 Hi there. I'm Kathleen Diggree. I'm so glad to be here. Last year we didn't do this because of COVID, but this year we have learned how to do things virtually. We're doing an update that we actually do annually about what's new in research around idiopathic intercranial hypertension. For many people, this is a new diagnosis. Some people have had this diagnosis for a long time. I'd like to review things that we all need to know, and then I want to talk about what's new, and I try to point out some of the new things that are going on. I'm going to start with this case of Sharon who's 34. She was sent to us because she had headaches and disc swelling. She had recent weight gain. Otherwise, she's been very, very healthy and doing actually very, very well. She has symptoms of dimouts of her vision, a whooshing noise in her head, and headaches that are more severe than her normal headaches. Her visual fields are not normal, and we're going to talk about visual fields because it's important to understand how we do the evaluation and workup. There are many people who get just an acuity and don't get visual fields. This is not enough to follow people with idiopathic and acrenial hypertension. I hope there are people who are support people to our patients tonight because it's important to understand all the things that patients have to go through when they have this diagnosis. When we looked in the back of her eyes, I'm going to use my arrow to point out, she had bilateral optic disc swelling and this is not normal. I'll show you what normal discs look like and then what swollen discs look like. Her nerves were swollen. We looked at her imaging, and we saw that she had what we call an empty cella, and we'll talk a little bit about that. She had extra fluid around her optic nerves, and a lumbar puncture was done, and her opening pressure was 350 millimeters of CSF. But she had normal protein, glucose, and cells. The question is what should we call this? Idiopathic endocrinial hypertension has had many names over many years. It started out with the name benign endocrinial hypertension. Well, this is not a benign disease. Then Dr. Corbett, one of my mentors, named it idiopathic endocrinial hypertension, and idiopathic means we don't know what it is. Then the group in England and Australia said, well, let's call them pseudotumor cerebri, pseudo meaning false tumor, like a brain tumor cerebri in the brain syndromes. That's stuck a little bit. When we think about it, the idiopathic form is the primary form. This is what most people have, is the form that comes out of the brain. We'll talk about what we think causes it. Then there's a whole group of things that are like mimicers or secondary causes. The reason so many tests are done are because we have to figure out is there venous sinus thrombosis or some other blood clot in the veins? Are there medical disorders that are going on like the parathyroid hormones or chronic obstructive pulmonary disease or heart failure or sleep apnea? We'll talk more about that. Iron deficiency, anemia, and then there are medications that have been reported to cause intracranial hypertension, like growth hormone in children, vitamin A, lithium, anabolic steroids, sometimes things like noreplant and marina have been associated with it and then there's some other medications as well. And we try to keep up with all the case reports of what things could trigger this high intracranial pressure. But keep in mind that most of these are idiopathic. Now this is a common disorder. It's not really common, but it's as common as multiple sclerosis in obese individuals. So everybody's heard of multiple sclerosis, but most people have never heard of idiopathic intracranial hypertension. And just remember that it's not rare in obesity. It starts about the time of puberty in both girls and boys. And it goes up to about age 60. Usually pressure settles down by then, slightly older in men and it's way more frequent in women, eight to one. So for every man, there's eight women that have this. And in the idiopathic intracranial hypertension treatment trial that I'll mention, 98% of the people enrolled in the trial were women. So it's really a woman's disease. In children, usually girls and boys are equal, but then after puberty, girls take off and have this more frequently. And there is a genetic aspect, although we don't have any genes identified. The symptoms, if you've got this disease, you know what they are. Number one, headache. And we're gonna talk a lot about headache because that's the part that disables most of the people. Transient visual obscurations are next. It's like a dim out of your vision and then it comes back up. Back pain, dizziness, light sensitivity or photophobia. Pulsatile tinnitus, like whooshing noises, like psh, psh, psh in your ears. Neck pain, visual loss, cognitive changes. Diplopia is double vision and ridiculous of it pain is pain that goes down the neck or the back and into the arms or to the legs. That's a lot of symptoms for these people that have this disease. Headache is the one that seems to be the worst. 94% of all people have headache and they can have pain in their neck and shoulders, pain with eye movements. And what's frustrating is how bad the headache is is not correlated with how high the pressure is. So it's kind of a frustrating symptom to have and I'm gonna talk a lot about that. Now, this is a study that came out just this year and they tried to ask patients who had this, which symptom bothers you the most? And of course, not surprisingly, headache. So this dark brown is the most severe symptom that bothered people the most. The next symptom that bothered people was depression and that was present in almost a half of the people who had it. And then people mentioned obesity, being a part of it that bothered them and then sleep disturbance was a small amount. And they asked a lot of other questions but these were kind of the main findings that bothered patients the most. I mentioned the whooshing noises, like this buzzing or whooshing, like a river running in your head, hearing loss, dizziness, but hearing symptoms are really common, like half the people get this. So now I'm gonna talk about what are the findings? So when you go to a provider and what do they look for? Well, the first thing they look for is visual acuity. That's how well we see, but only 13% of people, so only one in 10 about will have a change in their visual acuity, meaning that they won't be 20, 20 when we check their eye chart. It's not an adequate screening tool because that is the last thing to go. The acuity is the last thing to go. That's why we have to do what we call visual fields. Now visual fields, for those of you who have never done them, are boring. They're really boring. You put your head in a ball, you push the button when you see the light, you have to stare for a long time with both either eye, and it takes some time and it's boring. So imagine yourself having a headache and not feeling good or anything else, and you still have to do this test, and that's what we require our patients to do because that's one of the key measures that we monitor is a visual field. So up here you can see this visual field up here where I'm circling this big blind spot here on both sides of the both eyes, and that's a typical finding that we see. Now we don't like that because a field should look more like this. This is a much more normal visual field, but sometimes they're really bad, like this one has got a really bad defect, and then look at this right eye is really constricted and bad. So these are people I have to like, oh my goodness, I gotta do something right away. And we see all kinds of defects on these visual fields, but they help guide our therapy. It even predicts visual outcomes. So if you come in with a good visual field to start with, you're likely to have a good visual field throughout the course of the disease. If your visual field is bad to start with, we worry a lot because then we are worried about it. So when we get the visual field, this is a visual field with kind of a big blind spot here. We're looking at all kinds of things. We look at how many false positives, meaning how many extra clicks you did when there wasn't a light, or false negatives, meaning you saw the light, but you forgot to click it. We look at any defects. We look at the mean deviation. We have all these parameters that we look at in this test. So this test is an important test. Even though it's very boring, it's still important and we have to do it. Now the other thing we do is we dilate the back of the eye and look for papillodema. Now what are we doing going into the back of your eye? Well, we go through the cornea, we go through the cornea and the lens, and we go all the way to the back of the eye. So in this cartoon, I have to look all the way through the cornea, the lens, and I'm looking at this structure right back here called the optic disc. And the optic disc is the thing that swells. It is connected to the brain. And when there is increased pressure, that pressure goes along this optic nerve and pushes on this optic disc, and it creates papillodema, okay? And that's what we're looking for. And now some people have very, very little or no papillodema, like this person is pretty normal, okay? I can look all the way around that optic nerve and it looks just great. It's not swollen. But now look at this person. They have kind of a C-shaped swelling. There's some folds in the retina. And these people get like, you know, like wiggly, jiggly, like letters look kind of distorted. So this is not good. And then the swelling can get worse so it's all the way around the nerve. And then look at this one. It's all the way along and there's some hemorrhages and the veins get kind of big. And this is what I'm looking at. I'm looking at papillodema, okay? And that papillodema is this swelling of the optic disc and it's due to the increased intracranial pressure. Now, so we see somebody, we do the acuity, we do the visual fields. We look in the back of the eyes and we are very careful about what we're doing. But then we need to do imaging. And we do imaging because we have to make sure there's no brain tumor, that there's no venous sinus thrombosis, that there's nothing obstructing the flow to the brain. And that's really important, okay? Because if there's obstruction, then it's gonna give you a headache and it's going to cause papillodema and all these symptoms. So that's why imaging is critical. You cannot make the diagnosis without imaging. And many times not only do we look at the images, but we get a magnetic resonant phenogram and I'll tell you about why we do that later. Now, what do I look for when I see the MR scan? Well, in this person, you can almost see the swelling going into the eyeball. See, this is the eyeball and here's a dilated nurse sheath. And that extra fluid is like pushing on that little poor optic nerve causing it to swell. And I'm seeing all this extra fluid. Sometimes it pushes the pituitary down and causes what we call an empty cellar. And an empty cellar is where the pituitary sat, but the pressure is so bad that it's pushing the pituitary gland to the side. Now, there are normal people that have empty cellars. So it's not 100% a sign of high pressure, but it's something I always am looking for. Then we always do a lumbar puncture for those of you who don't have this disease. This is another very unpleasant thing to do. You get put on your side, you get measured with this manometer and then we take the fluid out and then we measure how high the pressure goes and we look for pressures that are over usually 250 millimeters. Some people use centimeters. So then it would be like 25 centimeters or 250 millimeters. Sometimes the lumbar puncture can help the headache and the whooshing noises, but sometimes it doesn't do anything. In fact, it can even give people a headache. We're always looking for sleep apnea because there are people who have increased intercranial pressure due to sleep apnea. And if we fix the sleep apnea, then we can fix the increased intercranial pressure. And Lisa Ord put out in our handouts the Berlin questionnaire. It's a very simple questionnaire. And if you've got a lot of positives in this questionnaire, you should talk to your doctor about sleep apnea because if you have sleep apnea, you're at high risk to have increased intercranial pressure. And sometimes you have to do sleep tests. Another symptom that people often complain about is cognitive changes that some people can't think. Their brain is fuzzy. And I'm going to talk a little bit more about this. There have been many studies that have showed slow reaction time, processing speed problems, and thinking can be slowed. And the cognition can be slower and, but it can be related to multiple other things like how high the pressure is, how bad the headache is, if that person has sleep apnea, and executive functioning. Executive functioning is kind of like, how do you plan your day? How are you productive? So a lot of people say, I'm just, I feel like a blob. I can't really get anything done. Now this graphic came out this year and I said, I'm going to put this in this talk because they did a study with 66 women with idiopathic intercranial hypertension. The gender and age matched and body mass index matched and we're talking about body mass index, weight matched the people. And then they did a bunch of cognition tests like attention, working memory, executive tests, verbal, short-term memory. And what they found was the attention and executive function, the sustained attention and being responsible for tasks was impaired in this disorder. And then what they did, if it was acute, if the person was just diagnosed, they repeated the test after the lumbar puncture and they were better. And then as time went on, they got a little bit better after 12 months, even though they still had the disease, it was a little bit better. And they found that the things that affected the cognition were how bad the headache was. If a person had comorbid depression, which again, remember I said depression was the second most common thing that patients who have this disorder are concerned about. Sleep apnea, a lot had sleep apnea, how long they had the disease. And then they had some blood tests that showed some abnormalities. But a lot of this got better when the person was treated. And you can imagine if you have impaired attention when you're being asked to do a visual field, for example, how hard that's got to be. And it can lead to poor performance. And that can actually give us falsely abnormal visual field. So we have to be really careful about the cognitive changes. Now the question I get asked a lot is why does this occur? What in the world is this about? So I'm going to talk about genetics, the venous hypothesis, glymphatics and lymphatic drainage, obesity. I'm not going to talk about vitamin A, but we do know that people who ingest a lot of vitamin A, like if you eat a lot of polar bear liver that has vitamin A in it, you can get increased intracranial pressure. So don't eat polar bear liver. But, and I'm being a little bit facetious, but we don't recommend high vitamin A dosages for people with this disease. So what's the genetics? Well, we definitely know that there is a genetic predisposition because we all see people who are like a mother, son or mother, daughter, sisters, brothers. We've had twins, one was symptomatic, one was asymptomatic. We definitely need more data. And at the Moran Eye Center, we actually have a genetic study going on. So if you have anybody in your family that has this disorder, we want you to participate in our studies. It's not a big deal. You can spit in a cup and we can get the genetic material, but we're looking to see, because if we could figure out the gene, we could figure out how to cure this disease. So if you have that in your family, please let us know. You can let Lisa or know or let me know and we'll get in touch with you. Now, in order to go on, I have to give you a few facts about spinal fluid, where it's made, how it's made, and where it's absorbed, because otherwise none of the rest of what I'm gonna talk about is gonna make sense to you. So the first point is spinal fluid is made in the chloride plexus. And this is like a bunch of little grapes that sit inside our ventricles and it produces fluid. And it produces a lot of fluid, okay? This, the whole volume, like a half a liter, you know how much a liter is? It's a lot, half a liter is a lot, is reproduced every eight hours. So that's a lot of fluid that's being made and reproduced and absorbed and made and reproduced and absorbed. And we measure the pressure down here by the spinal cord, but this CSF flows all the way around and it gets picked up by these arachnoid granulations. Now, these are like little cauliflower's sitting in the middle of the veins. And the veins are what drain our blood of all the arterial, so the artery goes into the brain to give the brain a lot of oxygen and nutrients. And then the veins take all the waste products out and dump it into the veins, which goes down into the heart, which gets recirculated in our blood and then back to being an artery again, goes through the lungs and gets oxygen, et cetera. So we think that a lot of absorption of the fluid goes through these, what we call arachnoid granulation, these little cauliflower's that sit in the vein. So if we put this all together, the fluid is made in the chloride plexus, it circulates all around and then it gets absorbed in the arachnoid granulations. Now, if the pressure goes up, these veins get collapsed or stenotic or they get kind of closed together, okay? That increases the intercranial pressure and then that even further pushes on those veins. And we know that obesity, I'm gonna talk about that and fat cells participate in this. There are also inflammatory factors that participate. And then I'm gonna mention this lymphatic, lymphatic drainage that is in addition to those arachnoid granulations that picks up the fluid. So the venous hypothesis is, as the venous pressure goes up, the intercranial pressure goes up and this is true. Our venous pressure goes up, our intercranial pressure goes up. So for example, at trumpet players, people who are body builders and always doing valsalva maneuver can have increased intercranial pressure. Interestingly, individuals with obesity do have increased venous pressure. And then all this venous sinuses get compressed and as they're compressed, the pressure goes even higher, okay? And so that is the hypothesis about this. So that's the venous hypothesis. Then we know that there's lymphatic and lymphatic changes. Now, we used to ignore this, but this is like lymph drainage from the brain and it also goes into the venous sinuses. And at night, these lymphatic channels are extremely active and they dump that flow into the veins. And this is normal and that's why we need to sleep. All animals, mammals, all living things, almost all living things need to sleep. And part of it is to get rid of the waste from the day before. And we think that the waste place is this lymphatic. So it's extremely important. And there's now some evidence that maybe blockage of the lymphatics play a role also in keeping the pressure up. It could stop the egress through the arachnoid granulations and it can also stop the egress through the venous sinuses and increase the intercranial pressure. Now, I have to talk about obesity because about 80 to 90% of people with this disorder are obese. And weight loss makes it better and we're gonna talk about that. And there are all kinds of associations that come with obesity, orthostatic edema, venous sinus pressure changes, sleep apnea, polycystic ovarian syndrome, metabolic syndrome, all this stuff comes along with obesity and our disease, IIH. I'm gonna just show you this, but watch the red, which is 30 to 35%, but watch what happens. We're gonna go to 40%, these are the dark red brown and now more and I'm in 2015, 2016, 2018, 2019, 2020. So our country is becoming obese. Even Utah has 25 to 30% of our population is obese. Now, how do we even know what obesity is? And everybody talks about BMI, so body mass index. How do you compute it? Well, you put your height in and your weight in and then it can compute and you can do this and there's a calculator at this website or we have a handout that we've given you also that has a BMI chart. Normal weight is 18 to 24, 25 and overweight is 25 to almost 30, but obesity is more than 30. And I'm gonna talk about this BMI in relationship to certain treatments that I want you to be aware of. So here is how you calculate it. So you have to take your height in inches. So let's say I'm five foot two, so I'm 62 inches and I weigh up all, give or take, depending on how many clothes I've got on, maybe 120 pounds. Well, I'm in the normal weight category. Now, if I weighed 164, then I would be obese. And that's how you calculate your BMI. I go up like this and I could see that I'm in the 30 obese range. So here's my height. Here's my weight. I can calculate my BMI, but if I weighed 170 or whatever, then I'd have to calculate a higher BMI. Okay. Now, the reason obesity is really important is obesity increases the incidence of this disorder. So if you're normal weight, if you're normal weight, then you have a much lower chance of getting this. Only three per 100,000. But if you're obese, it's 20 per 100,000. And the fat distribution does differ between men and women with this disease and also can differ with the pressure. Now, we are learning that this obesity thing is also associated with other metabolic disorders. And this I think is really important because we're learning some new things about this. First, abdominal adiposity. And that means that the, and I'm trying to admit some people here, maybe Lisa couldn't do that. The abdominal obesity, which is what is in our gut and makes our stomachs a little stick out, affects our central venous pressure. So that can raise the venous pressures again. And then venous hypertension can lead to this increased intracranial pressure. But what's interesting is we're finding there's more metabolic changes than what we realized. Insulin resistance is increased. And this is really important because that makes us much more susceptible to getting this disorder, okay? So the other thing is that the inflammatory markers can also get increased as well. And so this is more than just being overweight or obese. There's metabolic changes that are going on. And I wanna use this slide, this was just recently published, that this truncal obesity, so this is the fat that lives underneath our, what we call our stomachs, our bellies. And that fat has certain characteristics. And then our fat that's in our arms and legs and all that kind of stuff, that has also got, that's our subcutaneous fat. That has different characteristics. And what we're finding is that this fat can secrete things that make us have more fat and make us more prime for weight gain, not weight loss. Even when we try to lose weight, we gain weight, even though we're trying to lose the weight. And so this is a metabolic problem. And these researchers in England and Denmark and here in the United States are really working on this metabolic problem with this because I think this may hold the key to better treatments and helping us understand why people get it. And we do know that as the BMI goes up, the pressure goes up, there is a correlation there. Our eye pressure, inner ocular pressure is not correlated by our BMI. And then another important point that just kind of came out in 2019, the kind of at the end, right before the pandemic started, so I didn't get a chance to give this information to you, is that IIH confers future vascular risk factors. And this was a big population study done in Great Britain where they looked at almost 3,000 women and they matched these women with IIH to controls that were the same weight, same age, and they found that heart failure was increased risk, heart disease was increased risk, stroke was increased risk, type two diabetes was increased risk, and hypertension increased risk. All of these are part of this metabolic syndrome of vascular disease. So it's important that we get everybody treated with IIH and we watch out for these other risk factors as people get older, very important. Now, what's the evidence that weight loss is helpful? Well, the first evidence came by Dr. Neuborg. She was an internist and she put people on a rice diet, imagine eating rice. So nine patients with high pressures and papillodema and then she put them on the diet and then she said from December 1968 to February 1969, they lost the weight and their papillodema went away and almost all lost the papillodema with this rice diet. Then Harvey Sugarman in 1995 did bariatric surgery and got profound weight loss and normalization of pressure. Other people have found this too. Alex Sinclair did this an amazing study where she put people on 425 calories. Can you imagine how little that is? I mean, that's almost like a glass of milk and a piece of toast with jam on it. So it's not a lot of calories, okay? But people stuck to the diet, they got rid of their papillodema, they decreased their necraneal pressure and even their headaches got a little bit better. Now the new treatment for weight loss is kind of bypass surgeries and they're a bazillion of these. I mean, they do a little lap band, they do these little pouches, they hook things up differently and they're using it for people who have obese, for diabetes, metabolic syndrome, sleep apnea and so on and so forth. And there's more and more of these being done. But this study just came out this year in June, again by the Birmingham, England group, they randomized patients over five years and they had over 600 people or no, they had 60 people and they randomized them to one arm was just Weight Watchers, current Weight Watcher program versus bariatric surgery. Now all of them had to have a BMI over 35. So it wasn't just like into obesity, but it was definitely obese. Then they measured the pressures before and after and they dropped 60 millimeters in the bariatric surgery program and they lost about 46 pounds. Their quality of life improved by seven points, their headache improved somewhat and it continued for two years. So these graphs just show you the current weight loss program, but then if they did the surgery, they had a percentage greater percentage of body weight loss and their intercranial pressure went down as well. They had no deaths, they had one complication and that was an obstruction. But it was an important study I think that tells us that we can get improvement in the pressure by losing weight. Now I wanna switch gears and talk about why we use acetazolamide and everybody who's got IIH has at one time been on acetazolamide. And this was because we did a big trial. Utah was part of this trial, the University of Utah was part of this trial from across the whole country, studying, putting people on dimox, which looks like this or a placebo. And then we watched what happened with the visual field and that's how we studied this. We entered 165 people, there were only four men in the group. The average age was about 29, 88%, almost 90% were obese and their BMI was almost 40, okay, average 40. And the average acetazolamide dose was 2.5 grams. Now many of you are on 500 milligrams twice a day, that's only 1,000 milligrams. So we pushed it up to 2,500 milligrams. And we followed the visual field, I've talked to you about visual fields and how to read them. Now these people could not have bad visual loss, okay? None of them had severe visual loss, they all had mild to moderate visual loss. And at the end of the trial, the people who were on acetazolamide did better than the people who were on the placebo and diet alone. And the diet was a program run in New York, a very good program for helping people lose weight. People did lose weight, in fact, a lot of people lost weight, even in the placebo arm, people lost eight pounds. But the acetazolamide and diet group lost more and the papillodema got better. And remember I told you that papillodema is different grades and many of them, the papillodema kept getting better and better over the six month period of time. Those who had the worst papillodema had the best effect in their visual fields compared to those who were on placebo. And there was only one treatment failure out of all of those patients. And six of them were in the placebo arm and only one was in the acetazolamide arm. And the quality of life got better. So we measured quality of life and the quality of life got better in all the different ways we measured it. So that was really a success. But the headache didn't improve. In this study, the headache did not improve. So that's why I've got to talk about headache kind of at the end of this. So the spinal fluid pressure did also drop significantly compared to placebo as well. And their adverse effects from the acetazolamide were not too bad. Two people had kidney stones, one had some changes in their liver test, one had some little bit of pancreatitis, but nothing was severe. Some allergic reaction, but most people tolerated the dimox acetazolamide very well. So now what we do with everybody who comes into our clinic is we put them on acetazolamide, we recommend weight loss. We do the visual field, we watch the papillodema grade and of course get the spinal fluid and all that. And we try to get that acetazolamide dose up to a level that will help get the pressure down and also help people do better. And this study went on for an extra year and found that people continued to do well. And those who were on placebo switched to acetazolamide and they also did better. Now there are some other treatments besides acetazolamide. Some people say to me, well, I just hate that drag. It makes my fingers tingle and my toes tingle and I just hate it. Makes soda pop, tastes terrible. Well, there are other drugs but they're not all studied like acetazolamide. So furosamide is also known as Lasix. That can, it's a very strong diuretic, sometimes been used. Amelioride is a, it reduces fluid production out of that chloride plexus we talked about. Octreotide inhibits growth hormone. This is just case reports. The two new drugs that are out there being studied are glucagon like peptide one receptor is a big long name but this drug is being used for diabetes and promoting weight loss. We need to study it in patients with IIH because it does reduce intracranial pressure in rats but we don't know if it works in humans. The other drug is this 11 beta hydroxy steroid inhibitor that treats obesity and metabolic syndrome. And there are studies ongoing in Europe on that. This study is being looked at very carefully because it's a potential treatment for type II diabetes, for obesity, metabolic syndrome and it's been found to actually lower the intraocular pressure but we don't know what happens with intracranial pressure and it's not available on the market at all yet. All right, now I'm going to switch gears to headache which is one of the most, yes? Before you do that, there is a question in the chat about the cognitive issues caused by acetylzolamide. She asked about the cognitive issues. Cognitive issues caused by acetylzolamide. Well, remember acetylzolamide causes some cognitive issues but IIH causes a lot of cognitive issues. So separating out which is which is a real big problem and those studies that were done on cognition were done on people that were not on acetylzolamide yet. So we know cognition is affected by the IIH itself and acetylzolamide can affect it too. So good question and thank you for asking it. And we will get to your questions, I promise you and feel free, Lisa, to interrupt me if there is a question about what we're talking about. I don't mind at all. So headaches are the most difficult part of treatment. Many people report severe headaches, 10 out of 10. And their headache squares are terrible. And what we find is that acutely, the headache is bad. And then the problem is even when we lower the pressure and we get everything looking a lot better, the headache is still the same. It becomes kind of chronic. What did we learn from the IIH TT? We found out that almost 90% of people had headache. 41% had migraines in their background and interestingly only 18% of the general population has migraines. So something's weird about this because the people who get this disease, 41% have underlying migraine too. The headache phenotype, what it sounds like, what the headache sounds like when you hear about the headache, mostly it sounds like migraine or probable migraine, sometimes tension type headache. A lot of people had medication overuse headache, which means that they took analgesics more than three days a week. And their quality of life was affected. But what was amazing, headache disability didn't correlate with the opening pressure, the BMI, how obese somebody was, whether how much weight they lost, what papillodema grade they had or what their visual field looked like. And even more frustrating, there was no difference between the pressures of those with headache and those without any headache. Remember, there's about 16% without headache and the 84% with headache. There was no difference in the pressures and those people at all, they still were elevated. So some people are more prone to headache. Treatment of headache is weight loss still because it can actually, even if you're obese and have chronic migraine, losing weight is helpful. And then having a diuretic like acetazolamide plus a headache migraine treatment is helpful. Doing a lot of lumbar punctures, the study was done on this recently, showed there was little gain and you have to watch out for medications that make weight more, but also medication overuse. So these are the diuretics that we use, acetazolamide, methazolamide, ferrosamide, chlorothaladone. These are diuretics that we can use. And then these are migraine treatments that we use. Now the new kids on the block are to pyramid has been studied and shown to lower the intercranial pressure in laboratory animals more than acetazolamide. So frequently when people have very little papillodema we will go right to to pyramid if they have very bad headaches and get them on that medication because it will not only help them lose weight, stop the headache and get the pressure down. It does all the things we want it to do. The other new kid on the block are the CJRP monoclonal antibodies. These are antibodies to a little receptor that makes headaches of migraines worse. And so once a month shot and that is the prevention for the migraine and that's been seen in a lot of patients. We've had some luck with it in our patients and it's being studied up. There were several studies more recently published. And then anabachillinum toxin or Botox has been used for individuals with a lot of chronic migraine symptoms with IIH. And so that is also a recent study that came out. Now, any questions about headache and IIH? Cause that is the bugaboo. And I wanna make sure I answer that. We did have one other question about diamox and the fatigue that it makes it really hard to exercise with so much fatigue. All right, and that is true and fatigue accompanies IIH as well. And yes, it makes it hard to exercise. And just even a little bit of movement. The best exercise I have found for people is swimming. Number one, you don't have to fight it. You can just float around. Just being in water is great because you have to keep a little calories burning to keep yourself warm. And you don't even have to move hardly to burn some calories. So it really, swimming is a, and most of my patients that do swim find that that helps the most it's less fatiguing than walking and running and all that kind of stuff. Good question. Any other on the headache side of things or acetazolamide side of things? Doesn't look like it. Okay, now I wanna talk about visual loss which is the thing we work out. You can't treat visual loss with lumbar punctures. You can't treat visual loss with gastric bypass. It's not a treatment for visual loss. They used to take off part of the skull on people with IIH, we don't do that anymore. So really there are three treatments for visual loss. One is optic nerve sheath decompression. I'll go through each of these either unilateral or bilateral. The second treatment is shunts and they do have adjusted adjustable valves that are gaining popularity. And the third one is venous stents where they put a stent in the vein to open it up and keep it open. So now let's talk about these different treatments. First the ventricular peritoneal shunts. They do lumbar peritoneal shunts but they often will fail. Then the new shunts are ventricular and they go down into the abdomen. This is how many times shunts get revised. These are lumbar peritoneal versus ventricular peritoneal. How often they get removed. And the problem is that shunts often have to be replaced. The new programmable shunts work a little bit better because you can actually dial and a pressure but then you have to work with a neurosurgeon and sometimes that's kind of tricky. Optic nerve sheath fenestration is usually straightforward. You can come in above the eyeball or on the side of the eyeball, behind the eyeball of course, and make a little window and then let the fluid out and that just releases the pressure on the optic nerves. Now the problem with this is it's not available everywhere. It may not help with the headache as much and there are some complications with that as well. But for people who've got vision like this, black is bad in visual fields and you do an optic nerve sheath fenestration, it really, really, really does well. And there's been some new studies out there that maybe the people with extremely high pressure do better with shunts than with nurse sheath fenestration. But it really depends on what's going on and if you have visual loss or not. So the new treatment is this venous sinus stenting and what they do is they go in through the veins and they put a little stent in the middle of the vein to open it up, okay? And the problem with this one is if you get a blood clot in that vein, it's bad news. If it restinoses another spot, it's bad news. The stent never comes out of your brain. So it's there for life. So I'm a little bit nervous about putting, you know, things that stay with you for the rest of your life, especially if you're young. But it's being used now for refractory patients. It can't get any relief any other way. I recently kind of reviewed this with a neurosurgical review. This guy put these data together. So I've made a chart for you. And you can see that the papillodema gets better in everybody, no matter what procedure you do. The visual fields improve mostly in all people. The headaches improve the most with either the stents or the shunts and all of them have complications, some severe. The stents, they had death. So it's not an ipsy-pipsy procedure. Shunts fail, 43% failure rate, so that's not great. And optic nursing fenestration can have some failures but probably is one of the safer procedures that we can offer. What is gonna happen in the future? Well, we definitely need to understand the mechanisms of the pressure, the genetics, these arachnoid granulations, the glymphatics. We've gotta find better ways to improve quality of life. We've gotta find better treatment for visual loss. And for sure, we've gotta get better treatment for headache because that seems to be the most disabling part of this disease. I did wanna tell you about some resources. I'm gonna tell you about the novel library. Novel is a website that you can go to. It's free and it has a bunch of handouts. There are other websites. And we have a website here at the Moran called the morancor.utah.edu, I'll show you that. This is a new book that just came out and I've looked at it and it's written by a neuro radiologist who does stents. So guess what? He's really promoting stents, but he's got a lot of good information in the book too. And I let you know about this. It's the first guide that I'm aware of for patients and families, aside from the pseudotumorcerabri.com website. Now the novel website, novel.utah.edu, this is a joint venture with the University of Utah and the North American Neurophthalmology Society. And we have a patient portal and you just click on what we call the patient portal. And it gives you all kinds of diseases, multiple sclerosis, myosthenia, and of course, pseudotumorcerabri is in there. And then if you click that, it gives you information brochures. It can take you to the Medline, and you can look up new articles about pseudotumor and it has all kinds of other little networks that you can join. If you go to the patient handout, they've got it in a big, large print viewing. They also have it in Spanish, French, Hebrew, Italian, Chinese, a whole bunch of languages. So if you have a friend who has this and speaks a different language, we have it in multiple different languages. And I just did this literature search just last night, 5,302 articles, a lot of them in the last 10 years. So there's more being discovered about IIH and pseudotumorcerabri now than ever before. So we're working at it, believe me. We're working at this here at the University of Utah. We're working at it nationally and internationally as well. Medline Plus is a free website. It's run by the National Institute of Health and the National Library of Medicine. You can just go there. And then all you have to do is go to the P and put out pseudotumorcerabri. And it gives you all kinds of new information with illustrations. I highly recommend that. And then this is our morancor.utah.edu. And this is where this video will be for you. My lecture in 2019 is still on the website. And we have stuff for patients as well as educating our residents and fellows and things like that. But you can get more information at our website too. And with that, I'm going to stop sharing and also look at everybody and say, thank you for being here. And then I would like to, I wanted to save some time for questions. And then I know Lisa's gonna hold kind of a little group discussion about what works for people, what doesn't work for people. Cause a lot of you have learned tricks over the years about how to manage your disease. And it's always good to share. And then to learn that there's so many people that have this disorder, right? That it's not just, you're not alone. There is one question and it says, Dr. DeGray, you just saw my daughter, Katie, her IIH has resolved, how often does this happen? And what are the chances it will reappear? You were amazing. Good question, okay? That's a very good question. So here's the deal. It can go away forever. But what are the risk factors for it coming back? The number one risk factors weight gain. So it's kind of like, if you've got this disorder, you have to just be very careful about not getting into a weight gain issue. I think we have to pay attention to these metabolic syndrome issues that accompany this disease a little bit more because I don't know that we're being aggressive enough with this. And then I always tell people about medications that can re-trigger it, like the tetracyclines and like vitamin A. And some of these medicines that are out there, I'm just a little bit more nervous about that. But the biggest, the number one recurrence factor in all our patients and then the literature is weight gain. We've got a couple more questions here. Can you talk about the differences between low pressure symptoms, maybe from a leak and high pressure symptoms? Okay, so the high pressure symptoms, we've been talking about headache, wishing noises in the head, dimouts of the vision, pain between the shoulder blades, blah, blah, blah. Now, it's interesting that people who get shunt sometimes get low pressure headaches or after a lumbar puncture, you get low pressure. Low pressure headaches are positional. So for example, if you're lying flat, you're okay, but as soon as you're upright, it's really bad. The kind of headache you get doesn't help me at all. The appearance of the MRI scan is critical because the MR will be completely different, okay? The MR of high pressure will show an empty cellar, will show this dilated nurse sheath, blah, blah, blah. The low pressure will show me coning down almost like a carry-like malformation. The pituitary, instead of being empty, will be plumped up. So there'll be actual other symptoms or signs on the MRI scan that will help me as well. So that's a big deal. Another question. What are the risks for the dehesions that I experienced with long-term IIH? Okay, I'm glad you brought this up, but next time I will put this whole topic in. I gave a talk in Denmark this summer where I brought up the conundrums of IIH and one of the conundrums I brought up was individuals that have chronic IIH get these scooped out, not just the cellar scoops out, but the base of the skull gets like eroded through and then leaks occur, it leaks go in the ears, leaks come out the nose, leaks come out other places. And it isn't being studied. So the problem is the ENT guys see the leaks and then they fix them or this neurosurgeon sees the leak and he fixes it. But then the neuro-ophthalmologists don't often even get to see the patient because they don't have papillodema because they've self decompressed themselves the whole time. It's a conundrum and it needs to be studied and we need to be working on this one because I have a feeling this is what happens if you continuously have that high pressure this is like another one besides metabolic syndrome and all that jazz, another thing is these CSF leaks. And the researchers that I was meeting with all agreed that this was a conundrum we don't have a good answer for yet and we've got to figure that one out but I'm going to add that to next year's talk because any literature that I have on that I will present. Dr. DeGruy, this is Debbie Quigley, the one that was asking that question. Is that something that you're going to be talking to patients with long-term IAH about? Well, if I follow I'm long enough but sometimes they just disappear and they're doing great. They go off into the Netherlands, not the real Netherlands like Colin, but they go out. They go out and I never get to see them again. And so it's kind of like, okay, they're done one and done, you know? So I think it's not recognized enough and we have got to do a better job of understanding how often do people with IAH end up with the leaks because we don't even know that. How often do these leaks start? I mean, we don't haven't even done the imaging on people who do have chronic IAH, because we know what they have, we don't re-image them. So we have a lot of stuff to learn on this. So it's a really important topic, but I have to say we're ignorant on this. It's not a good situation. Another one. Is the positional always consistent with low pressure? Could it ever feel better sitting up and worse lying down? Yes, unfortunately, it's not 100%. There are people with high pressure who feel better lying down than sitting up. So it's not 100%. It's just that most people with a low pressure headache initially will have this positional most people. But as you found out, not everybody even gets a headache with IAH, I mean, so you see it's not, everybody is not everybody's the same. Can stress affect IAH? I had a bunch of stress in September and all of a sudden my symptoms all rushed back. I'm visiting my doctor next month as that's the soonest I can get in. Well, stress never, stress doesn't cause IAH, but stress can lead to extra eating. Like some people eat more frequently when they're stressed out. So a little bit of weight gain can definitely push you over. Stress can make headaches worse. It's not the cause of headaches, but it can make them worse. So it is true that stress can play a role and but watch the weight gain. That's the number one cause of recurrence. Is it true that low sodium diets work best for IAH? There's a lot of reports out there on low sodium diets. It hasn't been studied, but a lot of people go on them. So that is something that I've heard. It's just not been studied. So I try to give you, when I give you information, I don't wanna just give you like piecemeal or hearsay. I wanna give you stuff that's been actually studied so that you know, that it's the real deal. But I'll keep an eye out on diets cause it's another important thing. We used to have people go on a keto diet cause you could lose a ton of weight. And there's all kinds of diets like this and low sodium diet. So I've heard a lot of people do that too. So here's a comment. I have high pressure and I can't take naps. Yeah, I've heard that one before. Some people take naps sitting in a recliner chair so they're not completely flat, but they're kind of just partly lying down. And has there been any studies between food intolerances or allergies in IAH? No, lots of studies on food intolerance and allergies with migraine, but not so much with IAH. I'm not aware of any big food intolerance studies with IAH. Anyone else have any questions? Because we wanna make sure that you get enough time to talk to each other too. Cause there are some people here on this call that have been through this for many years and get some advice from some of these very wise people. I just picked one up. You mentioned something early on about iron deficiency anemia and IAH. Can you explain more about that? Okay, it's an association. We don't understand why people get it cause not everybody with iron deficiency anemia shows up with IAH. But there is this strong association with severe anemia like your hemoglobins like six or seven, something like that. And high pressure, but it's not a hundred percent cause you can see a ton of people with a hemoglobin of six or seven and they don't have high pressure. It's just an association, just like not everybody with tetracycline or minocycline gets IAH either, right? They don't get increased intracranial pressure. It's only a few people that do. And we still don't understand why do those few people get that? And I think it's this genetic predisposition piece that we're really missing in this disease. And that's why we're really going after genetics because I really do think that it's important. Looks like one more question came in and that is do antidepressants have an impact in IAH? Well, antidepressants can be very helpful because remember depression was one of the things that make people feel worse. And if you treat the depression, you feel a little bit better, you have more motivation to do things and get out and move and so on and so forth. So yeah, it does have an impact and it does improve depression and IAH has depression. Migraine has depression associated with it as well. It's not, it's real and it's not caused because you don't like your mom or dad or your sisters or whatever. It is caused by brain chemical changes that are occurring. It's often familial. If you have family history of depression, it can run in family. So you have to kind of know your family history because that really helps you too. With any chronic pain, depression was kind of him. Absolutely, where's you down? And if you have any questions about good practices for mental health, Lisa Ord is number one in my box. She is amazing. So you've really got a good treat here because Dr. Ord knows a ton about everything. There's one more that came in. I've had high pressure which has greatly affected my vision and caused damage to my optic nerves. I have been on medication for a long time now and am considered treated. Next month I'm expected to stop medication. What are the implications of it coming back? I still have bad migraines even with medication. Okay, so remember I said we have to treat the headache piece of this. And if the pressure is under control, completely under control, then that's not an issue. But if you have visual loss, you really have to follow carefully with your neuro-optimologist or eye care provider because you aren't going to get to swell as much in your optic nerves. If you've already got visual loss, then they have to use OCTs and they have to do all these fancy tests to follow your swelling. And so if you go off the medication, you wanna make sure the pressure is down. The headache piece, remember, is often is treated more like migraine. And there I would talk with your provider about going on some of those migraine medicines that I mentioned that are very, very helpful. Topiramate is one and that also treats pressure. The new monoclonal, CGRP monoclonal antibodies are good for treating migraine. And then if you've got chronic migraine on a botulinum toxin or Botox is really good for chronic migraine as well. So I'm going to, Lisa, you're the host, right? Yes, I'm not. Okay, so if I leave, I don't knock everybody off, right? No, and I will stop recording so that our support is not recorded, okay? Yeah, and we can even take some of the questions out at the end so nobody's name appears. So, okay, I'm going to leave. I just wanna thank you all for being here. And if you have family members that wanna be part of our study, let us know because we're trying to come up with the genetics of this. And I could figure that out. I think we could cure this disease and where all of us here at the Moran Eye Center are here for all of you, okay? Thank you.