "filum terminale". When a baby is-- in the third trimester, the distal third of the spinal cord atrophies and forms a tissue called the filum terminale, which is like a little fishing line from the end of the spinal cord to the base of the spine. And normally, this is very floppy, but in some people, the filum can be thickened, become inflamed, and pull downward on the spinal cord. And that probably causes injury to the lower spinal cord. It's generally assumed that pulling causes injury. So the patients have low back pain, leg pain, numbness and weakness, sensory loss in the lumbar and sacral dermatomes, and urinary problems, like urgency, frequency, hesitancy, incomplete emptying and incontinence. Almost all patients with tethered cord have urinary-- severe urinary symptoms which have to be confirmed with the urodynamics, which is electrical and pressure-testing of the bladder to confirm the presence of neurogenic bladder. In addition, about 10% of patients have rectal incontinence. About half of the patients have some degree of headache, due to the tethering. In my experience, some even have increased "dysphagia", difficulty swallowing. And we know, in children, that tethered cord can result in delayed speech. And children also have developed scoliosis. Many of them may have toe-walking, where they walk on their toes. Or, they have-- their legs may be turned inward, or one leg may be slightly atrophic. Very often, there are neurocutaneous markers on the back, like a hairy patch or a dimple. Radiologically, you might see a number of findings including scoliosis. You may see the spinal cord pulled downward. Usually, the spinal cord ends at the L1 level. But in tethered cord syndrome, it may be pulled all the way down to the sacrum. It might be a thickened filum-- that little band that I talked about. Or it may be filled with fat, and that shows up on MRI. Sometimes, we see a small syrinx, a small amount of fluid in the spinal cord in the thoracic or lumbar spine. And we often see "spina bifida occulta", which is a-- incomplete lamina, backbone, at the S1 level. [clears throat] So, they're the radiological findings. And when people say "occult tethered cord" , they're just saying that the spinal cord is ending what is generally a normal level. But, it's well-established in the literature going back to the 90s, somewhere between eighteen to 28% of those studies back in the 90s had-- patients had tethered cord syndrome, even though the spinal cord ended at a normal level. [Kaitlyn] Sticking with some symptomatic questions... One came up in the chat: is "strabismus", so being cross-eyed, related to Chiari? And can it be made worse from tethered cord, or something else being present? [Dr. Henderson] Uh, yes, you can get strabismus from Chiari malformation. Could it be worse with tethered cord? I've not-- I've never read that, but I'm sure there are occasions where. tethering can exert an influence all the way up-- but I would-- that's not something that I would really... that is to say. [Kaitlyn] Yeah. There are a handful of questions-- I'm gonna try and do this in a flow that makes sense from symptoms, diagnosis, and all that. So, there's a question here: are clinical symptoms, or provocations-- are there any clinical symptoms or provocation tests that a PT-- it would be safe for them to administer in clinic for specifically hypermobil-- blah-- oh, my-- hypermobile EDS. [Dr. Henderson] Okay, in order to diagnose Ehlers-Danlos syndrome? [Kaitlyn] Yes. [Dr. Henderson] Okay. Yes. In fact, I think that physical therapists could be really central to the diagnosis of EDS. And they-- they're really a great part of the medical providers. And this is right down their alley. So, number one: patients with EDS have to have joint pain. So, they have, you know, knees, elbows, big joints, not the little ones. The little joints tend to be things like rheumatoid arthritis. The hands, the feet. That's rheumatoid arthritis or lupus. But in EDS, it's the big joints. So, you have: elbows, shoulders, hips and knees, generally. Maybe ankles. And so they have joint pain. Several joints more than-- more than a few months. Secondly, they are um, very hypermobile. This may not be evident: the patients tend to be quite athletic. Most of the patients I've seen were very athletic, at least in their early adolescence. You can apply the test of hypermobility. And that's very simple. So, one of them is bending the thumb back to the forearm. Now, I can't bend my thumb more than that far but if I could bend it back to touch my forearm, that would be very hypermobile. Another test is bending the little finger. Now, I can bend my little finger back that far, if I could bend it back parallel to my forearm, then that would be-- that would be one point there. One point for each thumb going back. If I bend my elbow, but if you have EDS, then it goes backward a little bit. And that's 15 degrees more than normal. And the same with the knees. So, patients bend the knees backward and introduce a little reverse bend. A point for each of those. And then if the patient can stand with his or her feet together and place both palms on the floor, that's one point. For adults, you need six points. Or, if you used to be able to put your palms to the floor, and you're over age 40, then you can have five points. With a history of being able to have 6 points. If you have six points or more, then you're in the race for hypermobility spectrum disorder. That could be anything from benign hypermobility which is very common. It's probably, at least, four percent of the population. This is benign hypermobility. And that's probably one end of the spectrum. If you have that, plus the joint pain, and you have skin changes in a normal person-- if you pinch the skin, you can pinch it up maybe, you know, maybe a centimeter, but with EDS, you can pull up the skin and it'll tent from the metacarpal of the well, the knuckles here, back to the wrist. You can tent the skin from the knuckles to the wrist. They also have very soft skin. They have Piezogenic papules, little bumps on the ankles when the patient stands up. And unexplained straie-- stretch marks. So, if they have all of those and a couple of other things then they have EDS. By the 2017 criteria. If there's a family member with EDS, then that's you're 1/3 of the way there. There are a few other things like a high-arched palate. The palate is normally flat. If it's shaped like a turret, inside, that is one of the findings. And there are a few more, but I don't want to.. okay. [Kaitlyn] [laughs] No, that's great! Those are really practical, too. So, that's very helpful. [Dr. Henderson] Any physical therapist can look up the criteria and make the diagnosis. You know? [Kaitlyn] Yup! [Dr. Henderson] It's a-- I get annoyed when people say "It's too difficult to make the diagnosis." You know, because it's not that. [Kaitlyn] I'm gonna shift a little bit. There are two kind of, definitional questions that people were curious about. One of them is: would you be able to define and explain what a "pre-syrinx" is? [Dr. Henderson] Um, well. It's sort of a nebulous concept in that sometimes on an MRI you can see softening, or edema in the spinal cord. And that, in another month or six months, that could turn into a syrinx. A little fluid filled cavity. [coughs] But I don't think that's been firmly established, you know? I think that's-- a number of us feel that's what it is. And I think that makes logical sense. There's an injury-- an ongoing injury to the spinal cord and that manifests as tissue injury and then later the cells die, leaving a cavity, which fills with fluid. [coughs] Sorry. COVID virus... [Kaitlyn] Oh, God. Don't even say that. You good? Hydrate, hydrate! There was another question about-- I guess a good thing to start with is what exactly is a "pseudomeningocele"? And do they always require surgery is the point of the question. [Dr. Henderson] Okay. So, a [coughs] Sorry. Pseudomening-- A "meningocele" is a collection of spinal fluid covered by the arachnoid, which develops outside of the spine. That's a meningocele. They're usually after... some sort of injury. There may be an out-pouching or there may be a tear in the dura. And the arachnoid can pouch outward and expand and that would eventually become a meningocele. Now, if you have surgery and you get a CSF leak, the CSF can leak into the space around the spine and eventually be covered by a "pseudocapsule", or normal tissue that just envelops that CSF. That's a "pseudomeningocele". It's not an extrusion of meninges, it's essentially a CSF leak. Now, if you leave a CSF leak long enough, provided the tissues over it are sealed, the meningocele will reach a pressure that's equal to the pressure on the inside of the spinal canal and it won't leak out anymore. It'll achieve an equilibrium; it won't keep growing. And so, many of these will eventually stabilize and maybe, gradually get smaller with time. However, many of these meningoceles can be quite painful. And eventually disabling to the patient. Some of them can break out and leak CSF and then cause a meningitis. And so, it's my posture to... if I see a pseudomeningocele and unless there's some reason not to, I'll go in, find the leak, fix it, or take some measure to get rid of the pseudomeningocele and take care of the problem. [Kaitlyn] Um, a question came up... There's been talk recently about.. Oh man, I always say the ME part very wrong, but ME/CFS, so chronic fatigue syndrome, um, that it's been-- it might be caused by craniocervical instability. Have you seen some similar conversations about it possibly causing-- uh, sorry-- tethered cord possibly causing CFS-like syndromes? [Dr. Henderson] Well, so ME is meningoencephalitis (myalgic encephalomyelitis), and I think that's-- My personal feeling is that may be a misnomer because that implies inflammation of the meninges, like a true meningitis. And, in fact, usually on an MRI, you won't see an inflammation of the meninges and it's not-- you usually don't see a big inflammation; there may be a few white cells there, but it's not a true inflammatory condition. That said, there's always a full spectrum so on the severe side, there's some-- real infections where you will see meningoencephalitis. You know, like Lyme disease can cause that, for instance... But I think that a lot of patients who are diagnosed with ME/CSF-- oh, CFS-- chronic fatigue syndrome-- actually have craniocervical instability and Chiari malformation. The only way to differentiate or to know would be to do all of the dynamic imaging and a very thorough exam and history and so on. Could tethered cord syndrome cause chronic fatigue syndrome? I wouldn't say it would cause it, but it could certainly exacerbate the whole problem. Fatigue can be multifactorial. Many—hundreds of things could cause fatigue. And tethered cord can cause severe headaches, and tiredness, and weakness of the lower extremities. Things that make you depressed. When I get depressed, it makes me very fatigued, you know? [Kaitlyn] Mhm. [Dr. Henderson] I mean, In fact, whenever I hit a medical problem and I don't know how to deal with it, immediately-- it immediately makes me tired, you know? [Kaitlyn] So, it could make everything a little bit more complicated, if anything. There are a couple of questions about this that have come up in the questions that were submitted, and a couple of people have been asking it in the chat. It boils down to: what are the imaging tests that help to accurately identify craniocervical instability. For instance, types of MRI, some types of CTs or-- someone specifically asked about DMX, so-- I don't know if you can speak to those... [Dr. Henderson] Right. Well, for craniocervical instability, the most useful test is a dynamic MRI. The dynamic flexion/extension, upright MRI. And what this involves is the patient sitting up and then flexing the head forward as far as he can, and then flexing it backward. And a guy Harris, back in 1994, showed that In normal people, the basion, the base of the skull, the base of the clivus, never moves more than 10 millimeters from the odontoid. 12 milli-- it never moves more than 12 millimeters on an X-ray. And X-ray is a slightly enlarged view, so 12 millimeters on an X-ray, means 10 millimeters on a CT scan. We adopted, with the Chiari & Syringomyelia Foundation in 2013, we adopted a standard that the skull base would never move more than 12 millimeters from the odontoid, the tip of the odontoid-- the spine. If it did, that represented craniocervical instability, which means the ligaments are too lax to hold the head in the right position and that-- then the head is sliding, the cranium is sliding back and forward on the spine and that's causing traction to the lower cranial nerve roots and traction on the brainstem and that causes headache and can cause... It causes headache and all the neurological symptoms of the cervical medullary syndrome. That's the definitive way of defining craniocervical instability. Now, if you can't have an upright MRI, then you can do a supine MRI lying down, and put a pillow under the head to flex the neck forward. And then put a pillow under the shoulders and allow the head to extend backward and get a supine flexion/extension MRI. And that would get you in the ballpark, you know, eighty percent of the time. Now, if you can't do that, then you can do the same same thing with a CT scan. A "cat scan". Pillow under the head for flexion, pillow under the shoulders for extension. And then do the same measurements. And the head, the cranium, should be pivoting over one point, over the odontoid. It should not be sliding back and forward. So, by measuring the basion-axis interval (BAI) which is the distance from the base of the skull, the basion, to the line drawn behind the odontoid. That's the basion-axis interval, or Harris measurement and that measurement should not be changing more than one or two millimeters. If you have craniocervical instability, it may be varying up to 10 millimeters. So, that's that. Now, it's very difficult to make the diagnosis with X-ray, and it's um,a nd for the same reasons, it's difficult with "DMX", or "digital dynamic fluoroscopy". Because there's so much-- there's such a great thickness of bone, that you're not seeing one point of bone, you're seeing the full width of the bone and you lose the accuracy and it makes it much more difficult to you know, for me anyway, to make the diagnosis of craniocervical instability. [Kaitlyn] Um, let's kind of stay in radiology for a second. There's a very specific question that I have a feeling came from and engineer or a radiologist. So, I'm going to ask it almost verbatim. CSF flow abnormalities on axial PC MRI cine are seen in symptomatic Chiari patients-- Chiari 1 patients-- that are certainly the result of elevated pressure. Elevated CSF pressure, to be specific. Although it appears there has never been a direct and invasive measurement of CSF pressure in mild traumatic brain injury patients, it's likely that some degree of elevated pressure might be present. Would a similar non-invasive diagnostic for mild traumatic brain injury be plausible, and perhaps supercede the comple-- or be complementary with the Glasgow coma scale? What do you think? [laughs] [Dr. Henderson] There-- so, my 200 page response... [laughter] Okay well, let me pick at a few sides of that question. If you have a head injury, typically, there's a little bit of bleeding. And a little bit of blood can actually-- is known to increase cerebrospinal fluid pressure and can cause acute hydrocephalus, or can cause normal pressure hydrocephalus which is a a mild increase in pressure that occurs in older people. It has significant effects. It is known that if you have a head injury, that the cerebellar tonsils very frequently descend. So, they did a study in Europe. Hundreds of patients. And they found that after trauma, the number of the number of low lying cerebellar tonsils, or Chiaris, were five times higher than the average. And so, they made the logical conclusion that if you have a head injury, that very often, that can cause a Chiari malformation. There are probably a hundred reasons or causes for a Chiari to develop. Some are genetic, and some relate to shape and size of the bone In childhood and babies. Some are-- if you have a brain tumor up here, that can cause a Chiari malformation. There are many, many causes. And head injury is certainly one. Moreover, you are more likely to pick up a low lying cerebellar tonsil with the flexion/extension MRI than with a [normal] supine MRI. So that said, the author of that question talked about the the flow characteristics of CSF. And that flow is mostly-- Changes in flow is mostly the result of obstruction of CSF. If there's a reduction in space then that can cause an increased speed of CSF flow through there. Obviously-- if it's completely obstructed, then there's no flow. People like Raymond Damadian, who invented the MRI, have demonstrated that the flow characteristics when you're sitting upright are quite different to when you're lying down. I think most neurosurgeons they look for flow or the potential for normal flow, but they don't hang their hat on-- they don't base their decision to operate on that. It's just one more thing to look at. Like these measurements that I've talked about. They're just something to look at, to add to, or take away from your decision to operate, or not. [Kaitlyn] We're gonna do one more question about radiology. Do you know if any doctors, especially now, given everything going electronic with COVID; are doctors willing to read those extension and flexion dynamic MRIs remotely? And if so, how do you get that kind of testing read? [Dr. Henderson] Yeah, I-- I think everyone who does craniocervical work. You know, I have two colleagues and we all read them remotely. We do it in conjunction with the-- You have to look at it in conjunction with the whole history. You can have wildly abnormal measurements, and the patient may not need to have surgery at all, when you put it all together. And I know Dr. Bolognese, and I think everyone who's doing craniocervical work reads these things remotely, even in Europe. Yep. [Kaitlyn] Okay, that's great! That kinda leads into getting into the surgical questions we have because the first one that I can kind of see flowing well is: what are the criteria for surgical intervention for say, a teenager, or an adult with Chiari, craniocervical instability and EDS? Either a decompression, alone or a decompression/fusion? [Dr. Henderson] Alright, well. If they have a Chiari, if it's a small Chiari, usually they just need a wide, but a relatively small decompression-- a foramen magnum decompression. If it's a big Chiari, then they need-- they usually need a bony decompression. And usually, opening up the dura and putting in a patch to expand the dural space and allow normal CSF flow, and take the pressure there has to be enough space so the cerebellum-- the cerebellar tonsil is not pushing on the brainstem. Now, if that-- if you're doing a big enough decompression, with a big dural patch, there's a significant risk of a meningocele forming because you've got several inches of of closure and if you leak just one drop of spinal fluid every hour, in a week you will have a meningocele. And if you get a CSF meningocele, that can be a major impairment to a fusion. The decision to deal with the Chiari is based upon the significant headache attributable to the Chiari, and the presence of some neurological findings, like lower cranial nerve findings, abnormal gait, maybe some spasticity. Those would be the criteria for doing the Chiari surgery. Now, most of the patients I take care of have craniocervical instability as the major problem, with or without a Chiari. And so, if they don't have a Chiari, then I don't do a decompression. If they have a small Chiari, I do a small decompression, and then a fusion stabilization. And if they have a big Chiari then I'll take care of the Chiari first and then come back and do the fusion stabilization. Now, the indications for a craniocervical fusion would be a significant headache-- usually a 7 or above. Occasionally, I'll do some someone with a headache less bad if they clearly—Well, occasionally, I will take lesser headaches. And the presence of a cervical medullary syndrome. These are all the symptoms attributable to pathology of the craniocervical junction. That would be the headache, neck pain, nausea, dizziness, maybe vertigo, visual changes, diplopia, blurred vision, altered hearing, tinnitus, ringing in the ears, choking, dysarthria, difficulty speaking, dysphagia, difficulty swallowing, odynophagia, painful swallowing altered sleep architecture, sleep apnea, abnormal breathing, weakness and sensory loss. Oh, and dysautonomia, which is dysfunction of the autonomic nervous system, which are all the everything that happens in your body that you don't have to think about. It just happens automatically. Then, on top of the symptoms, they have to have demonstrable neurological findings. So they should have numbness, almost always weakness. there's usually hyperreflexia, or spasticity. Instability of gait; they may be wobbly. A positive Romberg, where they close their eyes and lose their balance. And a number of other neurological findings. I want to see, clear neurological findings to back up symptoms. And they have to have tried made a reasonable go at non-operative treatment. So, physical therapy, neck brace, medications, isometric exercises, avoidance of those things that make their craniocervical instability worse. Usually I see patients after they've been doing this for a year, or sometimes, ten years. You know? Before they get to me. And so, almost every patients has already run the gamut of the non-operative treatment. But when there's clearly no logical alternative, then surgery works well. But it's not to be embarked upon until you've done the "easy"-- safe, easy things. [Kaitlyn] You had mentioned a little bit there that there's some risk of CSF leak if that's not sealed up well. How-- oh, I lost the question-- [laughs] How often do you find CSF leak in patients with CCI, especially if they're hypermobile? [Dr. Henderson] I um, you know, I don't know how many CCI cases I've done, but it must be quite a number. And I don't remember a-- once in a while if we're doing a craniocervical fusion stabilization and a small Chiari, there may be a tiny hole in the dura and we'll sew it up. But I don't remember there being a meningocele with a craniocervical fusion. It's pretty uncommon. But if you were to do a big Chiari, then it would be much more likely. Yeah. [Kaitlyn] Okay. There's a follow up question. And I think you covered this last week. Or, whenever that was. Is there a particular type of neck brace that you recommend? Does it it need to be fitted to the individual? And why a three-week neck bracing, rather than traction? [Dr. Henderson] Well, I think... Well, first, the brace I recommend is the Vista. Vista brace. And it has a little clicker on the front, and it allows you to adjust the height. And so, and the Velcro-- you can tighten up the vest to fit a small neck, or you can you know, loosen the Velcro and accommodate a big neck. So, I've found that the one brace appears to fit about eighty-five percent of patients. But if the patients have a lot of temporo-mandibular joint problems, then the brace-- the Vista brace-- by pushing upward on the jaw, on the mandible, it can exacerbate the TMJ and cause severe headache. And for those patients, a Miami J, which is more on the side, is more comfortable. So, that's the brace that I prefer. And then the other part of your question? [Kaitlyn] Um, why three-week neck bracing rather than traction? [Dr. Henderson] Well, it's a little hard to walk around. When I recommend bracing, I tell people: put on the brace for two or three weeks and wear it night and day. Take it off for meals, but otherwise wear it. But the neck muscles would atrophy if you don't-- if that patient did not do isometric exercises. They must do isometrics, build up their neck muscles while they're in the neck brace. The neck braces allows for the neck to gradually get realigned and settle down and some of the inflammation and pain to go away. Most patients get much better in the neck brace. Patients would all feel better with traction, but it's a little bit hard to have someone walk around behind you holding your head up twenty-four hours a day. We don't-- We used to do a lot more traction in neurosurgery but I think, you know, with the hypermobility disorders, one of the problems is that the ligaments are too stretchy. So, they feel good when you stretch them out, but they don't-- really, we want unstretchy! We want more stability in the ligaments. And so, if patients feel better in traction, that's maybe one indicator that they might do better with fusion stabilization. But, when we do the fusion stabilization, we're not really fusing them in a state of traction, we're fusing them in the ideal state of alignment. Without stretching things. So, I'm not opposed to the concept of it, but it's in my view, it's a little impractical. [Kaitlyn] Kind of bouncing off that: there are some questions.. What is the natural history of CCI and atlanto-axial instability without a fusion? So, the question really is: is fusion inevitable? Um, and what are the non-operative treatments or therapeutic options? Especially for pain? [Dr. Henderson] Well, Clair Francomano and Peter Rowe my colleagues and I are actually beginning a study right now to compare non-operatives with operatives. But it's still a retrospective. It's really hard to do a prospective study because if someone really needs the surgery, it would be very cruel to say, "I'm sorry, I know your pain is an 8 or 9 out of 10, and you feel like you're going to stop breathing every other night and every week you feel like you're having a mild stroke and you can't work and you can't do anything but we're keeping you in Group B, you're the experimental control group. And in a year or two, we'll get to you." You know? I mean, that'd be a little cruel. So, it's really hard to to do a prospective study. But most of the patients I see they weren't diagnosed yesterday-- I mean, they may have been diagnosed a few months ago, but they've been dealing with this problem for ten-- or, you know, somewhere between one and ten years already. So, I think that the very-- My guess I that say 100 people with severe craniocervical instability who might need surgery, let's say who met the criteria for surgery: if you didn't operate on them, then maybe over time, ten percent would recover... And I'm taking about over a few years; maybe 10% might recover without us to do something useful. And I would think the other ninety percent would all be on disability and live in misery for the rest of their lives. That would be my expectation based on the 2,000 odd patients I've looked at. [Kaitlyn] Okay. What are the non-surgical treatments to kind of get you until you get to that state where you need a fusion? So, physical therapy? There's actually a question that came up about cell regrowth, like stem cell therapies and stuff like that. I wonder if you have any thoughts? [Dr. Henderson] Well, the basic things are: first, physical therapy. Not range-of-motion; we don't want to increase range-of-motion, we want physical therapy to stabilize the spine from the lumbo-sacral junction up. Your posture begins down in the base of your spine. And it begins by pulling in your core muscles, you know, in the abdomen. And then it proceeds up the thoracic spine and up to the cervical spine. So, only a physical therapist knows how to-- or maybe some physiatrists and some osteopaths-- know how to correct the spine from the base, up. Secondly, they need to strengthen the spine. Build out the muscles around the spine. Thirdly, they need to avoid all those activities which are damaging to the ligaments in the neck. Like any contact sports. Anything from rollercoasters to bumpy boat rides or bumpy car rides. You know? Or, even getting bumped into on a dance floor. Or sitting in front of a computer with the neck flexed or extended. Or, looking at the cell phone too much. Or, playing games-- computer games. These are all things that are very deleterious to the spine. So, the patient needs to adopt a perfect posture. And then, these patients have premature degeneration of all the discs in the neck. And so, it's not just the craniocervical junction that's--- it's also the levels below. But most of the mobility is at C1,2 and cranium,C1. They're the hardest hit levels. So, they need to then begin to keep their neck in a good position at all times. And not be bobbing their head around and twisting and turning and looking down at a cell phone. They need to stay in perfect posture while they're awake, sitting, studying, driving, sleeping. You know, sleeping on their side with a pillow at their shoulders. Then they need to pay attention to good nutrition. Strengthen their bones and ligaments. Maybe Vitamin D. Maybe Calcitonin. Calcium citrate. A modicum of exercise every day to maintain muscle tone and posture and help the tissues. The isometric exercises as I have mentioned. Now, there are new things like "PRP". There's a guy in Colorado who injects the ligaments through the back of the mouth and that can thicken the ligaments and toughen them up. I'd be very afraid of an infection, but I presume he doesn't have many infections or he wouldn't still be doing it. Every neurosurgeon has tremendous respect for the craniocervical junction. And so, sticking needles in that part of the anatomy we think would be pretty dangerous because the vertebral artery can have unusual loops and whatnot. But those are the main things we do to-- for every patient prior to considering surgery. Surgery is the last option when everything else fails. And, in general, if a patient can still be working and doing the things he has to do, then he should not have surgery. It's when when they 're clearly going downhill and my goal is to get people back to the workplace, not on chronic disability, but back to work. And we accomplish that in about-- two thirds of patients. Back to school, back to work. Whichever. [Kaitlyn] Mhm. Kind of to that point: we had a question submitted prior and there was just a follow-up. Do you have any recommendations on how to basically, do those day-to-day activities like you're saying, without doing too much flexion or extension on your head. So, while you're sleeping, is there a particular pillow that would be soft enough, but hard enough and maybe another way to prevent you know, with the typing. All that. [Dr. Henderson] Well, the MyPillow things-- they really work. MyPillow. [Kaitlyn] Oh, wow! Okay. [Dr. Henderson] And this is not a paid advertisement. [laughs] The um, and getting in the habit. You can get in the habit and discipline yourself to sleep correctly at night. On your side. And to turn every few hours. So, that's something you can form the habit of sleeping correctly. You can form the habit of standing and sitting. And don't turn your head. You look at Grace Kelly. Princess Grace. Perfect posture, all the time. You look at Melania Trump. Never see her slump, or even turning her head. She's perfectly-- perfect posture all the time. Even if she's standing there reviewing the troops for an hour and a half. And the queen is not bad, either. Queen Elizabeth. Alright, so. Discipline. And no foolishness of activities that you might get injured. [Kaitlyn] Okay, so... these are kind of CCI-specific. In a patient with EDS and CCI or AAI there was bilateral compression of both internal jugular veins. And they developed collateral veins-- um-- and has developed collateral veins, as well. Does that kind of patient require craniocervical fusion? Or, are those collaterals going to be surgically an issue? [Dr. Henderson] I'm very careful to pay attention to the venous anatomy when I operate. We try to spare-- to preserve all the veins because the blood flow out of the brain flows-- half of it goes out the jugular veins and half goes through all the other veins. The veins around the spine. And so, if you go in and you trash all the veins in surgery then actually, probably, that's one way you can get more headache. That's not been demonstrated. That's conjecture on my part. I think we used to think of all the blood flow coming out of the brain through the jugular veins, but actually, blood flow comes out the jugular veins at night when you're lying supine. And during the day, more of the blood flow comes down through the spine and the other veins. And I think the reason for this might be that jugular veins can be quite large. And if they were just open, if they were just wide open, then blood flow would drain too quickly from the brain and that could cause problems. I suspect that there has to be a little bit of resistance to preserve a normal pressure in the brain and not have and not have this... If every time you stand up, and your jugulars were wide open, then you would overdrain and you might get a headache. A spinal headache. So, when we stand fortunately, more blood goes through the non-jugulars. When we do angiograms, CT-angiograms, a lot of the time, we find that one jugular or even both-- they look compressed. When you send them for an angiogram, you're not looking at a snapshot, you're looking at the flow over a few minutes. Actually, the flow is much more normal. So the CT-angiograms and the MRVs can be misleading in that respect. People who have CT-angiograms for whatever reason-- a third of patients have-- show that compression. But only a third of those have symptomatic-- are believed to be symptomatic due to that compression. So, you have to be very… Looking at the veins is a little bit of a rabbit hole. You know? Because it's-- it can be a little misleading. Now, that said, in the brain, I've had many patients who presented with acute deterioration and then we found blood clots in the major draining veins of the brain, the so-called draining "sinuses", the transverse sinus, the superior sagittal sinus, the straight sinus, and so on. They're blood clots and they can cause acute, you know, alteration of behavior, memory. Or, a patient can become comatose. And if you had significant and permanent stenosis of the jugular veins, that could also cause major problems in the brain. And it could cause increase in venous pressure in the brain. And that increased venous pressure will then cause intracranial increased pressure-- otherwise known as intracranial hypertension, or pseudotumor cerebri, where it seems like there's a tumor in the brain because there's pressure. However, if you have jugular venous compression, blood will always find another way of getting out and over time, the patient will develop collaterals. So, all the other veins in the head will increase and blood will drain out of those over time. And the jugular veins will become less important. And there's a time thing here. It's a very-- it's a very complicated-- [Kaitlyn] Yeah [laughs] Definitely. Vascular neurosurgery is kinda crazy. So, I'll go back to fusion for a second. There have been a couple of questions that came in about tethered cord and fusion. So, if someone needs-- oh, I'm sorry! Decompression/fusion or tethered cord and fusion. This kinda came up last time as well. Is there an appropriate order in which those things happen? One of these patients had their tethered cord released and realized that the neck pain-- I'm sorry the neck instability got worse, subjectively. So, is there a specific order in which this should really happen? [Dr. Henderson] Well. A general rule for neurosurgeons is you start at the top and go down. So, all things being equal, you do the craniocervical instability, first. And that's my preferred strategy. And because, once in a while, the tethered cord symptoms will get better once you fix the neck. Now, in some patients, the craniocervical instability is moderate, but their tethered cord symptoms are really severe. And in those patients, it would seem logical to do the tethered cord, first. And in some cases, they may say, "Hey, my headache actually got better after we untethered the cord." And so, even though they may have some craniocervical instability, we might hold off on that operation. So, you do the worst problem, first. But other things-- all things being equal, you do the neck, first, and then the tethered cord. [Kaitlyn] And so, in the case of a decompression, that's higher, so you would do that first? Or, no? [Dr. Henderson] Right. Yes. Well, a decompression of the Chiari, you mean? [Kaitlyn] Sorry! Yeah, yeah. [Dr. Henderson] Yeah, right. [Kaitlyn] Um, there's a question kind of specific about duraplasty and what kind of tissue you use to do that duraplasty and why you came to that decision? [Dr. Henderson] Okay, well. You can get fascia from the back of the skull and you have to do a big, ugly incision and, you know, shave an extra four inches of hair. Um, and I don't think anyone would like that. Or, you can get the fascia from the thigh. The fascia lata. And that works very well. People don't seem to have problems with the thigh incision. And that fascia is more difficult to use, to sew in, but it does-- it grows into the dura really well. And that probably gets the best seal. Or, you can use a bovine dura, pericardium-- the heart [from a cow]. And um, and that's much easier to handle. It's much easier to handle. Much easier to sew. You don't have to worry about the thigh incision. But, sometimes the ethylene glycol that's used to preserve the dura can cause inflammation. I operated on a princess of a Middle Eastern country once and, of course, she's the one who had a chemical reaction to the-- And so, her strategy was tongue-in-cheek. When I told her this was a chemical reaction to the ethylene glycol or to the bovine dura, the next day I came in and she's saying, "I love cows, I love cows, I love cows!" [laughter] [Kaitlyn] Just reminding herself! [Dr. Henderson] And then, there's this so called [synthetic] dura, but... I think the best is the fascia lata from the thigh, but the-- the bovine works very well, too. And that's probably a little bit more standard. [Kaitlyn] Um, specific to Chiari-- Chiari 1-- so, say that's all the patient would have they have occasional headache and they're not sure if the symptoms are necessarily Chiari symptoms. Is surgery done in a case like that? Or, only when it's impairing quality-of-life? Or, should would someone have a decompressive surgery especially on a child or an adult-- I guess, either-- um, sort of prophylactically just in case the symptoms get worse? Or, do you just watch and observe? [Dr. Henderson] Yeah, I don't... we don't generally do neurosurgery prophylactically. We don't do it in anticipation of possible problems. Number one. Number two: there are so many causes of headaches. Like, if you have EDS, you can have a headache from mast cell activation syndrome, from temporomandibular joint problems, from instability at C2,3 or C1,2 or C2,3 or C3,4 or even C4,5. Tethered cord syndrome. Headaches from TIAs. A hole in the heart. From abnormal vasculature From... [Kaitlyn] The list goes on. [Dr. Henderson] Yeah. There's a legion of things. If you're going to operate for Chiari, there ought to be typical symptoms and a headache that's significant. You know, at least a 6 out of 10, preferably 7 or more out of ten. And it's interfering with what they need to do. If they can just take an ibuprofen and that's it, then probably not worth having the surgery. But if they can't continue the work that they have to do, then it’s really interfering with the progress of their life. And if they have both the symptoms and some neurological findings, congruent findings, then that's--- We always want to see the pain-- pain and disability. Symptoms, findings, congruent radiological findings, if they all go together, then that's when we operate. [Kaitlyn] Okay. This is more of a spinal question. Is it common for scoliosis to start developing later in the teenage years, even if it was never present before? [Dr. Henderson] Right, yeah. Well, tethered cord can cause scoliosis to develop in teenage years, or even later. Could you excuse me for one second? [Kaitlyn] No problem! We'll wait a minute. [silence] And just so everyone knows, there are a ton of questions and I'm trying to get to all of them. I really do promise! [laughs] I will say-- some of these questions, Dr. Henderson does go over in his most recent presentation-- the Q&A-- so I recommend that you-- if you're bored after this, you can go on to our YouTube. It's there. And you can take a look at that presentation, as well. [mumbling] Ooh! The YouTube channel name! That, I can do. So, you go to YouTube.com, slash... it's "CSFinfo2007". So, I'll put it in the chat. https://youtube.com/csfinfo2007 So, that's our YouTube channel... And we have all of our videos, going back to, I think, 2009 on there. So, there's literally, I want to say days worth of videos to watch if you're bored and, still, some of us are quarantined. So, if you're looking for something to do. You can definitely go in there and take a look. Hello, Dr. Henderson! Alright, um.. Oh! This is a good question: Do you think that a tethered cord release following a decompression and duraplasty would have a positive effect on a very large syrinx that continued to persist after that decompression? [Dr. Henderson] No. [Kaitlyn] No? [laughs] Okay. [Dr. Henderson] I think it can affect syrinx in the lower thoracic spine, but persistence of a syrinx in the cervical spine, to me, and to others like [inaudible] suggests ongoing instability or basilar-- at the craniocervical junction, or basilar invagination, or a a severe kyphosis of the brainstem. [Kaitlyn] Okay. This is an interesting question, as well. Can numbing in the legs be caused by a Tarlov cyst? The patient isn't able to find a physician that's comfortable to them, do you have any recommendations? [Dr. Henderson] Tarlov cysts usually cause pain in-- it's a little bit more dermatomal. Although, it's quite possible that, over time, the autonomic nerves can become involved and the pain may be non-dermatomal. But usually, a Tarlov cyst, if it's on the S2 nerve, then it will be pain in the back of the thigh. There might be pain on the front of the leg. Secondly, Tarlov cyst is almost always-- they're best lying down and worst sitting up. When you're sitting up, there's a higher-- a greater column of pressure, water pressure, in the spinal column pushing down, increasing the pressure on the Tarlov cyst, causing more pain. So, that's... They're very specific. And Tarlov cysts usually cause urinary symptoms or often cause urinary symptoms, although, I'm not sure exactly why. And um, but they do not cause weakness. Of course, they may cause numbness. The lower sacral dermatomes, especially S2,3,4. But they do not cause weakness. [Kaitlyn] Okay. There's an interesting question. This is an older patient, about sixty-six. They had a really rough time, earlier on with Chiari, scoliosis, syringomyelia. Um, she really has, like, a comment and was curious to hear your thoughts on it. She-- I said she like I know it's a woman, I have no idea-- "I went vegan a few years ago, and the pain seems to have disappeared." And then recently, they had undergone more facet locking-- joint locking situations and had some steroid injections but she was-- she or he-- was wondering if they should be seeing a physiatrist or, what do you think in that kind of situation? And if the-- diet had changed some pain symptomatology? [Dr. Henderson] Okay, I'm a little confused here. So, They've got a syringomyelia? [Kaitlyn] Chiari, scoliosis, syringomyelia, they'd undergone shunting and a lot of different surgeries. What-- the patient wound up doing some walking, and doing some exercise, went vegan-- changed the diet. Is there anything else, I guess, that you would recommend to fix some more joint locking situations that are going on? [Dr. Henderson] Yeah, well.. See, here.. A lot of the things you mention occur with Ehlers-Danlos syndrome. Either they're comorbid conditions that are happening at the same time... If you have-- [pause] mast cell activation syndrome that can be affecting all of your joints. Bladder function, brain fog, pain level, and everything. And so, altering the diet and activities may be improving those comorbid conditions. They're the real Joker in the pack. You have to identify all of the problems and not simply assume that the problem is due to the Chiari, or the syrinx, or craniocervical instability. You have to look for all the other issues. The nonsurgical treatments are really important. You know, diet… Sometimes people have allergic symptoms to the air, to the water. Mold in the house. These are gonna have a huge, huge impact on the patient. That's one good thing about osteopathic doctors. They're little more thoughtful about all the other things, whereas medical doctors are thinking about you know, what drug can I give this patient to get them out of my office? [laughs] Or, what test can I order so they'll leave and get the test and leave me alone, at least, for a moment? Whereas osteopaths, you know, they think about these things. [Kaitlyn] I'm gonna try and take two more. So, one of them is: are there any suggestions that you might have, surgically or non-surgically, in treating a teenager that has some dystonia localized in the neck, with head and shoulder tremor? The patient also has Chiari and is hypermobile. And unstable in some cases. [Dr. Henderson] Yeah, um, yeah, these... tremors are-- that's kind of a very non-specific term. And I saw someone today remotely. She had-- if she just touched her leg, it would start tremoring. And that was, I think, spasticity. You know, increased tone. That poor girl walked into see doctors in Florida and said, "Hey, I think I've got craniocervical instability and Ehlers-Danlos syndrome." And they told her to go see a psychiatrist. You know? And that's exactly what she had. And she had all the neurological findings, and everything to go with it. Dystonia-- dystonia is another very nonspecific term and there are a hundred causes of dystonia. But, instability at the craniocervical-- you know, instability in the neck-- or something that's irritating the nerves there of the neck or the brainstem can, I think, cause dystonia. So, the dystonia could be coming from up in the brain. It could be a seizure. Or, it could be some irritation of the nerve at the cervical level. And so, I'd be inclined to treat that with a-- see if a neck brace helps. And [medications like some benzodiazepines. And some] anti-pollenergic drugs. There's a whole host of them [that may] treat dystonias. But they can be quite difficult to deal with. And you know, there's something called "torticollis", which is where the neck is literally-- the muscles become contracted and and the neck is completely bent over. And these can be very difficult to deal with. So, they're tough, tough problems. [Kaitlyn] I-- we're getting so close to it being very late. So, I'm just going to ask one last question. I know we had extras, but I promise next time! So, I think it's a good one to end on, though because it's really about thinking about how we look at this systemically. [Dr. Henderson] I don't want any political questions! [Kaitlyn] [laughs] It's only a tiny political. [Dr. Henderson] No systemic-- [laughs] [Kaitlyn] How do we best approach our local health providers who are not up-to-date on certain-- Chiari, CCI, or EDS situations? And really, why is this information not making it to certain neurologists? Because they're really-- and GPs, really-- they're the first line of defense for these problems. So, how do we get out there more? [Dr. Henderson] Um, I won't say ignorance, arrogance, stupidity. [laughs] Um, the But, I mean... You know, we didn't learn a lot about it in medical school. Probably the [clears throat] the best thing is to-- you don't want to offend your medical provider-- But if you can come in and say, "Hey, I did a little research and I have to say, this fits my symptoms exactly, and I brought you an article on this." And if they're a good doctor, they'll say, "Oh, okay, I'll read that." And they'll be interested in learning about it. But, if you approach them well, and they're not willing to learn something about it, and they just-- then, find another doctor. I think doctors have this sense that they learned everything in medical school and there's nothing much more to learn. [Kaitlyn] Yeah, I think it's the cognitive dissonance that-- how much we really don't know about the body. Especially the brain and the central nervous system. I think it's just they don't want to deal. [Dr. Henderson] Yeah, I mean... the reality is, most doctors their knowledge is... two questions deep. Say, "Well, why is this?" They'll give you an answer. Say, "why is that?" They might give you an answer. Then, “and why is that?" And you've already hit the depth of their knowledge. [Kaitlyn] Yeah. [Dr. Henderson] But um, something like Ehlers-Danlos syndrome-- I didn't diagnose my first patient until 2010. And someone in my office-- one of the nurses in my office. I know, in retrospect, I've been dealing with these patients at Georgetown and I was intrigued by them, but I didn't know-- I couldn't put it all together. But in this case, I had a nurse who was a champion swimmer. And she came to me with a back problem. And while I was examining her, you know, for the back, I noticed a bruise on her thigh and I said, "What's that from?" She said, "Oh, I bruise easily." Then I noticed a scar on her leg. And she said, "Oh, well I had a knee problem." And I thought, Why would a champion swimmer have a knee problem? You know? It must be a joint thing... And then I thought, joint, bruising... and I thought, This sounds like one of those connective tissue disorders. And I thought-- "Ehlers- something or other" And I looked it up. And then I went and I went to meet Clair Francomano who is an expert on this and she said, "Hey, can I send you patients?" And I said sure! So, that's the first time I'd made a diagnosis. And it was just remembering a five-minute portion of the lecture, you know, 25 years before. [Kaitlyn] Yeah. [Dr. Henderson] And so, most doctors what they see is more circumscribed than what I see. And they're less willing to expand their expand their portfolio... [Kaitlyn] Well, that story is super inspiring, I think, to everybody on the phone because-- all it took was one patient and look where you've come-- what is this? What year is it? 2020-- Ten years later! [laughs] So, all it takes is one patient to come in and say, "I need help." [Dr. Henderson] Yeah. [Kaitlyn] Alright! Well, that was great! [Dr. Henderson] Kaitlyn, thank you for organizing all of this. And, Dorothy. You all-- you all have done so much for the knowledge factor. You all are the hinges of this whole thing. We would still be in the dark ages, if you will. And you too, Cathy! [Kaitlyn] You too! [Dr. Henderson] Alrighty. Thank you. Alright. Bye bye, everybody.

Revised: 7/2020