 Okay, so, I think you all know me, I'm the Neuroloftomology Fellow. I was a little ambitious when I first made this title, so I'm gonna have to change it just a bit and add that it's just going to be Neurologic Accompaniment to an Autonomic Disease. So our patient presented with a chief complaint of, I think I have a floater, and she's a 24-year-old graduate student in Manny. We already knew her well because she had just had a five-day history of loss of vision in the left upper quadrant of both eyes. She came in from triage and she had been in the ER. She had gotten an MRI scan for this, and this was about three days prior to her new chief complaint, and basically she was describing almost a kaleidoscope effect in the left upper quadrant of both eyes with loss of vision. So her MRI scan at that time was normal, no sign of infarction, and she had a history of migraine, so that's what we thought was going on, but then, much to my dismay, she calls and says I have a new problem, and this is a white spot with a blue outline that I see just in my left eye, and I think it's a floater, but it doesn't float. Her past medical history, as I said, she has a history of migraine since 2003, although she's never had a headache, so her migraines currently are occurring about once a week, and she gets basically loss of central vision, and then it spreads to take away all her vision, and then she gets tingling on the left side of her body, and then sometimes she has trouble speaking, and this whole process takes about 30 minutes. And then she also has frequent nausea, not necessarily associated with these migranous spells. She has trouble with panic attacks, and her sister was actually killed in a tsunami in Thailand in 2004, and her mother died from CNS lymphoma at age 40, and so she has a lot of stress and panic attacks, sort of PTSD. She was started on topomax and aspirin three months ago by a neurologist who was trying to control her migranous spells, and in evaluation for these migranous spells, she had an echocardiogram, which showed the patent frame and ovale, which was closed, and then after that, she started having the spells three times a week rather than once a week, so she had to get revision of that, and ever since, she's had shortness of breath with irregular heartbeats occurring several times a day. Other than that, she takes max salt as needed for her migraines, and she doesn't use alcohol tobacco, well, she rarely uses alcohol, no tobacco or drugs. On examination, her visual acuity was 2020, her people exam was normal, motility, muscle balance were normal, pressure was normal, and this is what she drew on the Amsler grid as her spot. She said that basically within this area here, she can't see any of the lines, it's sort of just blighted out, and then it has like a blue outline. This is her visual field test from the few days prior, and she says that seems just exactly the same as it seems at all. And this is her funness exam, and it looks pretty normal on this photograph, but in real life, there was a little spot right around here, and I can show you some more pictures where you might believe me. And on red free, she didn't have any changes to her funness, but this is her infrared, and you can kind of see that spot. And we did the infrared just because we got an OCT, but we happened to get the infrared image. So, here's her OCT, and at first it's like, okay, no fluid, but then if you're looking right here, there's an abnormality in the outer retina, and on zooming in, you can kind of appreciate that better. So I think this is like the inner outer segment, and then I think this is the outer nuclear layer. Correct me if I'm wrong, retina people, but. So our impression is we have this 24-year-old woman who has a persistent visual aura without infarct, and a new photopsy on the left eye, which corresponds perfectly to this macular lesion. So we had gotten the MRI scan, and it had diffusion-weighted images, but we hadn't really imaged her vessels, so we decided, because of this new lesion in the macula and the possibility of maybe ischemia, that we'll just go ahead and image her vessels in her brain. So we did that, and then we started thinking, maybe something like some unusual presentation of ampy, just because ampy is well-known to cause cerebral vasculopathy, and retina changes, so we were thinking about that. So when she went to get her imaging, I read up on ampy, and basically the pathophysiology isn't known, but it's thought that it could be due to some kind of infectious process that causes some kind of hypersensitivity reaction that causes some inflammation in the coroid, and maybe a partial corridor infarction, and then changes in the overlying RPE. And we all know that ampy occurs most common in white young adults, and it occurs equally in both sexes, and they often are preceded by a flu-like illness, and they often have changes in their central or paracentral vision. So, and on exam, generally with ampy, you see sort of discreet, multiple-round subretinal lesions, like in this picture, and this is in the acute phase, and in the chronic phase, you can see changes in RPE, coarser, or fine granular pigmentation. I didn't really remember the other ocular findings that can occur with ampy, like, of course, well, anterior and posterior chambers cell, but also corneal melting, subepithelial infiltrate, episcleritis, papillitis, optic neuritis, retinal vasculitis, engorgement of the retinal veins, and even vein occlusions have been reported. And the most common neurologic complications with ampy have been headache and CSF pleocytosis, but rarely there can be a cerebral vasculopathy, which we all hear about, and that can sometimes cause a stroke. And it can generally occurs either simultaneously with the diagnosis of ampy, or a few weeks after the diagnosis of ampy. So, in our patient, that was sort of backwards, because she had the brain finding the homonymous defect first. And it can affect the small or large arteries in the brain, and it can also cause a venous thrombosis. And there's been one case report of someone who had subacute sclerosis panencephalitis preceded by ampy. And so, in this literature review that was published in the Journal of Neuroophthalmology, they looked at 19 published cases of ampy associated with cerebral vasculitis, or venous thrombosis. And the most common symptoms of patients with cerebral vasculitis or venous thrombosis were headache, weakness, or hemiparesis, homonymous hemianopia, which is sort of what our patient had, numbness, seizure, and less common symptoms were aphasia, dysarthria, ataxia, stupor, slurred speech, and nystagmus. So, that same literature review actually looked at follow-up of patients with neurologic complications from ampy, and six were eventually better, completely, five had persistence of their neurologic defects, and then four had either progression of their defects or relapse when their drugs, their immune suppressant drugs were tapered, and three actually died. And those patients were young men, age 23, 24, and 25, who had one patient presented with headaches and seizure and didn't have any neuroimaging and didn't have any treatment, and then one patient presented with infarction of his MCA, received oral prednisone and died from cerebral herniation, and then one patient had infarction of the PCA, and also had an oral prednisone and died. And so the main recommendations for patients with ampy and neurologic symptoms or headaches would be a brain MRI with diffusion-weighted images, which our patient already had. And treatment based on the severity of their clinical presentation, so it's corticosteroids if they just have headaches or other immune suppressive agents for patients with imaging evidence of cerebral vasculopathy. So I was all ready to look at our patients' MRI and see if she had a vasculitis and considered treatment, but luckily for her, her MR, AMRV were all completely normal and again, her MRI showed no evidence of infarct on diffusion-weighted images, so good for her, but we still didn't really know what was going on. And she had a hypercoagulable workup that was all normal. Oh, so at this point, we decided to get the FA, and so this is for Dr. Bernstein. We got her autofluorescence first, which I've learned to do, but unfortunately, it wasn't very helpful. So we got a fluorescein angiogram, and again, this was completely normal, and as was the ICG. And we still had one test pinning, which was serial ansler grids, which we like to do, and basically her spot didn't change. So, does anyone have any thoughts about what could be going on? We came up with something, so I showed Dr. Vitale, and so we think that probably she has acute macular neuroretinopathy, and this was first described by Boss and Dewman in 1975. They thought that the lesions were affecting the inner retina, so that's why they called it neuroretinopathy. It's rare, it can cause permanent or transient visual impairment, and it can be either unilateral or bilateral. And generally, the lesions are petaloid, and they make a flower shape around the fovea. They're flat, they're well circumscribed. They can be brown, red, or even purple, depending on the pigmentation of the patient's fundus. And they're wedge-shaped. Oh, and they tend to correspond exactly with the patient's ansler grid. And they're most best seen on infrared imaging, so here's a patient from just last month in the Archives of Ophthalmology, and you can't really see too much on the fundus exam, but on infrared, it really stands out. And it tends to show decreased cone responses on multifocal ERG, which kind of indicates involvement of the photoreceptors, and so this is just an example from 2009 in the Survey of Ophthalmology. And on OCT, it tends to show involvement, just like our patient, of the inner outer segment and thinning of the outer nuclear layer. Oh, and so because of that involvement, some people have proposed to change the name to acute macular outer retinopathy, or a more, which means love in Spanish. So, and then this is just a picture showing resolution of the changes in the inner outer segment over time improvement, but maintaining some thinning of the outer nuclear layer. And that's from Ratna from 2011, and this is as well. And this shows changes in the infrared over time. This is at baseline one month and three months, and it just shows that the defect lessens a little bit. So AMN has been described, this is from a review of 41 cases from 1975 to 2002, and about 80% were found in women, and about 50% of the women were using oral contraceptives, which may or may not be important, but and then it was associated with epinephrine about 10% of the time, but about 15% of the time involved epinephrine plus some shock, clinical shock, and then rarely like 5% of the cases involved trauma or iodine contrast media. And interestingly for our patients, 7% of the cases were associated with headache or migraine headache with or without aura. So our patient had the aura and she'd been using her max salt almost every day for like five days, which has a sympathetic effect similar to epinephrine, so we're not sure if that was significant or not. So we do all this research and everything, but unfortunately for our patient, it doesn't really help with her treatment because there's limited follow up documented and we know that defects can get less noticeable with time and sometimes they're permanent, sometimes they're transient, and we think there's persistent of the thinning of the outer nuclear layer, but treatment isn't really known, but it's still good to learn about it and also because it's really easily identified, I think, on our OCT. So maybe with more cases identified, we can figure out what's going on systemically. So any comments or questions? Yeah, no run. Oh, no, I didn't read about like any persistent homonymous defects, just the fact that there was a paper from 1975 that talked about ORAS and associated with AMN, but I actually wasn't able to pull that article yet, so yeah, I don't know about like permanent homonymous defect or anything with AMN. She did, she had a closure and then she had a revision of her closure. No, her migraines got worse after the closure of the first time. They went from once a week to three times a week and then after the second time, they went back to once a week. Yeah, all ORAS, no headache. Oh, yeah, I don't, I don't... Oh, there you are. Any of the retina people have...