 So I'm Chris Baer, one of the first residents here. I'm on the neurophthalmology rotation right now, so this seems like a good opportunity to present kind of a neurophthalmology-oriented case. So we're gonna look at a patient who presented with bilateral vision loss, a company with a headache. So I saw this patient on call in the emergency room. In brief, she's a 64-year-old woman with a history of headache and bilateral vision loss. She developed a severe headache while overseas. She described this headache as very sudden onset, very high intensity, unlike anything she'd ever had before. She went to the hospital there, very reasonable. She had an MRI that was normal, and the rest of her work up there was normal. She came back to the States, but her headache persisted. It wasn't quite as intense as it had been, but it was still persistent. And about 10 days later, she noticed a decrease in her vision. She went to the hospital, another hospital, here in the state. She had a CTA of her head and neck, which was unremarkable. Labs showed a mildly elevated ESR, and so she was discharged on prednisone with plan to follow up with her ophthalmologist to get a temporal artery biopsy due to concern for temporal arteritis. After starting the steroids for vision, actually continued to decline, and so she came to the university hospital, which is where I saw her. She had no GCA symptoms, no jaw claudication, weight loss, muscle weakness, any of GCA symptoms. And just interestingly, her blood pressure had been in the 140s to 150s systolic. She said she was your runs in the 90s, so it was a bit of a departure for her. Her past medical history was unremarkable, specifically no history of headaches from migraines, and her eye failure, she was notable for retinal tear in her left eye that she'd had fixed a couple years prior. No medications, no allergies, no family history, and no use of alcohol, tobacco, or recreational drugs. So on exam, she was hand motion in both eyes. Pupils were briskly reactive, there was no APD, her motility was full, her pressure was normal, and the rest of her exam was really fairly unremarkable. We saw laser in around a tear in her left eye that looked well sealed, but otherwise very unremarkable. And so, thinking about our differential at this point, there are a lot of things that can cause headaches, and there are things that can cause vision loss, but the things that combine the two, especially with headaches that are this severe, the list gets a little bit smaller. And things that we need to be thinking about, obviously when someone presents with a big, big severe headache like this, intracranial hemorrhage is obviously one thing that pops right to the top of our list. GCA is certainly something we could think about. Our CVS and PRAS are something we're gonna talk a little bit more about here in a few minutes. Vascularitis can also cause headache and vision loss like this. Much less likely, or migraine, especially without any previous history, clots, again, less likely, given no significant other risk factors for that. So this patient needs imaging, and so we were able to get that in the emergency room, and a couple of important things to note, it's not subtle here, you can see heroccipital lobes, these areas of pretty large infarction of both of heroccipital lobes are kind of what we're drawn to here. Over on the far right-hand side, we have vessel imaging, this is an MRA of her brain, and I don't know if we have a pointer here, let me see if I can just show you over here, maybe not, I'll just describe it to you. So we see some kind of subtle narrowing of her PCA arteries right here and here in this MRA image, and we also, not shown here, but we also had narrowing of her branches of her MCA as well, bilaterally. And so, she was admitted to the hospital with a little bit more workup, she initially had an elevated ANA, this was repeated and was later turned out to be a repeat negative. Her ESR I mean mildly elevated at 33, the remainder of her workup was negative, and she even had specific vessel wall MRI, which was read as normal, there was no signs of vascularitis or other inflammatory conditions on that vessel MRI. And so, for treatment, her steroids were stopped, this was not consistent with any kind of GCA diagnosis, she was started on nemotopy, which is a calcium channel blocker, and over the course of a few days that resolution of her headache. And so, this is a case that certainly has features that are consistent with RCVS and press, maybe a little bit less likely to see an espaculitis, but I thought this was a good opportunity to talk about RCVS and press, this is a, these are conditions that we don't necessarily talk too much about in our general ophthalmology world, but these patients can present to our clinic, and it's a good opportunity to really delve into this a little bit more and just review this, for not only for ourselves for boards, but also for clinical practice as well. So, RCVS is reversible cerebral vascular constriction syndrome, and this is one of those syndromes that's very, very aptly named. It's an encompassing term that encompasses a lot of, kind of a collection of different entities that were previously separate things as postpartum angiopathy, benign cerebral angiopathy. They're now kind of under this umbrella of this RCVS syndrome. The pathophysiology involves transient, reversible, narrowing of cerebral vasculature, and it's unclear what the precipitating event is in these. Some people have speculated that headaches will result in vascular narrowing. Some people say that it's the vascular narrowing that comes first, and so people are not necessarily sure, but we do see this transient, reversible narrowing of the cerebral vasculature. Women are commonly more affected than men. There's no really significant predilection for age or race in the literature. Risk factors for this can include things like pregnancy, vasoconstrictive drugs, specifically things like cocaine, heroin, marijuana's been implicated, SSRIs are another common implicating factor as well. If you have thrombosis, that can do it, and then migraine is also a risk factor as well. It's important to know that these are associated risk factors. Not a lot has been shown to prove causation of these things. The clinical presentation, typically, folks present with a sudden onset, very severe headache, unlike anything they've ever had before, folks who have migraines and other types of headaches note a very clear distinction between this type of headache and their typical migraine headaches. Typically, they're located occipitally and they can be associated with nausea and photophobia. They'll typically have periods of these intense pain followed by areas of relief. And so, like in our patient, her headache went down, never went completely away, but got better, and then they'll have exacerbations of this pain going back up to that severe level over the next few days to weeks. It's very rare for patients to have RCVS without a headache. Less than 10% of people in the literature have a subacute or not a very severe headache, and so that's one important diagnostic criteria here. Folk ulnar allergic symptoms can develop. Headache is the predominant symptom, it can be the only symptom in about a third to half of patients, but folk ulnar allergic symptoms can occur, specifically for us. Visual symptoms that we need to be looking out for could be things like scutomas, hemianopias, and then cortical blindness, or severely decreased vision, like we saw in our patient here. The diagnosis is made by a few different ways. Number one, we have to have a high clinical suspicion for this based on the clinical history and the presentation of this type of headache and symptoms. Laboratory testing for this is typically normal. There's really no, usually at laboratory abnormalities, including inflammatory and other labs. Imaging is important in this diagnosis. MRI is very important to look for areas of vascular narrowing, but it's important to know that in a lot of cases, up to 30 to 70% of cases, actually, their initial MRI will be normal, and we saw that in our patient here. She had an MRI on presentation when it first started that was normal, and then she also had to repeat imaging a few days later when she came here to Utah, that was also normal as well. And so neurovascular imaging is an important thing as well. So, MRA, CTA is an important tool that we can use, and the classic description when you do angiographies, you'll see this kind of sausage on a string pattern. This is really nicely highlighted up here in this picture labeled A on the far left. You can see these areas of vascular dilation and narrowing. Kind of looking like sausage on a string at a butcher shop if you've ever been to one of those places, but that's kind of the classic description right there. Here is a little bit closer up view, and you can see on the left-hand side here, these areas of vascular dilation and vascular narrowing. This is a little bit closer up picture. And then over on the right-hand side, you can see this is a reversible process. You can see the vascular shearers return back to its normal state. This is after a month later. And so the management for our CVS really resolves around supportive care. So you stop any inciting agents, stop the vasoconstrictive drugs if they're on anything, and then work on controlling blood pressure if their blood pressure is high. Now blood pressure can be a little bit tricky in this because hypertension, if the patients have hypertension, it can induce further vasoconstriction, which obviously we don't want. However, if you drop their blood pressure too low, it can result in hypotension, which can trigger ischemia. So it's kind of a fine balance to walk. Calcium channel blockers are kind of the mainstay of treatment that we use. There's a lot of evidence for things like nemotapine and verapamille as far as reducing the severity of their headaches and improving the overall symptoms. They don't necessarily impact the time course of vasoconstriction and getting back to normal vasculature, but symptomatically they can certainly improve the headaches that patients have. So it's a pretty controversial intervention. We don't use them typically, and they're certainly not our first line for this condition. And we stopped those when she came here to our hospital. And then in severe recalcitrant cases where they're having progression and significant neurologic symptoms, there's been case reports of actually doing intra arterial administration of vasodilators, which is also a little bit of a risky proposition. Your risk by doing that, intra arterial angiography certainly has its own risk, but directly infusing vasodilators also increase your risk of having a reperfusion injury in these patients. So it's a little bit tricky how to manage that. Prognosis of RCVS tends to be fairly good. Most people have resolution of their headaches and angiographic constriction within days to weeks. It is reversible condition. However, about 15 to 20% have residual deficits from strokes, and we saw that in our patient here. Most of those deficits are minor, but some can have severe deficits as we saw in our patient here. Flipping to a similar condition, it has some overlap with what we just talked about, press. So posterior reversible encephalabic syndrome is a syndrome defined by characteristic clinical features of neuro-imaging findings. As in RCVS, it's more commonly found in women than men, and it can't affect any age group. Patients as young as two have been reported, and as old as 90 have been reported throughout the literature. The pathophysiology, we see some similarities with RCVS, but there's some specific things to press when you talk about. First, there's impaired augurated auto-regulation on hypertension. If you remember back to medical school, you remember that cerebral perfusion is very tightly defined, and as systemic blood pressures rise and fall, the cerebral vasculature will constrict and dilate to maintain equal perfusion to the brain. When you get to extremes of that, you can have impaired auto-regulation that can lead to blood vessels not behaving in the ways they should. It can lead to increased pressure to the brain, which can lead to a leakage of blood products and causing a bedema in the brain. On the flip side, in other cases of press, we can see actually cerebral ischemia and vasoconstriction, which is actually a similar mechanism what we see on RCVS. And this is all highlighted by an ethyl disfunction allowing leaky and ethylion-aligned blood products to leak into the cerebral, to the brain parenchyma. Risk factors for press. So hypertension is the one we classically talk about. It's important to know not only absolute hypertension, which is an important risk factor, but also relative hypertension. So if someone runs in like our patient in the low 90s, for example, and her blood pressure suddenly spikes up to the 140s to 160s, relative hypertension can disrupt the auto-regulation, auto-regulatory mechanisms and can cause press. It's also been associated with renal disease, pregnancy, specifically folks who have preeclampsia or eclampsia. Immunosuppressive medications have been linked to it in autoimmune conditions. Things like rheumatoid arthritis, are also commonly described as risk factors for press. The clinical presentation, again, patients will present with headaches, but the headache in press tends to be a little bit more indolent than the abrupt onset kind of thunderclap headache that you see with RCVS. Patients will often present with visual disturbances and the disturbances can be similar. They can have heminobium, hallucinations, auras, and even portable blindness, like we saw. Patients will often present with seizures and altered mental status around sevelopathy. Imaging, the classic finding is posterior white matter edema. And you can see here, this is kind of a textbook picture of this edema in the posterior circulation right there. What we'll see is, you can't have involvement of other areas of the brain as well. You can't have lesions in the frontal lobes, although it's atypical to see involvement elsewhere in the brain without involvement in the posterior portion of the brain as well. On vessel imaging, vessel imaging can show vascular narrowing, similar to what we see in RCVS, which can make a little bit of diagnostic confusion then if we're not careful. Complications from press. So you can have ischemic complications and you also have intracranial hemorrhage. And this is showing press complicated by hemorrhage surrounding areas of edema right there. Hemorrhage can occur in as many as 20% of patients and it's really something that leads to a lot of morbidity in patients who have press. The management of press, number one, is controlling hypertension. And again, just as an RCVS, we have to be cognizant of how we control that hypertension. We don't want to drop them too low. We risk ischemia, but we can't leave them to high risk hemorrhage. Treatment of seizure is obviously important. Treatment of eclampsia if they're pregnant is very important. And then again, in this condition, just like an RCVS stores are not typically recommended. Steroids can negatively impact your blood pressure, making it difficult to predict where it's going to go. And so we typically don't use steroids in these cases as well. The prognosis, most cases of press are benign without lasting deficits. And it's reversible just like our CVS with symptomatic improvement, usually occurring before we see radiographic resolution of edema and things. The minority of cases, however, do have lasting neurologic deficits. And recurrence is uncommon. Thankfully, less than 10% of patients do have a recurrence. And so our patient here has features certainly of both. Her blood pressure was higher than it was in the past. She had vascular narrowing on imaging and certainly areas of infarction on imaging as well. And so the diagnosis for her is a little bit unclear, but the treatment for her either way would be the same. And this is an important thing because as ophthalmologists, patients will come in with visual deficits. And it's an important thing for us to be just cognizant of and to keep our differential because these things are diagnosed not only by imaging, but with clinical suspicion. And if we don't have that clinical suspicion, there's one thing that we could potentially miss. So this case was a good example to kind of review these conditions and just kind of refresh our memory on these things. Any questions? Great. So how common are these? This is really bizarre. A isolated, relatively brief period of focal dysautonomia. I mean, that's essentially what this is. And obviously pretty scary as you think about how this all evolves. I mean, Judith Howefin, do you see one of these cases? Do you see one of these once a year? Well, we don't see the vast majority of the cases in neuropomology because most people don't have any lasting deficits. But over at the hospital, they've got at least a couple of months. So this is what you're saying is this is certainly rare, but not uncommon. Right, yeah. And if you lump RCBS and press together, which radiologically I think that they think of them as kind of a continuum. They're both a dysautonomia of some sort. Well, and there's a predilection for the posterior circulation because the auto regulation of the posterior circulation is less robust than the anterior circulation. But interestingly, you can even get a similar syndrome of press in the brain stem. So all kinds of weird eye movement abnormalities and things. Could be a real puzzle clinically to figure it out. But with all the young pregnant people, with all the people on the medications that are known to precipitate press, even over at primary, you'll see these conditions. And I mean, I can't really put a number on it, but I would say that at any given time, they probably have somebody in the hospital with it, whether it be on the OBGYN floor or in the medical ICU. In controlled blood pressure, they're a funding agent. Give them time, and it tends to resolve and not come back. Yeah, most of the time there aren't lasting deficits. But the presenting neurological symptom is frequently visual. Right. Well, you can see that. So funny spots in the vision, but their exam is normal. They may not have the fundus findings of hypertension. And in a child with this, their blood pressure may be 140. If their blood pressure is normally 90, 80, or 90 over a palp, as children often are, they can have this with what, to us, would appear to be a relatively normal blood pressure. Is it usually pretty symmetrical between the two Ys? Well, since it's cortical, mostly, yes. Turning where it is. Yeah, it's routinely spots in the vision. Chris, how's your patient? She improved it all. So she saw Dr. Warner on clinic. And essentially, her vision had actually improved a bit. She was able to say, I think, 2100, essentially. Her central vision hadn't gotten much better, but essentially, she was actually able to do visual field. So she can count fingers in the right superior and left inferior quadrant of both eyes. And yeah, she was actually able to see the eye chart, which there were tears of plenty, because she really had, she was functionally completely blind at onset. So we've got to move on. So if you would mind, you can set up. Just to answer this question, Chris, so you said, MRI could be normal in our CVS. What would you see differently for vasculitis versus the sausage on a string? Yeah, yeah, yeah. So with vasculitis, you can see similar findings. With vasculitis, you'll typically see also an ventilated inflammatory markers as well. With our patients, she had an elevated ANA, which raised concern for vasculitis. But repeat ANA was normal. Or see, your ESR is mildly elevated. But if you adjust it, it's not really that elevated. And actually, it might even be age-adjusted normal. You can see the usual thing with this kind as you jump to the heavy-heeled steroids. But that could make it worse.