 Our last presenter of the day is Reece Feist. He's going to be talking to us about a forest in the consults. All right. So this is actually going to count as one of my neuro-grain rounds. I talked to Dr. Warner. So just make sure you mark that off when you get back upstairs. So this was a consult request. So actually, Renee was covering consults, but he was busy studying North Carolina macular dystrophy. So he asked me to see the patient instead. So he got called by the neurology service, about a 50-year-old guy who was admitted for aseptic meningitis. And so their specific request was we need a F-8 evaluate for vasculitis. So this gentleman was transferred from an outside hospital a few days before for these recurrent episodes of aseptic meningitis. This was his third episode in about a year. He initially presented to the outside hospital this most recent time after developing a fever, feeling shaky and weak. At the outside hospital, he was found to have an INR of 4.1 and a subdural hemorrhage. So he was given vitamin K and FFP and then transferred here. So his pathematical history, it turns out he's got a history of factor 5-Lide. And we're not sure if he was like homozygous, heterozygous for it. We don't have any of his other records. But he was anti-coagulated for it. He had a history of a myocardial infarction at a young age. He's got hypertension, hyperlipidemia. He also had mesenteric artery thrombosis and has had a stint in his superior mesenteric artery, it looks like. He's got depression and then BPH. So he had a cardiac bypass, quadruple bypass at a young age, the SMA stint, and then also had an incisional hernia repair. Otherwise, he denied any pastocular history. So he's a current smoker, kind of varies, maybe two to three packs per day for the past 20 years. So he drinks about two beers a month and then, unfortunately, he was spiced for about four months, about four years ago, but got clean after that. He still lives with his parents. He's a former welder and metal artist. No international travel. Like Brian Stagg, he's never left Utah his entire life. He used to have a dog as a child, but no cats. He denies any IV drug use for any sexually transmitted diseases. The only drug use he admitted to is a spice a few years ago. Family history of heart attack and his father, but he seemed to be a little bit older age. And then maternal grandparents both had heart attacks in their 60s. So when I saw him, he was kind of confused, but was able to talk to him. He was appropriate, but just kind of forgetful about things. But really the only things he reported to having were some headache and then some itchy skin. Otherwise, he denied everything else that I asked him in terms of uveitis stuff. His initial exam show, he was fevering 102.9, but then, otherwise, hemodynamically stable. He was thin, kind of disheveled, kind of sweaty. His physical exam, the general physical exam, wasn't otherwise too remarkable. He was alert and oriented to person place in the year. But kind of missing out on kind of minor details about his past medical history. The neurology service when they evaluated and felt that he had a little bit of clonus in his left ankle. But otherwise, they weren't picking up too, too much. So like everybody that comes through the ED, he got a CT head. It showed these kind of diffuse foci of intraparenchymal calcifications that they were thinking could be a sequela of prior grainy lumbitus process. So when he first came in, their first thought was for something like neurocysticercosis. Then they also noted a lot of volume loss, more than they would have expected, and then the small frontal subdural hematoma, maybe. So these were mostly posterior. And then I'll show you the MRI later on. So he got a lumbar puncture and had a ton of white cells in his CSF. Mostly neutrophils. His glucose was a touch load, but not horribly so. His IgG was elevated, albumin was elevated, total protein was elevated. There is no growth on the bacterial cultures, and then the HSV PCR was negative. So when we saw him, he needs some glasses, but his best corrected visual acuity was like 20, 20 minus 2 in both eyes. Pressure is normal, no APD, and full confrontational fields and extracurricular movements. And then you can see down here, I noted that he was also confused. He had a lot of really bad dry eye and then some mild cataracts. But otherwise, when I looked in, we didn't really see anything else. He didn't have any cell in the anterior chamber, no vitreous cell. His optic nerves looked normal. And then his vessels looked pretty unremarkable. We didn't see any sheathing. There's no snowballs or snow banking out in the periphery either. So we did what they asked, and ended up getting a fluorescein. So the early phases were really unremarkable. But then on these peripheral sweeps, you can see these changes out in the periphery. So actually, it wasn't too dramatic, but I looked at these with Dr. Vitali right after we got him just to kind of everything by him. And he agreed that these were abnormal. So the initial differential was just kind of thinking in broad categories. So some kind of vascular events. So hypertensive retinopathy, Udall's disease, Susax disease, didn't really look all that much like Susax or anything. When you're looking at uveitis patients, you always have the infectious stuff on the list. Other neoplastic processes, so lymphoma leukemia, autoimmune conditions like Bechette, sarcoid, VKH, intermediate uveitis, or lupus, didn't really look like anything congenital. I didn't really expect to see anything like that after a traumatic episode, but he did have a fall down the stairs a few weeks ago and that's what gave him the subdural. Things like diabetic retinopathy, you could get some really far peripheral changes or just idiopathic. So his labs came back. So his CBC and BMP were both unremarkable. They drew a cystocercosis IgG, which came back negative. Annie Smith antibody, Joe One antibodies, SSA, SSB were negative. His ankle panel was negative and his aquaporn four was negative. So he did have some positive labs. So his anti-double-stranded DNA came back positive. It was one to 80, so not super high, but still elevated above one to 40. His ANA was really high at one to 2,000. His sedrate was elevated. They did a serum protein electrophoresis. Total protein was a little bit elevated. His alpha one globulins were up. And then they did an anti-phospholipid antibody panel that was positive for the cardiolipin antibodies, positive for the beta-2 glycoprotein antibodies, and then he had a positive lupus anticoagulant with the dilute rustle venom viper test. So after they cleared his stents, they were finally able to get an MRI. So they noted these areas of what seemed to be kind of subacute ischemia, that they felt like corresponded more to those what they were calling calcifications on the CT scan. And then based on the distribution and the appearance of them, they felt that it could be a sequela vascularitis. So they included lupus and anti-phospholipid antibody syndrome on their differential with this. They noted a little bit of vessel wall enhancement along with some of the cortical veins, but felt like that could potentially be due to the subdural hemorrhage. So just to talk, they haven't finished working up all the infectious processes. We asked them to do a lot and they didn't do any of it. But the stuff that he had come back, they felt like it's consistent with the diagnosis of lupus with anti-phospholipid syndrome. So lupus obviously is a life-threatening multi-system autoimmune disease. Renal involvement is kind of the most common serious complication. You can get widespread arthritis that typically affects the hands and knees. Mucocutaneous involvement typically involves the classic malar rash, but then all other kinds of rashes and all other kinds of mucus membrane involvement as well. You have pleuro-pulmonary involvement, pericarditis, and then accelerated atherosclerosis is also a feature. And then there's kind of diffuse neurologic involvement. You can have aseptic meningitis, cerebral vascular disease, demyelinating syndrome, seizure, and then even things like mood disorders and psychosis or attributed to lupus. The clinical criteria is there's a ton. So this patient, he met the clinical criteria with neurologic disease. When I asked him, he did not have a rash, or when I saw him, he did not have a rash and he denied history of rash, but I guess when rheumatology taught to him and talked to his mother, they did feel like he has had a malar rash in the past. So they felt like that further supported the diagnosis as well. To make the diagnosis, you need at least one of these clinical criteria and at least one of these immunologic criteria for a total of four. And so he had a positive ANA, a positive double-stranded DNA and then a positive antiphospholipid antibody panel. So he meets the criteria for diagnosis of lupus. So what does it do to the eye? It's pretty diverse. The most common would be just care to conjunctivitis. So one of the papers I found said that it's affecting about 30% of lupus patients and they identified it as the most common manifestation of the disease, but other corneal involvement can involve, include peripheral ulcerative keratitis. So in terms of the retina, you get retinal hemorrhages, cotton wool spots, arterial or nearing, vascular tortuosity. There's a lot of different things it can do and one of the things that can make it difficult is just kind of distinguishing just classic lupus retinopathy from a hypertensive crisis in lupus patients as well. Any phospholipid antibody syndrome increases the risk of ocular and CNS vaso occlusion by about four times. In terms of other parts of the eye, so sclera and uvia, rarely cause a scleritis or episcleritis, but it's been reported. A wide variety of neurophthalmic disorders, so you can get an ischemic optic neuropathy kind of less of an inflammatory, like a true inflammatory, but you can still have damage to the axons. That way, cranial nerve six palsy, internuclear ophthalmoplegia, optic neuritis. In the orbit, they've identified myositis, vasculitis and trochleitis. And then these patients are either, or immunosuppressed for a variety of reasons, either on our doing or kind of their own. So you always have to consider other kind of infectious processes. So it's things like retinal necrosis with a herpes virus, CMV, endophthalmitis. Any phospholipid antibody syndromes, pro-thrombotic disorder that can affect both the venous and then arterial circulation. So in terms of the venous, the venous hits that people get. Usually it's a deep vein thrombosis in the legs and then the most common arterial site is in the cerebrovascular circulation. Catastrophic antiphospholipid antibody syndrome is characterized by clots in multiple small vascular beds, which leads to multi-organ failure. It's diagnosed based on positive laboratory testing that's kind of extensive, so with antibodies that persist over a period of months. Testing for beta-2 glycoprotein-1, anti-cardiolipin antibodies, or a positive lupus anticoagulant assay. It's found in about 30% of patients with SLE. And most of the authors I found where they wrote about eye manifestations with us didn't feel like just regular systematic screening was beneficial for most people in the absence of other stigmata of the disease. So the retinopathy in lupus, like I talked about, the most common findings would be cotton wool spot, hemorrhages, and then optic dyskidema. The retinal vascular changes are thought to stem from fibrinoid degeneration with necrosis of the vessel wall, often in the absence of inflammation, both kind of on a pathologic basis and on a clinical basis. Which kind of fits with what this gentleman had. So no cell, no vitreous findings at all. So you can, when you have these kind of changes on the FAA and the absence of the inflammation, that's one thing that you need to consider. You can develop a severe occlusive retinopathy, but this is definitely more rare. But this is really what causes a lot of the vision loss in the patients that have kind of the sequelae from this. Most of the authors suggested immunomodulatory therapy. And then as the retinopathy that we're identifying is usually associated with renal changes. And one of the studies found about, maybe 50% of the patients with just diagnosed lupus without diagnosed ocular involvement had renal dysfunction. But it was about 100% in patients where they had eye findings as well. So really serious condition. Treatment is not always aimed at the eye, but just protecting the rest of the body. Any coagulation and any platelet therapy are used with variable success in treating the occlusive disease. So just to talk a little bit, this guy came in as been like just over a week, so not too long. But they started him on IV solumedron and gave him a pulse of that. And then we're gonna start him on cyclophosphamide. He's already been anticoagulated with warfarin, given his history of factor five, Lydon. And so they were planning to continue that. And I'm not sure if they've discussed doing any anti-platelet therapy, but that would be one thing to consider. Fortunately, at this time, looking at his FA with Dr. Vitale when he first came in, it was relatively mild and not felt to have anything vision-threatening. So it was good from an eye standpoint. But I think this was one of the kind of the rear cases where we've been able to help with the fluorescein. I know Eileen talked about this at Resident Alumni Day the other day. And the yield is pretty low, but this is one of those things where it can be helpful and one we don't see a lot of. So, Dr. Shkorn. That for the last picture we wanna see is on cyclophosphamide. Yeah, the fact is we don't have a normative. Database of patients of a whitefield anti-platelet therapy. And the fact remains that they have been studying that Dr. Shkorn has normal patients, have normal vasculogen periphery, and this can represent all kinds of things. So is this truly a lupus vascular? So he's coming to see you in a couple of weeks, so that'll be excellent. Yeah, and that's when I first saw this, I didn't know quite what to make of it, but the guy wasn't diabetic, his blood pressure's been normal, so we didn't have another explanation for it. And it was more than kind of what we've expected to see, because we do a lot of these fluorescein consults actually on some of these consult patients, and most of them are stone-cooled normal. They've got just like one little vessel that's lighting up just way, way out in the far periphery, but this was a little bit more than we expected, and that's what we ended up talking to Dr. Vitale about, and it wasn't vision concerning, but it was definitely more than we thought we were gonna see, so. Yeah. If any of the residents experience angiography and see everybody in this room, and see how many have abnormalities. Yeah. So I saw something like actually in one of the most recent shoes of ophthalmology, where they did do white fill and a bunch of normals, and I do think that's how we found non-perfusion. I'm not sure about which, or that has been reported before. Non-perfusion has to be proven, and if you know everybody with you, you have heterozygous or fever. Well, you were talking about normals. All right, in normals, like non-perfusions, I guess they published a substantial number of normals had non-perferal, non-perfusion, but I was just wondering about like what is not a leakage? Yeah, and I think that study in ophthalmology too was trying to come up with like a, you know, one of those like OCD kind of lines to say this is where you expect 95% of people to have normal flow, and then beyond this point, it's not unremarkable to have that. So I didn't see where the, yeah, like you said, I didn't see where they had any changes in terms of the leakage too. I think it was all the non-perfusion stuff. Super interesting case. Thank you.