Chapters Transcript Video POTS and Spinal CSF Leaks Laurence Kinsella, M.D., F.A.A.N., presents at the Johns Hopkins POTS Grand Rounds on July 1, 2021. Alright well hi everyone, my name is Lawrence kinsella. I'm a neurologist in ST louis, I'm the chair of the department of neurology for S. S. M. Health which is a five hospital system here in ST louis. And um you know a number of years ago when I first started practice, I was in Cleveland at Mount Sinai Medical Center in case Western Reserve. And there we had my colleague Tom Kalinsky who is a great colleague to have to refer patients to with autonomic disorders. And I learned a lot from Tom and relied on him very heavily. But ultimately um I left Cleveland and came here to ST louis and I didn't have a tom Collins key to refer to anymore. So um I was able to find a old abandoned tilt table in the basement of our hospital and the physical therapist used to use tilt tables but they eventually gave up on it because guess what? They make people faint. So there was this abandoned one down in the basement and I dusted off an old dinah map from the ICU. Um that allowed me to take blood pressures every minute or so and heart rates continuously. And then I had my MG machinima neuro muscular specialist by training and was able to do heart rate, deep breathing and sympathetic skin response and so after a while I could just add water. And I had an instant autonomic lab. And so um 25 years later I've been doing autonomic medicine, I was the 12 person to get my certificate with the academy for uh for board certification autonomic medicine We desperately need more people to take their boards and hang a shingle and offer this service because as you all know, these patients don't have many places to go. And so the other thing I've gotten into is watercolor and I started this when my, my eldest daughter, I was four and I wanted to have something that she and I could do together. So we picked up watercolor together and we do it at the beach and other things. But ultimately she grew up and outgrew me and I kept it up. So this is an example of some stuff I'm doing and what I'm trying to get here is a, the illusion of detail and you see just little tiny squares of bricks giving you the illusion that the whole thing is brick and in the background, just a few um strokes of pencil with a wash. Just to give you the sense that maybe there in the mist is a forest in the middle of the winter there. So this has been kind of my fun evocation and let's go ahead and get started. So I'm gonna need to change this just for a moment. There we go. Okay, okay, Get this up and out of the way of my slide. So this is an 18 year old with severe chronic daily headaches seen. Um in July of 2018 she'd had lifelong headache. She stated to me, I took talent all as a baby in the last two years has developed a chronic daily headache. Postural business near syncope E dysplasia, stiff neck anxiety and depression. Was initially referred to me as a possible pots patient. She has to headache types. One is a lifelong migraine which is 1 to 2 times per week. Occipital throbbing, squeezing pain with no phobia. And then in the last two years she's had this new daily postural headache Which is a seven out of 10 and severity in the cervical region and occipital shooting to the temples and the eyes occurs within 30 minutes of standing and she can get 50% relief within 30 minutes laying down. She's never tried rescue or preventative for migraine. She has earlier Danlos syndrome scoliosis and in 2016 phone investigation was found to have lowest Dietz syndrome type two And she had a history of the thoracic ceramics in 2017 On exam, she's a tall, thin hyper mobile 18 year old woman. She has lived a scoliosis and has postural tachycardia greater than 30 beats per minute supine to standing without a corresponding hypertension. So this would meet the criteria for pots and she was certainly quite dizzy and lightheaded upon standing. She had normal neurological examination with the exception of hypermobility with the bait and score of seven out of nine and herc winky maneuver reduces headache and those of you who may not be familiar with the quickie maneuver. This is a very gentle compression on the um external jugglers and this will raise intracranial pressure by causing venous stasis. And so it is you know, not a terribly reliable test but may be helpful in patients whom you may suspect have a low pressure headache syndrome if they find that their headaches are better with the quickie maneuver. Um and it doesn't take long to elicit maybe 10 seconds. Um And um then you may be may be dealing with the low pressure headache syndrome consequently or inversely I should say. Um patients who get a worsening headache when you do this may have a high pressure headache syndrome. But and then but then there's a lot of people who just don't like it when I do this. So it's not a terribly reliable test but it may give you some indication as to the path of physiology. So my preliminary diagnosis was that this was a mixed headache disorder with migraine and possible spontaneous intracranial hypertension. Given the fact that she had very prominent postural uh component to her headache. She has E. Ds. Type four which is the lowest deed syndrome and pots. So my plan was to start propranolol at a very low dose almost homeopathic doses of 10 mg twice a day for her migraine as well as her pots. I like low dose propranolol because it allows me to kill three birds with one stone. It allows me number one to control the palpitations To block Migraine which is present in at least 80% of my patients with pots. And it also functions as an anti anxiety medication because it literally turns off the fight or flight mechanism. So this this um this agent at very low doses has been found to be very effective for managing as a first line therapy in patients with pots. I shouldn't say first line first line drug therapy. Obviously my first line therapy is going to be volume expansion with three liters of water a day. Four teaspoons of salt Today um compression device is my new favorite compression devices, the Houma extra thick compression garment which goes from the ankle up to the abdomen and it only cost $20 on amazon home. A. H. O. M. M. A. Extra thick compression garments. So that's been a it's a little tough in this hot summers here in ST louis. But since most of my patients spend their time indoors and air conditioning I can usually get people to wear this. And it's also a somewhat fashionable garments so they'll wear it. They can spend a lot more if they wish for the lululemon yoga pants. Um Those are much more fashionable and they will to give you compression. But the home is our I think just as good. So the other thing I recommended she noted immediate reduction or palpitations and lightheadedness. Uh Not so much the headache. Um So we proceeded with M. R. Maya lager afi with Moby views. I'll talk about what that means an M. R. I. The brain with contrast. And then we would offer her a blood patch if a CSF leak was supported by our imaging. So this was her MRI contrast of the brain showed minimal to Chancellor migration and pituitary enhancement. No men in general enhancement. So I'm assuming you can see my pointer here, I'm pointing here to the cerebellum tonsils. You see this black line here, This is the posterior bone of the frame and magnum. And so we're just right here at the aperture so not a significant um to chancellor migration but it's certainly right there at the foramen magnum. We don't see any other signs of of brain sagging, such as a narrowing of the Tamil opon tien ah angle here, the length there. If that gets shortened that is an indication of brain sagging. If we see loss of the pre pontin cistern here, that's an indication of brain sagging. If we see enlargement and enhancement of the pituitary which we actually do see in this case. Um And although we see quite prominent sagittal sinus enhancement, we don't see it on the axle or criminal views. What we're looking for is is pacman and gel enhancement and we were seeing leapt Ohman and deal a little bit of enhancement here but not the pac man in jail meaning the thick dura lighting up because of the venus facilitation which occurs in patients with spontaneous intracranial hypotension by the Monroe kelly doctrine. If we lose spinal fluid we have to make up that volume with something else. And the only other two things that can make up volume is the is blood thereby leading leading to engorged mint of the of the meninges, thereby causing them to light up and giving sort of a pseudo meningitis appearance. The other thing that can swell though is the brain. And we have seen patients present with coma or frontal temporal dementia a rapidly progressive dimension. Somebody whose brain is literally swelling to make up for the loss of space left by the absence spinal fluid. So her memory in spine and M. R. Milligram were read as normal. She was treated with a blind lumbar epidural blood patch. She developed bilateral leg numbness. After 10 ccs of blood was injected consistent with aquatic aquatic compression which thankfully was spontaneously resolved. She did note a 1-2 week improvement in the postural headache. So based on that um I thought it would be good to refer her to my colleague water shaving. I once tried to call him water shaving and he corrected me that it's like outer space. So water shaving at cedar Sinai in L. A. Who performed a blood patch with fiber and glue at the T. Six diverticular um which on retrospect even on RMR milligram which was read as negative. It does show a large cyst here at the right T. Six with CSF leakage here into the para spinal tissues. Here is the the longitudinal view here is the actual view here is the spinal cord and then here is the cyst right here with some extra visitation of CSF into the para spinal. And this is very common. I'm always going over my own films because this isn't something that our radiologists are are used to looking for. Now. My radiologists are quite adapted at finding these but early on. Not so much so she noted improvement for almost a year and then her postural headache rickard. So in January 2021, she was returned to see dr shaving and he found this very large CSF venus fischelis. So here it is. This is the the the dura. And he's this large patch list cyst. And then what he does here is he lifts it up with the instrument and you can see this draining vein coming off this diverticular um So this was the large diverticular um that we saw on the EMR milligram here. And then on exploration. He's able to find this and then litigate it. And he was able to demonstrate that she actually has this persistent draining vein and that's why her response was temporary and she had a dramatic reduction in headache. She did develop a rebound, high pressure headache. And I suspect about one in five patients who has a blood patch gets a rebound high pressure headache and kind of a response. You know, they're so used to producing so much more CSF that suddenly you've now altered the hydraulics that this patient has developed a home a static space with. And then once you change those hydrostatic pressures that patients can get a rebound high pressure headache. And they may temporarily have to be treated with DMX in a paradoxical way for a couple of weeks typically and uh and then they can stop taking it. So when your patient says, well doctor I no longer have my headache standing up, I'm only getting it lying down. That's when you want to think about. This might be a high pressure headache after a spinal um epidural blood patch. And the good news was that she was able to resume full time work. I mean fully recovered. So that is sort of the dramatic uh this is when your patient writes you that google review that said the doctor gave me my life back and that's really what we all hope for in because as you know, this is a terribly disabling disorder that we're dealing with. So CSF anus fistulas have been recently recognized cause of spinal CSF leaks. They're difficult to demonstrate without digital subtraction. Biological fee. These are dilated nerve root sheets are a marker of potential fistulas and most of us will have some evidence of nerve sheath neural sheath debilitation. I'll show you some pictures of milligrams, m r milligrams that show that along the nerve roots as they exit. We always, most people you'll see some um narrowings little dill irritations but when they get large like this and this was another patient is a 60 year old woman. So very active. She and her husband were out on their bikes and she fell hit her head thankfully had a helmet on and she developed cognitive function and was found to have subdural hematomas thankfully the neurosurgeons didn't operate on her cr subdural hematomas and referred her. And I recognized that this was actually um spontaneous intracranial hypertension from her traumatic fall. And she I sent her as well too doctor shaving after on M. R. Milligram. We identified very large dilated. Um I particularly like this and um she responded temporarily like our other patient to blood patching. But after the third blood patch I sent her to dr Shiva and he did this um digital subtraction milligrams. You're able to document this draining vein. So this is a CSF venous fistula. These have only been described in about the last five years. So this is a fairly new thing on the radar and so it's something that we should all become familiar with that. This is increasingly being identified as a cause of of spinal fluid leak and these can be successfully litigated with complete relief of headache. Other things that can cause CSF leaks. Well. We've all seen these before. These are disc osteo fights and these can actually be like a dagger that splits the fibers of the dura and causes a ventral leaks spinal fluid which gathers ventral to the to the um to the spinal cord. We can also see them door slowly. Sometimes there can be a dorsal osteopath fight. Um And as I mentioned we see nerve sheath diverticular with or without a venus fistula. So you know, with E. D. S. Earlier Danlos syndrome in my series as well as in many others, their documentation that that earlier Danlos syndrome is far higher. Representative patients with spinal fluid leaks and you know there's a sagging, the collagen is loose and um you know very often any of you have tried looking for spinal fluid leaks, they're very difficult to find. And I wonder, you know whether or not patients always have a leak when they have uh sagging brain and postural headache. Um Philip Lowe and dr Mochrie from Mayo Clinic published an article looking at four patients. Postural headache is frequently reported by our patients with pots. However, in those four patients, they did not find that doing a blood patch or further investigation, they were not able to find a leak. Um And two other patients who they gave a blood patch to. They didn't find any improvements. So based on the fact that there are two opinion leaders in the field of of pots. When they said there's no link. You know pretty much everybody toss that aside. But I just began to see a number of these patients who thought we should re investigate the possibility of a leak link. No pun intended no link between this postural headache that our patients with pilots often complain of and whether or not is it a leak or maybe there's just so much compliance of the CSF that they get a turkey baster effect, that the CSF just hangs down in this area and thereby causing a drop in the null point from the frame and magnum down somewhere between the shoulders. And many of our patients will complain of what's called the coat hanger headache where it's not just a headache, it's in the back of the head, the neck and the shoulders and that hypo that's been ascribed to hypo uh a lack of profusion, hypo perfusion of the para spinal muscles and shoulder muscles. But I also wonder whether there may be a spinal fluid leak on relative leak. A drop in the um null 0.0 point of where the pressure is zero uh, down in between the shoulder blades. So this is what a epidural blood patch does. Here's the fecal sac. Here's the dura and the blood patches in the epidural space. We inject 20 to 30 CCs of blood and um even if the leak is not at that location, These are often done as we we call blind lumbar epidural blood patch. It's blind, meaning we don't know where the leak is. It's lumbar and very often in fact as high as 50% of the time in some series 11 of these will be enough to turn off the headache for on a permanent basis for patients. Even if we don't know where the leak is. The here is a actual epidural blood patch in place and you can see this is this line, here is the dura and here is the blood. And so that's about 20 CC's of blood which is placed in our interventional anaesthesiologist, pain management anaesthesiologist does this for us, michael boda feld and he always checks and confirms that the the the epidural blood is in place. So patients with the D. S. Have this phenomenon called cerebral ketosis. Where um in addition to patients with the DS have have a symptom snowball, it's not just that their joints are loose and they're hyper mobile. They also have chronic abdominal pain because of viscera apoptosis. The viscera actually under the influence of gravity just kind of sag. And the same is probably true of the brain and the spinal fluid that because of the increased compliance in these patients that CSF may just simply hang down in the epidural space. That's a that is a a hypothesis. Waiting for proof. So spontaneous intracranial hypertension. This is an Ortho static headache plus the presence of at least one of the following a low opening spinal fluid pressure of 60 millimeters or less sustained improvement of symptoms after epidural blood patching demonstration of an active spinal CSF leak or cranial M. R. I. Imaging of intracranial hypertension using the pneumonic seeps, which I'll go through shortly. And no recent history of the dural puncture and not attributable attributable to another disorder. And in my experience I have at least four or five patients who presented not with postural headache but postural dizziness with or without completing all of the criteria for pots. And as many of you know, who see patients with pots very often they don't have Postural Tachycardia in your office and you can't demonstrate it. But they sure have chronic Ortho static intolerance. And I had a young, 40 year old woman. She was a bodybuilder came, she crawled into my office on on all fours because she had such severe postural dizziness. Do you have a headache? No, I don't really have much headache. It's just I cannot stand without getting severe. And it was sudden in onset. And um it happened shortly after doing some military presses. And because of that story, we went looking for a leak and she in fact had a large and diverticular um on the right T. Six as I recall. And she responded Beautifully up to 14 months at a time to a blind lumbar epidural blood patch. And she's now on her third patch and continues to do well. So that is sort of one of the overlying themes of this talk is um if you're seeing patients with early Danlos syndrome joint hypermobility. Um think about this as a potential contributor to patients um headaches. So here's that pneumonic. I promised you it's called seeps and it refers to things to look for on imaging, particularly the M. R. I. The brain with contrast. To try and identify whether or not this patient has evidence of intracranial hypertension. So the ss subdural fluid collections would be up here. We don't have that in this particular patient enhancement enhancement of the meninges. I mentioned that this is packing men and deal so it looks like a thick rind of an orange that goes all the way around engorged mint of venous sinuses. So you may see some encouragement of the venus sinuses and in fact this can be so severe that patients can develop a secondary um cortical venus thrombosis and then you wind up getting distracted thinking that the primary process is a hyper co available state. When in fact the primary process may actually be a CSF leak, pituitary hyperthermia. They get enlargement and a hyper um Enhancement of the pituitary and then features of sagging brain hears that downward herniation of the cerebellum tonsils. Remember the black line represents the border of the frame and magnum and you can see this is down at least six. And you also see some obliteration and flattening of the ponds, you see that that mammal. Oh ponting length is quite narrowed. So and you see some reduction of the pre ponting cisterns. So this patient has many features that lead us to consider this a evidence of sagging brain conditions that occasionally coexist with spontaneous intracranial hypertension. I mentioned cerebral venous thrombosis. I also mentioned a frontal temporal dementia. You should consider this in any patient with a rapidly progressive dementia. This is on our list now is spontaneous intracranial hypertension, pituitary enlargement or apoplexy. So you can get compensatory enlargement and congestion of the hypotheses veins. This can even go on to Presenting as apoplexy something we haven't seen as a modern day 21st century neurologist. For the most part I have yet to see my first case or maybe I missed it. But this is more of 1/19 century disease of childbirth apoplexy. And um we should start looking for it because we're seeing more cases. This reported pots. So prolonged supine de conditioning secondary to spinal CSF leak and then superficial cirrhosis. Because of the venus traction. These patients may get micro hemorrhages and superficial cirrhosis presents with a taxi to and hearing loss because the acoustic nerves are bathed in blood and him aside Aaron that's very toxic and inflammatory and these people lose their hearing and they develop a taxi. So I mentioned that first seminal report by Mochrie and low as you know Dr. Lowe is the father of pots and in 2003. Almost 20 years ago um they poured it sort of put the nail in the coffin of considering CSF leaks and patients with pots based on their series of four patients, they didn't think that was an issue. So my colleagues and I published this at the american autonomic associated the autonomic society meeting in Clearwater florida in 2019. And we found a number of patients with the stigmata of leaks these uh these diverticulitis which formed along the nerve root sheath and um and in here as well and I'll go through this in a little bit more detail shortly. But the this is the the the study that we order, it's called an M. R. Mile a gram Moby view. And what that means is that there's no die here. Number one. This is all a inversion recovery of stir sequence that allows you to outline all the CSF spaces. And this is a lot of the mobile view allows us to see from stem to stern, right from the frame and magnum all the way to the tip of the sacrum. Now this is a Tarlov cysts. These are very common. They're generally not thought to leak or be a major source of CSF leaks but these are these mid thoracic and sometimes cervical diverticular and you can see this one here and there's a number of them. They're they're quite um you know, polymer model, they're they're they're kind of non regular and almost grape like cluster. And these we think are the the markers if you will for the possibility of the CSF league. These can occur uh you know, uh CSF hypovolemic can occur after val salva after even minimal trauma. I've seen it after just straining at stool. The risk factors are earlier Danlos and neurofibromatosis. There's increased, possibly increased lumbar epidural compliance, which I have yet to prove. And I suspect that these patients don't actually have many of them don't actually have a leak. They just have too much compliance and they get this what I call the turkey baster effect. And and as I tell patients what our job is is we're going to try and squeeze the turkey baster by putting in blood up next to your lumbar epidural uh space and squeeze the turkey baster and raise that note point from somewhere in between the shoulders, back up closer to the frame and magnum as a way to relieve headaches. So This was our 1st 50 patients, we had 49 women, one man age range 18-61. The median age was 32, 30 a 76% have a postural headache. 44% had met criteria for pots. Um 42%, almost half have evidence of of joint hypermobility, 36%. A third of them have evidence of massive activation and about 78% had some abnormalities on imaging whether it be dilated neural sheaths, diverticular para spinal fluid collections of dilated epidural veins or features of sagging brain, The response. Epidural Blood Patch was transient in 28 and more long lasting in about 34%. So 17 of of these patients that we did a blood patch on had major improvement more than two weeks. So why did I pick two weeks? Well um you know I haven't controlled here so I haven't controlled for a the possibility of a placebo effect. But if you look at the literature on placebo effects, most placebo effects degrade within about a week. And certainly by two weeks placebo effects have pretty much been eliminated. So I figured I would call this a major response if it lasts greater than two weeks with a greater than 30% reduction headache and dizziness and an improved functional capacity scale. So here is all patients at the top with chronic Ortho static intolerance. And then just looking at the subset with pots down below. You can see the percent of patients that have E. D. S. Mast cell. A large percentage of our patients with pots, if you ask them notice that in addition to the postural business, their headaches are worse standing out. And a good chunk of these folks do seem to respond to epidural blood patch. Um You know some minor less than two weeks but some of them much more prolonged. So our Lord Danlos syndrome. Many of you are familiar with this but just to back up how do we make this diagnosis? It's certainly supported by the finding of a positive bait and score. Um uh We think that at least a third of our patients with pots have joint hypermobility as demonstrated here using the bait and score. And uh so if they have four plus out of nine then that we consider that positive If their age is less than 20 they really need six or higher to call it because so many kids are hyper mobile and then if it's negative but you still suspect that I asked them what are called the Mulford questions. M. A. L. F. A. I. T. These are five questions that ask. Um Can you now or could you ever palm the floor? Could you now or could you ever do the splits? Do you consider yourself double jointed? Did you spontaneously dislocate your shoulder or your kneecap? On more than one occasion There's 1/5 question that I'm blanking on. But if they have more than two, two or more questions that are positive and the likelihood is that they in fact do have joint hypermobility. Even if your bait and score does not meet the criteria. So how do we score them? Two points for each hand For greater than 90° of flexion. Two points if they can bend their thumb and touch their forearm, two points for a hyperextending Elbow. Two points for hyperextension of the knees and one point for palming the floor with the knee straight. And that is a total of nine. So the majority of our patients with the D. S. Have the hyper mobile variant and um genetic counseling may be helpful to screen for rare disorders of type one type three type five collagen. It's important to exclude Marfan syndrome. Um These are mainly cardiac issues, defects in the microfiber ular protein gene FB. And one it's important to exclude louise Dietz syndrome. These patients often have cleft palate and aortic aneurysm a dissection. Our patient had evidence of a small the anterior cerebral artery actually an inter communicating artery aneurysm was not considered necessary to intervene on that. And our patient did actually have the G. G. Br BR two mutation. The evaluation evaluation of patients um is primarily cardiac and excluding debilitation of the aortic root, an E. K. G. Echocardiogram and ct of the chest. And then the main evaluation and treatment is getting them to a physical therapist who has some familiarity with heirloom Danlos teaching them about joint safety. Joint stabilization exercise is important to try and build muscle to support the joints in the absence of a strong ligament and tendon and then pain management. Many of these patients have chronic pain and this is one of the situations where I have found low dose naltrexone to be quite helpful. So the take home points non contrast em are my la graffiti with Moby views is more sensitive than radionuclide study or CSF opening pressure leaks Often not demonstrated the markers of possible leaks, diverticular may be seen spontaneous intracranial hypertension may occur after val salva and minimal trauma. The risk factors are E. Ds and neurofibromatosis. The presentation maybe postural dizziness more than the headache and a trial of a lumbar epidural blood patch is reasonable. It's reasonably benign. As I as you saw, it isn't always benign. I had a patient who had a transient cardiac wanna syndrome but for the most parts um this is one of the less invasive things, dangerous things that we can do to try and help our patients. So this is something to consider. Also keep in mind that when you're patient tells you they have a chiari malformation, take a look at those films. What you may be actually seeing is to consular migration from a chronic CSF leak and not an actual genetic congenital malformation. So with that I will be I will stop and I will be happy to take any questions that you may have Created by
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