 All right. Good morning everybody. I'm going to get started. We have two presenters today. First is Dr. Lee Chung. He's a second year neurology resident. He's been on the neuro ophthalmology service with us for the past month. He's going to be presenting a case of word blindness. And then second up will be our Dr. Jim Bell. He's going to present a case of red eyes and intubated a uveatic curiosity. Thank you guys for having me. Today I wanted to present a case of a symptom I've actually had myself before. Specifically I'll write something down and then look back at it later and I have no idea what I wrote. I can't read it at all. So actually we had a patient who complained of the same problem. He basically came in and said, I can't read anymore. His exam was completely normal and his visual fields were fine. So I had to dig back in the literature to see if that was even possible. So the patient had a question. So he's a 16-year-old right-handed boy. He was previously healthy. One day he wakes up with this bright white light in both his eyes. He can't see through and he's got this pounding headache. It's a migraine headache. His vision gets back to normal but he's just left with this headache day after day nuance instead of like a daily chronic headache. He gets a brief work up initially, which is normal. And about four months later he starts having this problem where he can't read. He can write. He can understand people. He can speak normally. He just cannot read written words. He can't even recognize the letters one by one. And otherwise, he's totally normal. His mental status is normal. And it gets so bad that he has to have all of his schoolwork transcribed into audio so that he can hear the instructions for his tests. His exam, I won't belabor, but is completely normal. He had formal visual fields done, which were normal. He had a neurologic exam, which again just showed alexia or inability to read any written words. He couldn't even recognize letters. Everything else was normal. His hierarchical functioning tests were completely normal. So the big question was, is this even possible? Could you even have a lesion in the brain or something that could just take out your ability to read, but you can still write? So this patient has alexia, which we define as a deficit in word reading. Usually there's a preservation of letter reading, so you can still recognize each individual letter, but if it's severe, then early on, you might not even be able to do that. It's not caused by a field cut, and it's acquired as opposed to the dyslexia that we often encounter in developmental abnormalities as kids. So in trying to think about alexia, it's probably first described by Carl Wernicke in the 1870s. As you know, with Wernicke's aphasia, there's a pretty severe language deficit. You can't speak normally. You can't understand anyone. You can't read. You can't write. So it's a pretty global problem, and alexia is just one of the many features of that. And of course, that's caused by a lesion in Wernicke's area, which controls kind of language understanding. And then subsequently, in the 1890s, Dejeurin was a French neurologist. He described a patient who had only lost the ability to read and write. And he, on autopsy, had a lesion just behind the Wernicke's area, the angular gyro, so it starts to piece together how our language is formulated. And then the next year, he presented a case of a French gentleman who, he was 68, and he started having this right-sided numbness and weakness. And then a week later, he had, you know, he was no longer able to read a single word, all the while writing and speaking very well and distinguishing the objects and people that surrounded him as well as before. He actually presented to an ophthalmologist. He thought this was primarily a visual problem that he just could not read anymore, couldn't see well enough to read. And subsequently, on neuroexam, he had a right-homonomous hemianopsia and was unable to distinguish colors in that side as well. And so this was actually Dejeurin's initial drawing. The patient went on to have another stroke. His initial insult was probably caused by a stroke. He did another one and passed away. And on autopsy, Dejeurin was able to identify where his strokes were. And he could tell from his older strokes that he had a lesion not only in the left fusiform and lingual gyri, but also in the spleenium, which is the rear part of the corpus callosum that connects the two hemispheres. And so he thought that maybe there was some interruption in the fibers between the visual cortex with the left fusiform gyrus where the language is processed. And so possibly this patient wasn't able to read because of that disconnection. And as you know, the optic radiation is passed through that area. And so he also had this right hemonymous hemianopsia. And so this later led to the cerebral disconnection theory. So Geshwin wrote a series of these disconnection syndromes. And he thought that maybe if you could unplug the visual input in both hemifields from the language areas in the left hemisphere that you could cause this symptom. But again, that usually implies that you're cutting off some of your visual field as well. And so I was looking through the literature for cases where someone could have totally normal visual field exam but still not be able to read. And out of over 100 cases, the vast majority have a visual field cut. But there were some cases where the patients had normal visual fields. And specifically this is a case where a gentleman had a stroke in his left parietal occipital cortex. No involvement of the corpus callosum and had this exact problem. He could not read normal visual fields in normal language otherwise. And then these are two cases of the exact same syndrome. On the top you can see in the left lateral fusiform, gyrosusy, post-contrast enhancing lesion. It's pretty large. And in the lower patient you see, again in the left lateral temporal area you see flare abnormalities. And then there's a couple other cases caused by various etiologies including hematomas, subcortical hematomas, and even herpes simplex encephalitis. This has even been seen with epilepsy surgery where post-operatively they will develop this exact syndrome without any visual field involvement because of the epilepsy surgery. So this has led to sort of a new theory about why this could happen. In this case series it's very interesting they took a series of patients with just pure alexia on the left and then in the middle is a series of patients with pure hemianopia and they superimposed them and subtracted the areas of their lesions and they found that there is an area which they call the visual word form area which if you lesion then patients will not be able to recognize words but everything else is intact. And so just to return to our patient we did decide to, we did obtain imaging. These are diffusion images which are looking for ischemia or infarction and they're totally normal. Here are coronal T2 images showing the left fusiform gyrus which is normal, there's no vision. And again T2 flare axial images again show no abnormalities in the area of interest. And then here is a sagittal T1 just to show that the corpus callosum was not involved as well. So in summary we were unclear about the diagnosis but we actually found the patient texting on his phone at the end of the clinic visit and so we realized that this was probably less structural and more behavioral in etiology and so our recommendations were just no smartphone privileges until he felt better. But I thought this was a good case just to kind of go through the possibility of this as a presenting symptom and so most importantly alexia without a graphia or hemianopsia. It's rare but it's a described syndrome so it is possible and these patients may present first ophthalmology as that index case from the 1890s did and maybe thought to be a visual problem at first. A lesion of the visual word form area can produce this symptom and that's kind of a new thought that there's actually a locus for recognition of words and letters. And finally neuroimaging is necessary to rule out an organic etiology. So that's it.