 Our last presentation is from Patrick Nicholson. He's one of the neurology residents that get to come spend time with the Neuro-Athemology group here. He's in his last year of residency and originally from Logan, Utah. He's going to be speaking to us about central serous resinopathy versus optic neuritis. And he said that he had wanted it to be more of an unknown, but since it was already printed out here, it's not so much when you don't know. But OK, thanks. Great. All right, thanks for that introduction. So again, I'm from the Department of Neurology, so it's been an adjustment in a little bit of a new world here at the Moran Eye Center for the last several weeks. They've been a lot of great learning opportunities in neuro-arthemology. But as it's an adjustment, hopefully, I don't mix up my dexterous from my sinister eye and confuse everyone. But I'd like to present a case that we saw in neuro-arthemology of unexplained vision loss and implications when treating the unknown. So just a brief outline. Again, I'll present the case and then I'll discuss a treatment course that was implemented actually before the patient came for evaluation at the Moran Eye Center and then the sort of impacts that had on the case outcome. So this is a 35-year-old man who presented to an outside ophthalmologist with the acute onset of diminished vision in the left eye. He had actually woken up in the morning, had had normal vision the night before, and woke up to find that when he would close his right eye, look out of his left eye, things were blurry. And darker is what he reported. Of note, he did not report any eye pain, did not have any double vision. And he did have some abnormal kind of coloration tinting phenomena, which he described as when he was looking at a white background with that left eye, he would see kind of a purple or greenish tint or coloration. So just a little more patient background, otherwise very much mostly healthy 35-year-old male, some hyperlipidemia, but he was on no medications, no family history of visual problems, or himself any past history, and no family history of autoimmune disease or multiple sclerosis. He did not report any drug use. So on the records that we were provided and on outside evaluation, on exam he'd been noted to have 20-20 vision in the right eye and a slightly decreased 20-30 in the left eye. It was clearly documented that he did not have any evidence of an afferent pupillary defect. The provider noted that he had some trace cells in the left anterior chamber. But there was no disc edema on fundoscopic exam. The margins appeared clear and no evidence of swelling. He did, so formal visual field testing showed a small area of central visual field defect in the left eye with Humphrey visual fields. But retinal nerve fiber layer OCTs were done and they were normal. And of note in the evaluation, the provider noted that the patient was observing kind of this time a pink tinting to a white background when being evaluated. So maybe not a whole lot to hang one's hat on but a treatment plan was kind of developed by the on the outside for a presumed left optic neuritis. The provider had this patient with kind of otherwise perhaps unexplained vision loss and kind of and developed a plan to treat with this in mind. Because of the trace cells in the left eye, there was also some documented concern for iridus. So that influenced the treatment plan as well. Before doing that actually an MRI brain was obtained and orbits and that was normal. But for the treatment, the patient was started on systemic steroids with an IV burst of solumedrol and subsequent oral taper for presumed optic neuritis and then topical steroids to the left eye for this perhaps iridus. So the patient was then seen by us approximately two weeks later. So in the interval history with the implementation of these treatments, he had actually really experienced no significant improvement in his symptoms. Still kind of continued to complain of the same blurry and darker vision in the left eye and abnormal color phenomena. So now he arrives at the neuro-ophthalmology clinic and on exam he was actually 2020 in both eyes for visual acuity. His pupils were very small, reactive, little hard to evaluate because of their small size but there was possibly a less than 0.3 log unit left APD. Fields were full to confrontation. His motility was normal. He did have some central distortion in the left eye with Amsler grid testing. But the fundi did on our evaluation also appear normal with cup to disc ratio of 0.7 bilaterally and no clear evidence of optic disc edema. Our formal visual field testing with Humphrey visual fields was normal. So whereas on the outside he had previously had a very kind of small central defect on the left, this was not present on our evaluation. The retinal nerve fiber layer OCT showed normal, young appearing robust thickness of the nerve fiber layer but no substantial swelling or substantially increased thickness. So at this point we are kind of maybe in a similar position to where the outside provider had been. Not a whole lot to go on, but because of some of the tinting and the color abnormalities that he was experiencing that raised some question of if possible, could this be a retinal problem and macular OCTs were obtained. And this got us perhaps a little more on track. So in the left eye we can see this fluid inclusion, sub retinal fluid inclusion that pointed us in the direction of central serous, central serous choreo retinopathy. So in reviewing this case and kind of going over it it was actually CSCR really provided a good explanation for his symptoms and presentation. Certainly he was also the correct demographic, young male and as you know with the high stress postulated to potentially play a role which I didn't mention in the initial HPI but certainly he had that actually as part of his history and had been very busy with his employment and somewhat stressed. And perhaps unfortunately but CSCR as you know was likely quite possibly perhaps even likely exacerbated by the steroid treatments that had been undertaken. So certainly over the course of the two weeks before he presented to us had not had improvement in his symptoms. And then not exactly what we were expecting but unfortunately it appeared that he'd had another adverse outcome from the treatment that had been implemented. His intraocular pressures were 22 on the left, 51, or sorry, 22 on the right. I did what I said I would do, 51 on the left. I think we can go beyond likely. Yeah. So scratch that secondary to topical steroids. So needless to say the steroids were tapered. He was started on Combegan and Latana Prost. And fortunately it returned for one week follow up and intraocular pressures had normalized. Also at one week follow up the macular OCTs were repeated and as you can see this is the baseline. This is the one that I had already showed when he first came in and this is one week later. There had been substantial improvement in the central cirrus corioretinopathy. And this sort of shows the relative change in thickness from the first macular OCT to the follow up here. So clear improvement in that. And also the patient reported not complete resolution of his symptoms but definitely improvement that he'd experienced during that week. So to kind of bring this together a little bit as a take home message, we know from optic neuritis and the kind of landmark study of the optic neuritis treatment trial that there's benefit to high dose steroids in optic neuritis. They influence the speedy recovery of the visual deficits. And also there's a little bit of a take home message and also there's the evidence shows that they may reduce the risk of further demyelinating events or progression to multiple sclerosis. So certainly that has been something that really influences treatment in optic neuritis but I think as this case shows it's important to, there are certain implications for that treatment that certainly have adverse effects beyond kind of what we even normally think of with steroids. And I think in kind of reviewing this case just for my education and just things that might be gleaned from it are certain points that probably should have suggested against optic neuritis. And those were the lack of relative different pupillary defect which would normally be seen and no eye pain which I didn't actually know just how quite sensitive the presence of eye pain is for optic neuritis. So in the optic neuritis treatment trial 92% which I found pretty high had eye pain and subsequent studies in optic neuritis have shown that really over 90% of patients with optic neuritis will complain of eye pain. So although it's not 100%, the lack of eye pain certainly would be suspicious in this scenario. And then the kind of maybe more positive phenomenon or abnormal colorations that seem to kind of vary in terms of his color tinting perception of the white background was for us perhaps the feature that led us to also consider a retinal process or want to get a better look at the macula which then ultimately helped with the final diagnosis. So that is kind of what I wanted to emphasize from that particular case but again it was a good learning case for me and something that I will see in neurology with optic neuritis and an entity well known to ophthalmologists that was very new for me with central cirrus choreoretinopathy. So thank you. Yes? So I'll defer to Paul on this because he obviously knows a lot more and I do about it but it's interesting from what I've been able to gather that this is a disease in which we pretty don't have a good idea of the overall incidence. You don't have a good idea how many people are out there that are essentially minimally or to them asymptomatic. There's not enough to bother them. And the reason that's come along is that you know we'll do an OCT or what kind of pathology and one eye, ooh there's a little central cirrus in the air that goes a little funny and some things like that. And so I just submit because that's the case that our concept that this is the hard driving professional you know yuppie type who's more commonly might it not be the ones who are most likely to complain OCD event like in general. I just don't think we've verified that's a necessary risk factor we just see it more commonly and what we need to do and to my knowledge we haven't done yet Paul I'm wrong again but I've not seen a study in which we just take you know a population of 30 or 40 and just do an OCT of hundreds of thousands of everybody and see you know what is the overall incidence how many people are asymptomatic. But I've certainly seen plenty just from my viewpoint where I picked up a little obvious little central cirrus and frankly the face said oh I've never really noticed it. The measure may be a little something at that point but because people are used to by not their vision in the end as long as it's not too often unless they're checking it they don't really notice if there's something off where I think more OCD people are likely to think of. Paul I'm right it's possible I mean I think that there's still at least some written people there's still a little bit of association with stress and it's thought to be cortisol levels and so it's still going to be tied into steroids so it's hard to say but we see more another way that we evaluate these patients with central cirrus which I didn't see which I would recommend once you've diagnosed is to see where the leak is and decide how you may want to treat it and also auto fluorescence can be very good because you can see patches there's a lot of people out there with chronic central cirrus and a lot of patches where you can see where they've had episodes that were not in the fovea and you can see that there's something there's a whole group of chronic central cirrus but the point I wanted to ask you is if you were getting an OCT or we'll talk to the outside doctor if you're getting an OCT a nerve fiber layer OCT would not be my first choice I'm not even sure if an optomethoritis would be seen there if you're picking up glaucoma at least in the retina world we would be getting a regular OCT we'd get it on almost anything do you think about it yet? yeah and a nerve fiber layer is pretty low yield a macular one clearly whichever the outside doctor had an OCT just ordered the wrong test and if they had done that $120 test they would have avoided everything you know a multi-thousand dollar worker and actually get the worst yeah it did the one thing about an OCT is it makes the central cirrus pretty straight any qualified person says that's not normal oh there's this one here's the point so I predict the way technology is going within a decade you're going to have an app that will do an OCT I'm just going to make three quick points so Patrick advertised these visual fields as being normal they're not the one thing that's abnormal is the foveal threshold over here in the left eye and that's his symptomatic eye so but the problem is is that most of us turn to the threshold pot especially if you're in a busy community clinic you just turn it off to make the test faster and you would have completely missed the problem if you had your foveal threshold turned off this is a mistake that I see a lot of people make especially if you're examining a patient with central complaints you want to have the foveal threshold turned on and then Patrick if you could go to the the things that said it's not optic neuritis and the one thing that I would add here is that the MRI was normal and I think there was a time when MRI scanning wasn't good enough to pick up a mild case of optic neuritis like this would be and there's also radiologists that want this mild optic neuritis if they're not looking for it there specifically especially if they weren't given a proper history which is often the case which is why we encourage people to go over the MRI with your radiologist make sure they understand what the problem is make sure they understand what you're looking for but this clearly was a normal MRI and then you know to Aileen's point which I think is really important and that is Patrick had mentioned that if you get IV steroids in the patient with optic neuritis they get better quicker and he also mentioned that they have a lower risk of developing MS but that risk is only decreased for two years and I think that's only true if you have at least one other white matter lesion in your brain which this patient did not have so actually there was zero benefit of giving this guy IV steroids even if he had optic neuritis unless he was having a lot of eye pain or a very decreased vision it was really the wrong thing to do and my exacerbated his underlying problem is something we say yeah James just had a question would it have been useful to have color vision planes and or red cap desaturation because that can kind of at least I think sometimes point to you in the direction of optic nerve versus retina and I usually like to use those and see if it's a positive red cap desaturation I'm much more concerned that it is the nerve or a positive you know decreased issue heart planes that have been helpful in the initial I think in differentiating optic neuritis I think I'm guessing that both would have been decreased I'm not sure that would help a flicker fusion might have helped most of us don't have that in our clinic but even a flicker fusion can be decreased by a little problem deciding whether it's optic nerve or retina is such a fundamental part of what we do in their ophthalmology clinic and there's never one test you can have on it's doing it's looking at the yeah one other question actually from Paul on the retina as I've always been puzzled by this the association with stress and pursuers retinopathy I just maybe it's just me and I should never say never if I ask a patient are you under stress and if you're under stress now I'll say yes yeah are you under stress oh thanks thanks thanks for sharing I agree with that I do remember why this guy came in with a plastic surface surgery absolutely tonight never having stress in his life and if you really didn't care they believe that it's actually how you respond to the stress on your type of personality more than the amount of stress you have in your life the thing that people that internalize stress more and push harder when they're stressed tend to have higher levels of cortosteroids in their serum and also tend to have higher adrenal responses both of which experimentally cause CSR and you can actually cause an experimental CSR by injecting somebody with a bit of epinephrine epinephrine and so they do that in mouse models quite through the whole thing they think that that has a high association and we also share this through other studies where people think it has to see whether there's some pathogenetics that cause a central serous so the thing it has to do a lot with how you respond to the stress not necessarily how much stress you're under I might ask that in asking that question about under stress you might ask them how they have responded to a situation that we would normally find for stress thank you