 There we go. Sorry. I promised we'd get started on time, so we will get started. Hello and welcome. I'm Lisa Ord and I'm the director of the patient support program here at the John A. Moran Eye Center. A few little things. Handouts. There is a link in the chat if you are online. Otherwise, they're out front. Restrooms out to your right. A few snacks in the back. And please stick around afterwards. We will talk about how we're doing things that have worked for us or things that haven't worked for us. So without further ado. So I am very happy to introduce Dr. Kathleen Degree. Dr. Degree specializes in neuro ophthalmology and headache and practices here at the John A. Moran Eye Center. She evaluates and treats complex visual complaints which can be due to optic nerve or brain disease, such as papillodema, photophobia, vision loss and diplopia. She also treats migraine headaches and other unusual headaches. She is also listed in the best doctors in America. Thanks. Thank you so much. And I'm just pleased to do this. We do this every year. I have an update on idiopathic intracranial hypertension because this is much more common than people realize. And the way I structured this lecture is I want to start with things that are just an understanding of the review of what is IIH or idiopathic intracranial hypertension. And then I'm going to be going into kind of what's new. And those of you online, you can put your questions in the chat, and we will answer all your questions this evening. We don't want anybody having questions at the end, and then stick around because we can have a discussion about what works for people and what doesn't work. And I'm sure there's things that people would like to talk about and maybe meet online other people who have this disorder. So I'm going to review how we call idiopathic intracranial hypertension, talk about the symptoms, the signs that go to make up the diagnosis. And then we're going to talk about why obesity and being overweight is a big part of it and what we can do about that. And then we're going to talk about treatment of the disease to prevent visual loss. And we'll cover medical treatments and also treatments about headache. And I'm just going to start with a case. This is a person we saw many, many years ago, who Sharon, who is 34 years old, and she was sent to our clinic for disc swelling and headaches, and she had recent weight gain, otherwise she's been healthy. Her symptoms were dimouts of vision, a wishing noise in her head, and headaches that were more severe than her normal headaches. She also had migraine. Her visual fields when we did them showed that she had big blind spots and we'll talk about visual fields and why these are important. And when we looked in the back of her eye, she had papillodema. That means swelling of the optic nerve. And I'm going to explain what that is and how you get it. She had imaging done of her brain because everybody with this disorder has to have imaging, usually an MRI scan. And we look for very characteristic findings like flattening of the, of the, the globe in the back of the eye, where all the fluid comes up and pushes on the eyeball. And we look for a big empty cell and other things that we can see. She had a lumbar puncture and had an opening pressure that was 350 where normal is less than 250. And we'll talk about that. And then her fluid was normal. So this, I just want you to see how this is a case, a typical case that comes into our clinic with this disease. Now the first problem is what do you call it because you're going to see idiopathic intracranial hypertension listed in some places and pseudo tumor cerebri listed. Well, this, this condition has been around for a long time. It started out as benign intracranial hypertension, but it's not a benign disease. So why call it benign. So we got rid of that one. Then the idiopathic intracranial hypertension came into vogue. And that just is one part of it because that you can have primary increased intracranial pressure and you can have secondary increased intracranial pressure, and they can look the same. So pseudo tumor cerebri is the name that got put in next and meant a false tumor pseudo means false, a tumor in the brain but you do the imaging and there's no tumor in the brain. The primary form of this disease is what we call idiopathic intracranial hypertension. And that's what I'm going to be talking about. But providers doctors have to take patients history and exam and that's why we do all these tests is to look for the also look for these secondary causes, like thrombosis of the veins and I'll talk about why the veins are so important. And then underlying medical problems like Addison's disease, parathyroid problems, chronic obstructive pulmonary disease or what we call COPD, heart failure, sleep apnea, anemia even can cause a picture that looks identical to the idiopathic form and if you don't look for it, you might miss it and this is really important. Also medications can cause high pressure and if you stop the medicine, usually people get better. And the biggest offender is usually tetracycline minocycline, but things like vitamin a lithium, anabolic steroids, nor plant and Marina have been associated with this condition, and some antibiotics. So that's why the provider has to go through a very detailed history to understand what's going on. Now one of the causes is sleep apnea. And so we always ask our patients about sleep apnea. And this is a little handout that I have for you on on the questions that if you are positive. In this questionnaire, you should talk to your doctor about sleep apnea. It comes from, it's called stop bang. And it's a mnemonic for snoring, tiredness, observed apnea and high blood pressure, that's the stop part. And then the bang part is BMI, and we're going to talk about BMI, age, neck circumference, if you have a big thick neck and gender, males are more at risk for sleep apnea. So I gave you this handout because people who have idiopathic intracranial hypertension are at higher risk of sleep apnea, about 64% of people who have IIH have sleep apnea as well. And a sleep test might be necessary. Now, now we're going to just turn our attention to idiopathic intracranial hypertension. And I want you to understand that while this disease is sort of rare one per 100,000 in people who are overweight or overweight, it's 10 to 20 per 100,000. And that means it's as common as multiple sclerosis. And everybody has heard about multiple sclerosis. But how many people have heard about IIH? Not that many people have heard about this disease. So it's really important that we pay attention to the IIH just as much as we pay attention to things like multiple sclerosis. The age of onset is a little bit between 14 and 60. So it hits women more than men, almost 90% of the people who have this are women. In childhood, it's equal between boys and girls. And we're going to talk about the genetic aspect. There's no gene that's been identified, but there are people who have this disorder that are genetically. Now, the symptoms of increased intracranial pressure are headache is the most common symptom, followed by transient visual obscurations. Those are dimouts of vision. Back pain and neck pain are very, very common, especially pain between the shoulder blades. Dizziness, light sensitivity and almost half people. Pulsatile tinnitus. We'll talk about that. It's a whooshing noise, whoosh, whoosh, noise. Visual loss is in a third. Cognitive slowing or having difficulty with thinking in about 20% double vision also in about 20% of people and ridiculous pain just means pain going down the arms or to the leg, the legs. Headache is the most common symptom. It's also the one that disables the most people. It also is associated with pain with eye movements. And how bad the headaches is does not correlate with how high the pressure is. In other words, you can have a very high pressure and have almost no headache. What's really interesting is 40 to 60% of patients with idiopathic intracranial hypertension have a previous migraine disorder. That means that there's this overlap between migraine and this disease. Normally, in the normal population, 20% of women, 10% of men have IIH, but in this disease, almost half of the people have underlying headache or migraine, and the headache piece correlates with worse visual quality of life. So the other symptom is hearing symptoms. And they're very common. This whooshing noise, sometimes hearing loss, sometimes dizziness. Cognitive impairment also can occur. And there's many studies out there that show that the thinking can be slowed. It's important because when you do a visual field, which is how we follow this disease, sometimes that thinking being slowed can give us erroneous visual fields. And the slowing of the thinking can be related to a number of different things like how high the pressure is, how bad the headache is, whether somebody has sleep apnea, whether their executive functioning is also reduced. Now, so those are the symptoms. Next, I'm going to go to the signs. That is, what do we look for when we see patients? The first thing we do is visual acuity. And that really is important, but it's not enough. It's the last to go. If you've got problems with this disorder, that's why we have to do visual fields. And anybody who's done a visual fields know if you've put your little chin in this Humphrey machine or in this Goldman machine, this can be a tedious task because you have to push the button when you see the light go on. And this is an example of a visual field that we get on people with IIH. And visual fields predict the visual outcome. So that's why we have to follow the visual field because if the visual field is abnormal, then the visual outcome could be poor. And we don't want bad visual outcomes where we don't want anybody to lose vision in this disorder. Next, we look in the back of the eye. The reason we do that is we're looking at the optic nerve. So here's a cartoon of the eyeball, and it's connected to this optic nerve, which is connected to the brain. And the pressure in the brain gets distributed along the optic nerve, and that's what causes the papillodema to occur. So when we look in the back of the eye, we're looking at this optic nerve. Here's a picture of the optic nerve. Now, what does papillodema look like? Well, this is somebody who doesn't have very much papillodema or no papillodema at all. It looks like a normal nerve. That's what it should look like. But here, look at this. This one, you can see that there is that C-shaped swelling along the nerve. There's even some wrinkles in the retina. This person has swelling all the way around the nerve. This one has really severe swelling to the point where there's hemorrhages in the back of the eye. And that's what we're looking for is papillodema, which is disc swelling, which is a sign of increased intracranial pressure. And I already showed you a picture of the imaging that we look at. We look at the optic nerve sheets. You can see how that fluid has come along the optic nerve and is pushing on the back of the globe, giving us that picture of the way that looks. The lumbar puncture is necessary because we need to know what the pressure is. 250 is considered normal. In children, it's a little bit higher. It's up to 275. And the fluid has to be normal. If the fluid isn't normal, then it could be one of those secondary causes that I talked about at the beginning. So we have to know what the fluid is. Now, we are looking for the genetics of this disorder, and that is one of the, it's kind of the holy grail. But I'm going to share with you other hypotheses of what causes this. Because everybody goes, well, why do I have this? What's going on? Genetics is one part. The venous system may be one part. Lymphatic drainage may be one part called the lymphatics. And then obesity, and I'm going to talk a lot about that because about 90% of the people who have this are either overweight or obese. In the genetics, we have many examples in the literature of mothers, sons, daughters, sisters, brothers that have the disorder. I had twins that are symptomatic. I had one case of twins where one came in with symptoms and I looked at the sister sitting in the room and she had it too, which was really interesting that they would genetically have it at the same time. The data is needed. We have studies going on here at Moran Eye Center looking for families. So if you have family members with this disorder, we'd love to hear from you and we'd like to include you in our study. Now, in order to go further, we have to know about how spinal fluid is made, and then how the pressure goes up. This is a cartoon of the brain, and the brain is covered with a dura, and then of course we have our skulls on the outside. But CSF is made in a structure called the choriplexus and it lives in our ventricles, and it secretes spinal fluid. It secretes a lot of spinal fluid. In fact, the spinal fluid is renewed every six to eight hours in our brain. Can you imagine? Every six to eight hours that fluid has to be washed through our brain. And it's absorbed by these arachnoid granulations that live up by the venous sinuses and it goes into the venous sinuses. It's all over our cerebellum. And the reason we have spinal fluid is to make our brains buoyant inside our head so that every time you got a little knock on the head, you don't have a brain injury. Okay, it's what protects our brain from injuries. So the veins are all over too. There we've got these big, big veins and many, many, many veins. We have the sagittal sinus, we have straight sinus, we have transverse sinus. But you can see these little arachnoid granulations that stick out into the, to the big veins. And that's where the fluid gets absorbed into our system and also through the lymphatics, which are by the veins. So the venous hypothesis is, as the pressure goes up, the veins collapse, and as the veins collapse, the pressure goes up. So people who, let's say, are bodybuilders, trumpet players who exert a lot of pressure in their abdomens can increase their venous pressure and can, for a while, increase their spinal fluid pressure, but we don't run around, tuning a trumpet or lifting weights all the time. So the venous sinuses can be compressed in this disorder and, and, or they can be absent or a trophic or have little arachnoid granulations in them. So that's why we have to look carefully at those venous sinuses, because they can be affected in this disease. Now lymphatics have been known for years, but these have been just shown to be present in brains where the drainage occurs. And these lymphatics take all the sewage from our brains from the day to get rid of the waste and cleanse our brain and get rid of all this, this way. So they are an important part of our, our brain chemistry, and they may be involved in this disease as well. They could play a role by stopping the spinal fluid exit through those arachnoid granulations, and also they could cause, if they don't drain properly, they could cause more stenosis of the veins, and thereby increase the pressure. So this is a disease that's kind of complex. And, and, you know, the fluid gets made in that chloride plexus, and, and it gets picked up by these arachnoid granulations, and then the venous sinuses can also get compressed, and that leads to the pressure getting higher as well. So it's kind of a sort of a whole system problem. And if there is obesity, and we're going to talk a lot about this because this occurs in this disease alone. This disease is, is not everybody who's obese has this disease, but people who have this disease are overweight, have over be are a little bit overweight and or obese. And that alone may release other factors that play a role in, in this whole disease so you can see that it's a complex disorder of the brain, the spinal fluid, and the egress of the spinal fluid. So now I'm going to talk about obesity, and, and this has been recognized since early on that 80 to 90% of everybody who has this has a little bit of problem with weight. And weight loss does improve this. And weight, the obesity can be associated with lots of different other syndromes like orthostatic edema syndrome. This is a syndrome where people are upright during the day and they collect fluid and then at night, they have to pee all night, because they've, they've got this edema thing going on. People have increased venous pressure, and I already told you that that can really increase the intercranial pressure. They're more at risk for sleep apnea I told you 60% of people with this disease can have sleep apnea. They're more at risk for polycystic ovarian syndrome and we don't know how those hormonal changes play into this. And they're more at risk for metabolic syndrome and metabolic syndrome is associated with diabetes and then having metabolism metabolism go haywire as well. So this graph is just of the United States. This was 10 years ago. And I want you to see that the dark red here is 35% of the people of, let's say Mississippi and West Virginia are obese. And then, as you can see as time goes on, our country is becoming more and more obese this is 2020. And you'll notice now it's 35 to 40% in that dark red. And you're going to notice that the dark red is 40 to 45%. They had to change the color coding in Utah where fortunately we're on the lower end but we're still 30 to 35% of our population is obese. And we're not alone. Look at many, many other states are with us as well. So what is obesity and how do we characterize it we go by what's called the BMI body mass index, and it's your height, and by feeding inches and your weight, and then you compute of this. Overweight is a BMI of 25 to 30, and obesity is a BMI of over 30 and there are calculators on the internet, and also handouts that we've given you so you can check what your BMI is because I'm going to tell you some of the studies that have been done about a certain level of BMI about what, what we should be offering more of. So, like, you know, you have so if you like, if you're five foot two inches and you weigh 130 pounds you're a little bit overweight, you've got a BMI of 25. That's how this graph works. So you take your height and inches, you take your weight, and then you can find your BMI. All right. Now, what is the rationale about relating obesity to increase pressure. Well first, abdominal adiposity or the fat we have in our abdomen can exert pressure on the venous system so that's one piece. I was really curious about the obesity and overweightness of IIH is there are metabolic factors that keep people from losing weight. So, like, insulin resistance so there's problems where insulin is released but it doesn't work the same in people who have IIH compared to women who are of the same BMI and the same age. And it's like it's not fair. Okay, leptin also is increased and increased leptin is associated with more body fat mass, larger fat cells, overeating excessive hunger. Then there's inflammatory markers that are present in people with IIH that aren't in present present in other people with out IIH. And as the weight goes up in some individuals so does the pressure. But what we do know is if we can lose the weight, five to 10%, we can bring this disease into remission it can go away. So this is just a cartoon about all the things that go wrong and IIH compared to women of the same BMI that don't have the disease. So this insulin resistance, hyperleptinemia, different changes in the fat cells and different changes in the fat and the body as well. So it's a metabolic disorder really that is really present in this IIH. Now the evidence for weight loss has been for a long time. Dr. Newberg did a study where she put people on a rice diet. Can you imagine eating rice all the time? Anyway, these people lost a lot of weight and look at and she published that their papillodema went completely away within about three months of being on that rice diet. Harvey Sugarman did bariatric surgery and normalized the weight in individuals with IIH and their papillodema went away. Mark Coopersmith in New York found that a 10 pound weight loss improves papillodema and Alex and Claire put, and her colleagues put people on a 425 calorie diet for three months and watched their weight go down and also their papillodema went away and their endocrinial pressure reduced. But 425 calories. Can you imagine living on 425 calories? That sounds almost impossible. Now the reason we have to consider this weight also is because in IIH, if you have this disease, you've got to get it under control because there is a higher risk of other cardiovascular disease. Heart failure is almost twice as common in women who have this disease. Stroke is also twice as common. Type II diabetes is more common as is hypertension. So it's important that we get this under control so that our future vascular risk factors are not there either. So what can be done for weight loss? Well, there's lots of weight loss programs. They need to be multidisciplinary. They need to take in consideration diet, exercise, but they also have to consider emotional factors, medication factors, body factors. So you want to work with people who know what they're doing. Weight loss drugs, which I'm going to talk to you about, should be done by specialists, and then weight loss surgery has also been shown to be helpful in people who have BMIs over 35. Now, in the news, I'm sure many of you have heard about these new drugs for weight loss. Oprah Winfrey was on one of these and lost a ton of weight. They're called GLP-1 agonist, glucagon-like peptide-1 agonist. It's in the news. It's a hot topic. It's secreted by the gut neuropeptide. It's secreted in the small intestines after a meal. And this drug has been used to treat diabetes and also for weight loss. What it does is it signals satiety to the brain, so it tells the brain, hey, you're not hungry anymore. It stimulates insulin to secrete more insulin and inhibit glucagon, and that lowers the glucose. There are receptors in the coroid plexus, so that means these drugs can affect the CSF secretion. Remember, I told you that the spinal fluid comes from the coroid plexus. And then they've done studies on rodents that have lowered intracranial pressure by putting them on these drugs and does lower the intracranial pressure. And I'll tell you about studies that have been done on IIH. There are many different types of these drugs. I'm just bringing out the two most common ones. Semaglutide and exenotide. Exenotide also goes by biata, which is an injection for diabetes. Semaglutide with Jovi or with Govi is for weight loss, but the same drug semaglutide is ozympic for diabetes. Don't ask me why they have different names of the same drug, but anyway, it can lower the weight by 15%. So there have been trials in idiopathic intracranial hypertension, and one trial was done in Great Britain by Alex Sinclair and Susan Mullen, their group, where they enrolled women to exenotide or placebo. These women had agreed to have a pressure monitor put into their brain and monitor their pressure for 12 weeks or three months, and they were able to show that in two and a half hours, the pressure was lowered by four millimeters of mercury. And this lasted for 24 hours and also for three months, whereas the placebo group did not have any adjustment in their pressure. What was kind of sad was their headache days per month did decrease, but the severity did not. Then there have been other studies. This group had an open label study where they put 39 patients with IIH on semi-glutide or lyraglutide, and they got 12% weight loss versus 2.8% in placebo. So the people who are on this drug definitely lost weight. They had a reduction of headache days per month of four. They had a lower dose of acetazolamide, and they had very mild side effects. However, I want to caution everybody that these drugs have other side effects and many are just being discovered. For example, there is a nine times risk of pancreatitis, a gastroparesis of three-fold, and bowel obstruction because the stomach doesn't empty as quickly, and so then there can be other side effects. So it's not a drug that you want to just put everybody on because you got to think about what the consequences could be. But if it's done appropriately by providers that know what they're doing and giving this drug, it may be helpful. Next, I want to cover surgical weight loss procedures. Now, I've got cartoons of what happens. This is called a laparoscopic adjustable gastric band, where they put a band around the top part of the stomach, and it just makes it so that there's just this little pouch for food to come in and makes you full, and you can't eat anymore, and it still uses the same stomach all the way down. Then there is a vertical banded gastroplasty, which just bands part of it from the esophagus into the stomach. Then there is a major surgery where you hook up, the intestine gets hooked up to the stomach and it bypasses. That's why it's called a bypass surgery. It's called a ruin my bypass. And I'm going to tell you about the studies that were done on weight loss procedures. It's been used in people who are obese and have diabetes, metabolic syndrome, sleep apnea and hypertension. So these are gaining popularity. The ruin Y gets the most weight loss, but it also has higher complication rate. This study also came out of Birmingham, England, the Susan Mullins and Alex and Claire group, where they studied women over five years, who had BMI over 35. So you have to check your BMI to see if you'd be a candidate. They were all enrolled in a current weight watcher program, but they had a group that went for bariatric surgery. So the control group was just the weight watcher program. So that's the control group and the bariatric surgery is the actual surgery. And they had different types of surgery, although different types I mentioned, but they were able to get a lot of weight loss 21 kilograms, which is 46 pounds of weight. Their quality of life improved by seven points. Their headaches improved in the weight loss program and the weight loss continued for over two years. 24% of the people had had normal pressure with the weight loss, and they had only one bariatric complication. And this graph just shows you how the weight went down with a surgery group. And then they, and this graph shows you this is the intercranial pressure, the intercranial pressure went way down with the surgery group compared to the just the current weight watcher program. Those people lost some weight, but not a lot of weight. So, so that's the story on weight. Now, we don't, when we see people in our clinic, the first thing we want to do is get the pressure off the optic nerves so that the people don't lose vision. So, we give people acetazolamide, and that has been shown in several trials to be very important to preserve vision, reduce papillodema, and it may help a little bit with weight loss as well. And in the most important trial that came out on this, the idiopathic intercranial hypertension treatment trial, and the University of Utah, Moran Ice Center was a part of this study. We found that if you got, if you went on acetazolamide and a diet together, you had a better result in improving your visual field outcome. And you can see that the placebo and diet, they got a little bit better too, but not as well as the people who were doing the acetazolamide and the diet. And you can see here the weight loss was much more with the acetazolamide and diet compared to the people who just were in the weight loss program. What they found was the papillodema grade also reduced. So, these people, their papillodema got better also within six months. It went down to much lower grade of papillodema. The acetazolamide group had fewer failures. So that and all the people that failed were those people that I showed you that had the high grade papillodema and had visual field defects kind of right off the bat. Their quality of life got better with the acetazolamide and diet and all the different measurements that we did for quality of life. The unfortunate thing was that acetazolamide did not treat the headache so the headache didn't get that much better in either group so the headache stayed the same and that's kind of the bugaboo of this disorder. The pressure did get lower so we did in a number of people agreed to get their spinal fluid pressure checked and we found that their pressure definitely reduced by almost 60 millimeters. So if you were at let's say 300, you would go down to 240 within the normal range. Now, acetazolamide can have effects. There weren't that many serious effects. The normal stones occurred in two people but out of, you know, 300 change in liver function and one pancreatitis and one, and then very various other things the most common side effects are pierce these are tingling around your mouth and, and your hands taste changes. So a little pop and any kind of carbonated beverage is going to taste terrible. It can cause fatigue some people have nausea some people have vomiting diarrhea tinnitus, and some people their electrolytes go a little bit off so we recommend people drinking Gatorade light to combat some of these peristhesias and changes in taste. What we found was the acetazolamide plus weight loss diet and weight loss program did work, and, and we recommend the maximally tolerated dosages of acetazolamide it's really important to get up there as much as possible. So there are if you can't tolerate acetazolamide what other choices do you have methozolamide is a cousin to acetazolamide doesn't work quite as well hasn't been studied but does work pretty well. So it's like a rosamide also has been used a milleride also can reduce spinal fluid production, our triatide can also work for some people, and a drug that we use for headache to pyramid also does reduce the entercranial pressure so this is a drug that people can can take, and I will combine to pyramid anacetazolamide together to get the best reduction in the intracranial pressure and the best headache result. So my ending part is to deal with the bugaboo of headaches. It's the most difficult part of treatment. I'm going to talk about visual loss and what we do with that. But the headache piece of this is so hard for people. And part of it is that half the people who have this disorder have constant daily headaches. They're hit six. How bad the headache is is 60. That means it's bad. And over 40% to 50% have pre-morbid migraine, meaning they already had migraine. And then on top of this, they have the pressure headache. And then you can get the papillodema gone. You can lower the pressure. And then the headaches continue in about 43%. And so the best outcome is usually is in people who are younger who've had high pressure and we bring down the pressure and get it under control right away and control the intracranial pressure. That's usually the best outcome. What we learned from that idiopathic intracranial hypertension treatment trial is that most people, the phenotype of the headache that people have with IIH is migraine or probable migraine. And that a third of people have what's called tension type headache. That means that they have no nausea, no light sensitivity, or sound sensitivity. But about half the people in that group had medication overuse, which makes headaches worse. And the worse the headache was, the worse the quality of life was. And here's another thing that just boggled my brain. The headache disability did not correlate with the opening pressure. So you could have a high pressure and have no headache. It didn't correlate with the BMI. So the higher the weight didn't correlate with the headache. How much weight they lost didn't correlate with the headache. How bad the disc swelling was, the papillodema or the visual field didn't correlate with the headache. So this headache is like its own behemoth problem. And there's no difference between the pressure with or without headache. In other words, they looked at the people who didn't get any headache and they looked at the people with headache and the pressures were identical. So how do we treat it? Well, we work on the weight loss because we know the papillodema will go away and remember even 5% to 10% can be helpful. And there are studies that show in chronic migraine if you can lose some weight, the headache can get better. So weight loss can help chronic migraine too. We use medications that lower the pressure like acetazolamide and those other diuretics I mentioned. And the best treatment is the diuretic plus a migraine preventative. Some people say, well, I'll just go get a bunch of lumbar punctures. Well, it's not worth the pain and there's little gain because that spinal fluid I already told you remakes itself every three times a day. And then you have to watch what medications you're given because some of those medications can cause weight gain. So here's kind of a list of the diuretics, acetazolamide, methozolamide, ferrosamide, chlorothaladone. These are diuretics that can be used to lower the pressure and these are the migraine medications to pyramid remember may lower intercranial pressure all by itself. So this one is one we use a lot plus it does help with weight loss. Tricyclics are great, but they can have weight gain. Velproate definitely causes weight gain. The new kids on the block, CGRP monoclonal antibodies once a month shot for migraine does work and has been reported in IIH and on a botulinum toxin. If the phenotype of the migraine is chronic migraine has been reported to be very successful as well. So these are ones that we go to if the headaches are really severe. So now I'm just gonna mention the surgical treatments for visual loss. If people lose vision with this disorder what's in and they've been on their medical therapy and they're still losing vision, what can we do? Well, we can do lumbar punctures and sometimes in this high risk group of people who've got bad papillodema, bad visual fields and already have visual loss. We will put a lumbar drain in and stabilize the pressure to get the pressure off the optic nerves, but that's not a long-term solution. Way back in the 50s and 60s they took off somebody's bone off their head. Can you imagine getting part of your skull removed? I wouldn't recommend that one at all. So we don't do that anymore. Gastric bypass I mentioned but that's not a treatment for visual loss. It's only a treatment for weight loss. So the three procedures that we can have for this disorder with vision is optic nerve sheath decompression, I'll show you what that looks like. Shunting, lumbar peritoneal versus ventricular peritoneal shunts but ventricular peritoneal shunts are way more popular now and programmable valves are more popular and then what we call venous stenting and I'll show you that. So first ventricular peritoneal shunts. So here's a cartoon, they put this little shunt in the ventricle and then they run the tube all the way down underneath the skin into the abdomen and put the fluid the spinal fluid into the abdomen. Now, these shunts fail, okay? They have to be revised, they get removed, they get replaced and the failure rate is about the same for the lumbar peritoneal versus the ventricular peritoneal and here's the bottom line, if you are using a shunt for headache, 50% will have chronic headaches eventually after the shunt is placed. So the shunt is not a panacea for the headache. It does get the pressure off the brain no question about that. People who have idiopathic endocrineal hypertension without papillodema do worse with these shunts so we do not recommend it. Optic Nershie Fenestration, this is a, if you don't like blood, close your eyes. This is a person's eyeball and this is what we call a lateral approach. We don't do this approach anymore. Now we do a medial approach or a supraproach where we go above the eye crease so you can't even tell it but our goal is to get this fluid off the optic nerve. This is the optic nerve going into the eye. It's usually straightforward done by an oculoplastic surgeon. It protects the optic nerve. There are very few reoperations. It helps the headache a little bit but not as much. There are complications of course and problems. It's not available everywhere. Not everybody has an oculoplastic surgeon. It may not help the headache and you can get some double vision afterwards. Sometimes there's a pupillary change and so on but most of the time it's pretty safe. The other one is venous stenting. I told you that the veins get compressed in this disorder by the pressure and if you do a lumbar puncture frequently the lumbar puncture will allow the vein to open up and then the pressure can drop. But sometimes people have those little arachnoid granulation sitting in the veins and it like here is an arachnoid granulation that's plugging up this vein and then the stent is like a tube that's put in the vein to open it up and it really is good maybe for people who have failed all medical therapy, other surgical therapies. And here's what it looks like. This is a vein and this is a big arachnoid granulation that's plugging up this vein or sometimes there are septi that plug up the vein but here's the caveat is you have to show a gradation over the eight millimeters of mercury or more across this stenosis otherwise there's no point in doing it. And you have to have a pressure in the head of over 300 millimeters and you have to have no contraindication to dual platelet therapy. That means aspirin and then this clopidrogel because if you don't go on anti-platelet therapy you can clot off this stent and then venous sinus thrombosis and everything else can occur so that's like bad, bad, bad news. And people do it sometimes for disabling headache, focal neurologic deficits, papillodema and visual changes. Now there was a big study that took all the studies in the literature together and said what happened? So they found 49 studies and 250 patients and they found that the pressures got to normal so pre-stent the average pressure was 330 after the stent was normal at 197 the visual obscurations got better in 81% the whooshing noises got better in 85% the headaches got resolved in 36% improved in 70% but were the least likely to resolve in every study. The papillodema resolved in 41% improved in 38% and these are just pie charts showing that most people got better their symptoms got better, their tinnitus got better but headaches didn't get much better but there were serious complications thrombosis where the vein can clot off or you can get a hemorrhage or you can get swelling in the brain or the stent can migrate where it shouldn't go and there's even been a report of death. So it's not like you wanna go out and put the stent in everybody for no good reason. And I've just shown you here comparison of the procedures the papillodema and all the procedures gets better the visual fields improve and all of them and the headaches improve as well. So our future here is we need to understand this disease better. We've gotta get the genetics down here I think if we can understand the genetics why would somebody who has the same BMI and then have completely different metabolic features going on? We've gotta understand these arachnoid granulations better the glymphatics better. We definitely have to understand the weight loss here. It's complicated, it's not simple. And we have to have better treatments to improve the quality of life. We've gotta have better treatments for visual loss and we really need to treat these headaches better because it's what disables most people. Now I've got, we're gonna, if you want a copy of these slides Dr. Ord will send these to you and set it up for you to look at the slides but there are resources for you. There's this book, idiopathic and cranial hypertension explained. You can do it on Amazon or order it from the bookstore. I also want you to know that the novel library which was founded with the North America Neuroophthalmology Society, I'm a neuroophthalmologist and the Eccles Health Sciences Library here at the University of Utah set up a whole library and we've got a patient portal and that patient portal has all kinds of resources for all kinds of neuroophthalmic conditions including Pseudotomorcerabri and we have information brochures. You can look at other people who have this disorder. You can connect to other groups and in your handouts, I've given you one of the handouts that they have on idiopathic and cranial hypertension or Pseudotomorcerabri which goes through kind of what we've taught I've gone through in a little greater detail. I also looked up two days ago when I finished this talk and I just want you to know people are studying this disorder. You can see that over the years it's becoming more interesting to researchers and publishing more. There's almost 3,000 articles on IIH in the literature. Medline Plus is also a really good resource for you and then I want to tell you that here at the Moran Eye Center, we have been studying IIH since I came to this place over 35 years ago and we're still studying it and we're not giving up and we've got lots of resources in our Moran Core which is the clinical ophthalmology resources for education and this lecture will be placed on the Moran Core by Dr. Ord and our colleagues here. And with that, I wanted to make sure I left time for questions so that we can answer questions from our Zoom audience as well as our questions from our audience here. So I'm gonna stop sharing right now and please, and you can unmute yourself and ask or you can put questions in the chat. We're happy to answer questions either way. Any questions from our audience here? Did I explain it well enough that you feel like you've got a better handle on what this is about? So how does medication reduce pressure I use to pyramid and what happens if you stop it? That's a really good question. And to answer that question, it's been shown in laboratory animals and in people but there's never been kind of a controlled trial on this but to pyramid has been shown to lower the intercranial pressure in laboratory animals and in people. And so the way it does it is it's the same mechanism as acetazolamide where it has a carbonic and hydrates inhibition so it reduces the amount of spinal fluid being made. So that is how that drug works but it also has a lot of other actions for migraines. So for example, it's been shown in many, many, many studies for episodic migraine and chronic migraine it reduces migraine headache. So that's why we often will use to pyramid if there's a lot of headache going on we'll use it along with acetazolamide we have to be a little bit careful with electrolytes because they both do a little bit of the same thing. Now, if you stop the to pyramid could you have an increase in your pressure? Yes, you could. If you stop the to pyramid, could your weight go up? Yes, it could. If you stop the to pyramid, could your headaches go up? Yes, it could. In to pyramid, the secret to tolerating that drug is to start low and go slow. So I start like with 12.5 milligrams and every week increase it by 12.5 milligrams to 25 and then 25, 37.5 and then 50 and then you can see how I do it very, very slowly. Okay, a question is do I have to have a referral to Miranda Eye Center to discuss or receive drugs like acetazolamide or to pyramid? You have to have those prescribed by a provider. Now, if you have a provider that takes care of you and you could have a discussion with them about acetazolamide and to pyramid and ask them if you could be put on those drugs but we won't give out drugs unless we've seen the patient because we have to follow people and we wanna make sure we're doing the right thing for people and we don't want people to have bad side effects or have complications and all of that. And yeah, IH is a journey. It's a journey for everybody. It's a journey for us. I have to say I am studying this disease as often as I can. Every single thing that comes out new, I'm studying it. I'm talking about it at the American Headache Society meeting later this month because people with chronic headaches have this disease and nobody knows what to do about it. So, okay, do you consider stenting or other surgery in cases without papillodema but where the other symptoms aren't getting treated by medication? When you know the patient still has high pressure via an angiogram or LP. That's another good question. Yes, we consider it but we are very careful because remember I told you that if you don't have papillodema, the people that get shunted don't do very well. They still have chronic headaches. Stenting, same problem. The headache can still be severe. Although, I would definitely talk to your provider about a vinaigram to see if you have a fixed stenotic area that could be taken care of with a stent. But I'm very careful about it because I really, I mean, I told you about the complications, thrombosis, hemorrhage, edema, death. I mean, you know what I mean? You have to really think about whether you wanna do this or not. Okay, how long can you take medication? I've been on my tapirumate for five to six years. Well, I've got people who've been on acetazolamide for about 15 years, 15, 20 years. Now, I try to reduce it whenever I can but then if the papillodema comes back or if there's weight gain and the papillodema comes back, they're back on the acetazolamide. So that unfortunately is something that you know, is a problem. Yeah, people can be on these medicines for a long time. We, you know, I work in the headache clinic too. So I have lots of people who've been on tapirumate for many, many years and it's controlled their migraine and if they go off, the migraines come back, they go back on, the migraines get better. So they wanna stay on it. I don't have any data about long-term complications of tapirumate or acetazolamide. Another question is, I've noticed cognitive impairment since my diagnosis, what tools can you suggest to help combat this symptom? Good question again, very good question. So cognitive impairment I think comes from multiple angles. One is the pressure itself. So getting the pressure down can help with that. The second thing is the headache, getting the headache control because if you've got a chronic migraine headache going on all the time, I can tell you, your brain does not work very well at all. You've got cognitive impairment right there. So the key is to get those symptoms down. And then the third thing, I just wanna mention depression and anxiety. Depression does run with this disorder. A lot of people have depression and if you're depressed, your cognition is not good either. So it's another one of those things that takes your brain out of gear. So it's like, you gotta control the pressure, you gotta control the migraine or the headache part, you gotta control the depression, anxiety. And I mean, it's like a whole package of unfortunate things that you have to be on top of and it's like not fair. Pyramid is topomax. Yes, topyramid is the generic name, topomax is the trade name as well. Topyramid is not usually used in mood disorders. In fact, topyramid occasionally can cause depression when you first start it. So you have to be careful if depression increases when you get topyramid, if it doesn't go away, you should immediately talk to your provider about it because we don't want people having depression on top of everything else they've got going on. That's right. Pain can really wear you down. It's, that's exactly, that's exactly right. Anybody have any more questions? And I'm gonna get Dr. Ord up here. Oh, here. I think my family member could have the disease as well but they do not have insurance. They have similar symptoms as me and have a partially empty cella. Is there a good recommendation for screening? Go to your primary eye care professional optometrist or ophthalmologist and have them look in the back of the eyes and look for papillodema. If there's papillodema, they can get referred up here and we take care of people. Okay, what's the long-term prognosis if you're diagnosed as a teenager? Okay, good question. Well, I can tell you this. That study was done by one of my mentor, James Corbett where he brought in people 25 years after the diagnosis did spinal taps on several of these and many of them had continued to increase intercranial pressure. So it is a chronic disease. It is not like a one and done. And if the weight comes back, that is the number one trigger for the condition to come back. So that's why it's so important to get the weight down the pressure down, the headaches under control trying to get everything under control so that you don't have the long-term consequences of this disease. Is it safe to get pregnant with IIH? Another really, really good question. So yeah, it is. Now, acetylzolamide, we tend to try to stop it in the first trimester, but the studies have been done on taking acetylzolamide throughout pregnancy. There've been no major birth defects and no major complications and you can have a pregnancy. We recommend not getting excessive weight gain and then work with your provider to make sure your optic nerve and your visual fields are followed. Oh, here's another question. In terms of birth control, I've been advised standard birth control should be avoided previous marina patient. Are there recommendation discoveries in this area? Are hysterectomies feasible possibility? Well, we don't recommend hysterectomies unless there is a gynecologic reason to get a hysterectomy. Tubal ligations work great for birth control and the other problem for oral contraceptives, well, marina IUDs have been associated with this disorder, although the data are not completely clear. Oral contraceptives that have a lot of estrogen are not good for people with migraine because of increased risk of stroke and heart disease and blah, blah, blah. So it's like rock in a hard place here. There are some progestin only birth control options that could be tried and IUDs are great. The non-alluding IUDs, ones that don't have the hormone in it are great for birth control as well. I just got a Kylina and unfortunately, I don't know what a Kylina is. So, okay, it's an IUD, all right. And does it allude a hormone, do you know? Because I don't know that one. Oh, progestin, okay. Yeah, well, that's marina is a progestin alluding IUD. So I would just keep an eye on it. Keep an eye on the pressure of the papillodema, the visual field, headache, et cetera, and make sure that there's nothing that's getting worse. Any other questions?