 Well, good evening and welcome to the IJACOPPO webinar series. My name is Evan Jacobson and I'm the Director of Information Technology for the International Joint Commission on Allied Health Personnel in Ophthalmology. Each month, IJACOPPO hosts several continuing education webinars. The IJACOPPO webinars feature leading ophthalmologists, ophthalmic medical technicians, and professionals presenting on current topics in eye care. We want to remind everyone that our presenters generously volunteer to share their time and expertise to help us bring high quality educational opportunities for you to gain knowledge, earn continuing education credits, and help you advance in your ophthalmic careers. For the certified ophthalmic medical technicians in tonight's audience, you can earn one IJACOPPO Group A Continuing Education Credit by viewing this webinar and passing the post-webinar quiz with a score of 80% or higher. You will have through Tuesday, June 11th, to view the recorded webinar and complete the quiz for CE credit. Our presenters will be reviewing and responding to questions in our Q&A forum, so if you have any questions about this topic, we encourage you to submit them there. The Q&A forum, by the way, can be found below the link to this recording. Now, tonight's webinar is titled, Where Hallucinations and Illusions Live in the Brain, and it's presented by Srav Vaganta, MD, and Bradley Jacobson, MD. Nice going on the last name there, Bradley, by the way. That's great. Not many Jacobsons around. All right. Dr. Srav Vaganta is currently a resident physician at the Moran Eye Center. She received her medical degree from the University of Arizona College of Medicine and is interested in neuro-optimology. Dr. Bradley Jacobson is also an ophthalmology resident at the Moran Eye Center. He received his medical degree from the University of California Irvine School of Medicine and is published in several journals, including the Western Journal of Emergency Medicine and BMC Ophthalmology. And now, I am pleased to introduce to you Dr. Srav Vaganta and Bradley Jacobson. Take it away, guys. Awesome. Thanks for the great introduction, Evan. We're really happy to be doing this. Just to state, we have no financial interests or disclosures, and so we're actually going to start off with a case, and I want you all to just remember this case. We're not going to go through the whole thing right now, but we're going to kind of go through the presentations and the symptoms, and then we'll come back to the case at the very end of the presentation. And so moving on to the next slide here. So, Ms. Smith, my name is Dr. Jacobson. What brings you in today? Hi, Dr. Jacobson. I came in because my doctor and the ICU said I should have an eye exam. Oh, okay. Great. Well, why don't you tell me, do you know why your doctor sent you here, the doctor from the ICU? What brought you to the ICU? Well, I'm just 36 years old, but I actually had a heart attack. I went into the hospital because I was having chest pain and they diagnosed me with a heart attack. They took me to the heart catheterization lab, and the interventional radiologist was trying to put in a stent, and while they were trying to do that, they ended up dissecting several of my arteries, and I ended up bleeding out. I had to have transfusions of a lot of blood and platelets, and I was awake during part of the procedure, but I remember passing out, and the next thing I know was... Okay, Ms. Smith. So, this is all great information, but I am an eye doctor. I mean, as much as I would love to hear all of this, why don't you tell me about your visual complaints? So, I'm getting to that. Well, I remember waking up in the ICU and just having difficulty seeing. Like, I could see what was in front of me. I could read some words, but I just kept getting startled when people would walk into the room. So, for example, my ICU doctor, she came into the room and was talking to me, then all of a sudden, I saw this other man in the room, and it was her resident. And apparently, he was in the room the whole time, but I was very startled because I didn't see him walk in with her. So, was it that you weren't able to see the resident physician, or was he blurred out, was he blacked out, or was he just completely invisible until he started talking? When I looked over, I could see him, his face clearly, it's just that I didn't realize he was in the room at first. I only saw one person, and it keeps happening to me. I keep being startled by other people in the room when I think there's only one other person there with me. Very interesting. Well, let's move on to your exam. So, it looks like here, your visual acuity is 2020 in your right eye and left eye, which is perfect vision. Your visual fields are full, but it looks like you did have a hard time localizing and actually saying that you see the number three or number four, but you still performed perfectly on it. Your pupils were equally round and reacted to light with no afferent pupillary defect. And your extra ocular movements also were intact, however, it did take you quite a bit of time to move your eyes in the different directions I was asking you to move. And then when performing the finger to nose test, it looks like you were able to really localize your nose very easily, but when you were reaching out for the objects, it looked like it was pretty difficult for you to actually touch the objects. So, I think we need to do a little more testing. So, what I want to do next is I'm going to put up this screen, and I want you to describe to me what you see. Well, on the screen I see a T. I think there's another T next to it. Anything that the T's are making up right now? What shape are the T's making? Well, I'm not sure what you mean. I see another T. I see three T's. Okay. All right. Well, let's move on to this next screen. And that actually formed an H, Ms. Smith, just to let you know. So, I want you to tell me what, describe this picture to me. What do you see? Well, in this picture, I see a lady. And she just looks like she's washing a dish. And do you see anything more than that? Anything more in the picture? She's standing next to a sink. How many people do you see in the picture? I don't know what you mean. I just see the one lady. Okay. So, there's actually three people in this picture. All right, guys. So, thanks for listening to that. So, like I said, just kind of keep that in mind throughout this presentation, and now we're going to move on to some content. I'd like to start off with defining the difference between hallucinations and illusions. So, hallucinations are conditions where there is no true sensory stimulus, but the visual cortex perceives a stimulus. So, there isn't, for example, light in our vision, but our visual cortex thinks there is light. Whereas in an illusion, there is a true sensory stimulus, but then the brain misinterprets it. So, there's a visual stimulus, like an object, but the brain misinterprets it as something else that it actually is. And we'll go through some examples of this. So, we're going to start off with hallucinations stimulated by the eye. And to start off, we're going to talk about photopsias. So, essentially, a photopsia is flashing light scene. And we often see this, for those of you that work in the retina clinic, we always ask our patients, do you see any flashing light? And what this is is, essentially, the photoreceptors in our eyes are getting stimulated, but not by light, like they normally are, how they're made to be stimulated. They're actually getting stimulated by a mechanical pressure, which then sends a stimulus to the brain, and that's what the brain perceives as light. And, you know, in retina detachment, for example, it's a vitreous pulling on the retina. And then on the next slide, here we see pressure phosphines. And this is a different type of mechanical stimulus. So, definitely don't recommend doing this, but maybe as a kid, when you close your eyes and you press on your eyeball, you would actually see these flashing lights. And once again, this is another example of a hallucination. So, it's not there, it's not present, but your brain is perceiving these flashing lights. Now, I want you guys to stop pressing your eye, because I'm sure some of you are trying this out right now. And now we're going to move on to illusions. So, illusions, again, are conditions where there is a true sensory stimulus, but the brain misinterprets it. So, there is, for example, a light stimulus that exists. It's real, but our brain misinterprets it as something else. An example of illusions include entopic phenomena, and we'll give you some examples of this. So, the first one I want to talk about is sheer phenomenon. So, basically, what this is, is when we see moving stars or small lights, especially when looking at a bright field of snow or blue sky, we're actually perceiving white blood cells traveling in their retinal capillaries. And so, oftentimes, especially here in Utah, people will be going down the slope, skiing or snowboarding, and they'll come in and they'll say, I saw these black dots in the snow. But really, like I just said, it's just the white blood cells traveling through the retina. So, these visual stimuli are actually present. The brain is not perceiving anything that's not present, like hallucination. So, again, entopic phenomena occur when there is a true stimulus within one's own eye, but it can't be shared with another person. So, only you can see it, but someone else can't see it, but it's still real. Another example of this that we see in the ophthalmology clinic is called a Purkinje tree. When you're examining a patient at the split lamp, for example, they might say, whoa, I can see my veins. Right, and that happens to everybody. So, it's not that special, but it's when a bright light is shining in the eye and the patient can see their own vessels and the outline of the vessel, especially when they're looking far up or far down. We have some examples of other types of illusions. Xanthopathy is another example of an illusion, and this occurs when we see yellow. So, xantho means yellow. So, images appear yellow in our vision, even though they're truly just normal color. This can happen when patients have cataracts or due to drug toxicity, such as digoxin, which is a heart medication. As you can see on the image on the left, the image looks more yellow, whereas the image on the right looks normal. Another example of this in art history is Xanthopathy and Van Gogh's yellow pallets. So, there have been many studies that have been reported studying Van Gogh's yellow pallets. So, his images used to have a lot of yellow colors in them, and people thought that this was due to his episodes of malnutrition, substance abuse, environmental exposures, or drug experimentation. They thought that he had taken digitalis, which is the flower that is used to make digoxin, the heart medication, and this caused his vision to become yellow. However, that actually panned out to not be true, because he used yellow even when he was completely healthy, and it's likely that he was just following the trends of the time, and he just wanted to try out a yellow pallet. And then the next thing we want to talk about is something called metamorphopsia. So, essentially what this is, is when objects appear thinner, fatter, shorter, longer than they actually are. And so, oftentimes in retina clinic, what we'll do is we'll provide someone with something called an amslur grid when we're looking for transition from dry to wet macular degeneration. So, we have the patients test one eye at a time, and essentially what we're looking for is metamorphopsia. Looking at those straight lines, we ask them, is there any distortion, any waviness, do they appear shorter or larger than they actually are? Another interesting example is Alice in Wonderland syndrome, where patients can have mycopcia or macropcia. Mycopcia is where images seem smaller than they are, and macropcia is where images seem larger than they are as they did for Alice in Wonderland. The causes of this include damage to the frontal temporal part of our brain. This can be due to migraines, encephalitis, which is an infection of the brain caused by, most commonly, Epstein-Barr virus, which is the same virus that can cause mono, or even epilepsy or seizures. So, I thought it was a very interesting testimonial of a patient who reported what she experienced when she had Alice in Wonderland syndrome growing up. I'm going to read her testimonial to you. It always starts the same way. I'm lying in bed, drifting off to sleep, and suddenly, everything starts to feel fast, frantic, and there's an accompanying feeling of dread. As the feeling builds, the walls start to shrink. Shrink in, and all sense of perception goes right out the window, like I'm inside a massive telescope. My limbs feel out of proportion with my body and my tongue feels too large for my mouth, and it's all happening inside my head. So, she talks about in this testimonial that she used to have these episodes just randomly and more commonly when she was a child, and they'd often be followed by headaches or migraines. She had a whole workup. She had head imaging done, and had met with neurologists throughout her childhood, and there was no specific cause for her condition. She didn't have seizures either, and as she grew older into adulthood, the frequency of that episode decreased over time, thankfully. There are also other conditions called allicin-wonderland-like phenomenon. So, this includes lilliputianism, which is interesting. Patients see others as small people. There's also a hallucination version of this. So, whereas allicin-wonderland syndrome is an illusion, lilliputian hallucinations are, as you can see in this picture, the patients see lots of little people who are not actually there. This can be quite alarming. The causes of these include migraine, seizures, but also schizophrenia or drug abuse, including cough syrup. Okay, so we're going to move on now to something called higher-order cortical visual loss. And essentially what this is, this is visual loss that is caused by abnormalities in the brain. There's nothing to matter with the actual eye, but the insult has actually occurred in the brain. And to start this off, I want to show you all a video that was put out by NPR. It's called the blind woman who sees rain, but not her daughter's smile. And so we're going to go ahead and play that for you. And just so you know, the lady in this video does have a very, very strong Scottish accent. You'll get used to it after a couple of seconds, but we'll come back after this video. The last thing I remember is lying here in my own bed. And I vaguely remember being taken to the hospital. And then after that, I don't remember anything. When I woke up, it was completely black. Absolutely black. When Molina Channing was 29 years old, she was left blind by a stroke. My eyes are perfect, but it's the damage that the stroke did to my brain. It had completely decimated her primary visual cortex, that area at the back of the brain that processes all the information from your eyes. But then, a little while later, I was giving Stephanie a bath and, you know, running the tap, running the water. I see the water moving, but then they went and told all the doctors. They said it's her imagination. And then I started seeing the rain coming down from the sky, the windscreen wipers on, the steam coming from my coffee cup. And though she couldn't see her daughter, I would see her ponytail moving left to right. It seemed to be things that were moving, but nobody believed me. She visited neurologists in Canada who showed her this weird shifting grid. I actually started crying because I could see it. It turns out there are these modules in the brain that are specialized for processing higher order aspects of vision, like recognizing faces or letters or motion. And it seemed that in Molina's brain, the information coming through the eyes had found a way to bypass that broken primary visual cortex and still get out to that motion module, a part of her brain that was apparently still intact. It's just amazing what I can see. I can avoid obstacles and fill the kettle. I'm seeing colors better, but I can't see people. I don't see your face. I mean, you're there, but I just see this shadow. That compartmental nature of vision that may have been her blessing is also proving to be a quiet curse. Just now and again, it hits me, you know, why can't I see my daughter's face and who does she look like and it's so frustrating. And then I think about it for a while and then I think, oh well. At least I'm here. So we'll move on to the next slide here. So what this video is essentially presenting is something called the RIDOC phenomenon. And what this lady had in this video was essentially destruction of her primary visual cortex. So this is where the majority of our vision is processed, but she had this residual conscious visual function with the ability to perceive motion. So she wasn't able to see things that were stationary, but the minute they started moving, she was able to see that. And as you guys can see, let me get our arrow here. So there are two different pathways. There's the V5 pathway, which is the dorsal pathway. And this is responsible for the wear of our vision, so essentially motion. And then over here we have the V4 pathway. This is the ventral pathway. And that deals with more of the what of our vision. And then the V1, 2, and 3 that you see towards the back, this is the primary visual cortex. And that's what was essentially destroyed in this lady in this video. And because of this preservation of the retinotectopulvanar pathway to V5, she was still able to see her daughter moving, to see steam, to see water pulling in the cup. Great. What? I know. So that can be kind of confusing. So we're going to go over this a few more times just to make sure we learn it really well. So essentially neuroanatomists divided the brain into several different regions, especially the cortical areas of our brain into regions called broadband areas. And then we've also subdivided into V1, V2, and so on. So the primary visual cortex in our brain, as you saw in the previous image, consists of V1, V2, and V3. And this area relates information that we receive from our eyes to other parts of our brain. And that includes the V4 and V5 regions. So essentially those regions help interpret the data. So raw data and light arrives from our eyes to the primary visual cortex, the V1, V2, and V3 regions. And I'm going to go to the next slide to show you the picture again. And that information is just raw data that is then sent to the V5 and V4 regions to help us interpret what we're seeing. So to help interpret where an image is in space, we have the V5 area. And then to help interpret what an image is, such as identifying an object or a face, we have the V4 area. So we call the V4 area the where stream or the dorsal stream. Again, this helps with our spatial processing, identifying where an object is in space, its location, whether it's moving, whether it changes shape, and its relationship to other structures around it. And then the ventral stream, or the V4 area, helps with object processing. This helps us determine what the color of an object is, its texture, the details of a picture, its shape, and its size. So examples of disorders of recognition include problems with our V4 area. So object-agnosia, percept-agnosia. Object-agnosia is when we have trouble identifying what an object is. Percept-agnosia is when we have trouble identifying faces. Topographic-agnosia is difficulty identifying locations. And then cerebral achromatopsia has to do with difficulty identifying colors. So color vision problems can not only originate in the optic nerve or our retina, but they can also be directly related to a defect in our brain cortex. So here's another case that I want to share with you guys that talks about this inability to recognize objects and faces. So a 41-year-old man awakes one day, feeling like there is something wrong with his vision. He says, it's all just wrong. He has difficulty recognizing faces and has to rely on the clothes the people are wearing, their voices or hairstyle. He no longer sees any colors, describing the world as a dirty black and white picture. He feels like skin tones are especially grotesque to him. Over time, he has been able to start seeing some colors, but finds it hard to distinguish colors that are similar to each other, for example, blue and green. He now gets lost a lot in his neighborhood, which he thinks is because all the buildings and cars look the same. And so what you see in this picture right here is something called prosopagnosia, which is what Sarawak was just talking about. And so it's the inability to recognize people's faces. And this is essentially how they see their world, except even worse, he also had acromatopsia, and he wasn't able to distinguish between different colors. And so this is, I think, a great picture displaying just what somebody with this disorder might be seeing in their world. All right, so after all that, let's get back to our first patient. So in summary, this is a 36-year-old woman. Remember after the long story she told me, she essentially had a heart attack. She had catheterization to try to unblock those arteries that were clogged, and essentially a complication was dissection of the coronary artery, so she had major bleeding, which essentially led to an ischemic stroke. So when she came in and saw me, her vision was 20-20, and both her eyes. She had full visual fields, although if you do remember, she did have a difficult time, meaning it just took her longer than usual. And then she also had full extroocular movements, although once again she had difficulty initiating those eye movements. She eventually could look up, down, left, or right, but just initiating it was difficult for her. And then lastly, she had very difficult time finding objects. And so that was represented really by that T-picture we saw making the H, and then also the finger-to-nose. So when she touched her nose and she reached out towards the object, it was really hard for her to find that object. We have diagnosed her now with balance syndrome, and she has fit this diagnosis perfectly because she has all three findings that we see in this syndrome. Specifically, she has simultanognosia, oculomotoropraxia, and ocularytaxia. And we're going to break each of these down and talk about what they are. Okay, so to start off, simultanognosia. And so this is the failure of the ability to pay attention to more than one object at a time. So as you recall, I showed her this top picture, the T's that make an H. She was able to identify the individual T's, but she could not express that these T's actually made an H shape. And then this picture that you see of the lady washing dishes and the cookie jar is classically referred to as the cookie jar thief picture. So she could see the woman washing the dishes, but she wasn't also able to tell me about the kid on the stool stealing the cookies or the window or the water overflowing. So people often view this as missing the force for the trees. Our patient also had oculomotoropraxia, which is a defect in the initiation and guidance of eye movements to visual targets. So she had full extracular movement, but following a finger and following it smoothly across her vision was very difficult for her. It was easy for her to lose where the finger was going, but it was also difficult for her to move her eyes to follow that image, as you can see in this picture. Finally, she also had optic ataxia, which is inaccurate reaching to visual targets. So as you see in this image, this gentleman in the top picture is reaching for the spoon that's held in front of him, but he keeps missing it, so he's reaching just below the spoon. And in the bottom picture, again, the spoon, he can see where it is, he can see where his hand is, but he cannot actually physically touch the spoon, so he's missing it each time. So this has to do with poor hand-eye coordination, essentially. Our patient had good finger-to-nose touching in terms of being able to touch her nose, so she knew where her own body parts were in space, which is good proprioception, but when she was asked to touch an object in front of her or the examiner's finger in front of her, she kept missing it, and kind of overshooting or undershooting, essentially. So where is the lesion in the brain when someone has valence syndrome? A typical lesion is actually in the bilateral, a supraparietal cortex. So as you can see in this picture all the way to the left, patients have lesions in the occipital regions, which are in the green here, and also in the yellow regions, the parietal lesions. And in the MRI pictures in the center image and the image to the right, you can see there is a stroke here in the bilateral occipital parietal region. The causes of lesions in these areas are cerebral vascular disease, so, again, stroke. So we see these in areas that are called watershed regions. So areas where there's a gas or less blood supply due to two arteries meeting, essentially. Inoxic brain injuries can also cause this, so a lack of oxygen to the brain. This can be due to, for example, carbon monoxide poisoning. And we also see this in kruzvaldiakrobe disease, which is also known as mad cow. Another more common cause of valence syndrome is actually Alzheimer's disease, which is a neurodegenerative disorder. So a certain form of Alzheimer's disease called posterior cortical atrophy especially affects the occipital parietal region. As you can see in the MRI pictures, again, the occipital regions here have a lot of spaces. So you're actually seeing cerebral spinal fluid in the darker black here and a loss of brain volume, essentially. You can again see that in this image. So we'd like to thank you for your attention. And yeah, thank you guys all for joining us. And there is a form that Evan talked about in the beginning that you can post any questions. We're more than happy to answer them when this goes live. And just some pictures up here. This is our intern class, I guess, to your left using the different lenses. And then on the right-hand side are all of us that are resident retreat. We're a fun group here at Moran Eye Center. So if you guys are interested in the opto or coming over here, just give us a call. Yep. And that's me with the green arrow. And we'd also like to thank... Dr. Don Rafael-Winn, who is a former neuro-ostomology fellow at the Moran Eye Center and who also helped us with this presentation. Thanks again for your attention. Good luck on your guys' quiz. You'll do great. It's easy. Awesome. Well, hey, thank you so much, Dr. Vagunta and Dr. Jacobson. We'd like to thank all of you for attending this webinar presentation. And we remind you to successfully complete the quiz for CE Credit. Now, to register for the next iJacopo Continuing Education webinar, please go to our events page by visiting our iCare Marketplace website at icaremarketplace.org. Once there, click on the webinars and regional events link. Finally, we rely on your feedback to help us provide engaging and high-quality online education. So we'd appreciate you taking a few moments to complete the webinar evaluation that accompanies your CE quiz. Again, thank you for joining us for the iJacopo webinar where hallucinations and illusions live in the brain presented by Dr. Srav Vagunta and Dr. Bradley Jacobson. Thanks, everyone, and have a good night.