Transcription:
How to Avoid Neuro-ophthalmic Catastrophes: Safe not Sorry

Melanie: Welcome to Better Edge, a Northwestern Medicine podcast for physicians. I'm Melanie Cole. And today, we're discussing neuro-ophthalmology, how to avoid neuro-ophthalmic catastrophes. Joining me in this panel is Dr. Nicholas Volpe, he's Chair of the Department of Ophthalmology and a George W. And Edwina S. Tarry Professor of Ophthalmology at Northwestern Medicine; and Dr. Shira Simon, she's the Director of Neuro-ophthalmology and an Assistant Professor of Ophthalmology and Neurology also at Northwestern Medicine.

Doctors, I'm so glad to have you with us today. Dr. Simon, we're going to start with you. So tell us about some of the common symptoms and signs that patients present with to neuro-ophthalmologists.

Dr Shira Simon: Absolutely. And thank you so much for having us today. So the most common things that will cause patients to come in, it's usually some change in their vision. It could be sudden, it could be, you know, in the last couple of weeks or chronic vision loss. It could be painful or painless, complete or partial, in one eye or in both eyes, and that could be caused by a whole host of issues. But usually, it's a vision change. It could also be double vision that people are seeing things with both eyes open on top of each other, side-by-side, obliquely, double. Those are usually the most common. Dr. Volpe, did you want to add some others?

Dr Nicholas Volpe: Yeah. I point out that in the background, in terms of things that neuro-ophthalmologists encounter would be pupil abnormalities as well as potentially drooping eyelids and all the versions of ptosis. This is a good time to point out that many of these things that you just outlined are very common and from relatively benign etiologies. So ophthalmologists, optometrists, neurologists that encounter these patients are often going to find themselves worrying about the worst thing or the potential catastrophe. When in the background, there are many innocent causes for what may seem like a big change in the eye appearance or the way patients are seeing. So neuro-ophthalmologists play a critical role in sorting through these various symptoms and signs and determining what is a potential catastrophe or an impending event.

Melanie: Dr. Volpe, can you expand a little bit on signals for a doctor to be more aware and make sure that they are on top of it and/or referring to the specialists at Northwestern Medicine?

Dr Nicholas Volpe: I think, you know, this is a good time to point out that most of the things in neuro-ophthalmology are relatively easy to recognize, have a limited differential diagnosis in which you can move through the things you have to worry about in terms of ordering tests and workup. But I would point out to the person who isn't seeing a lot of these patients or encountering them only on occasion, that the first thing that generally you should worry about is when older or very old patients have some of these symptoms because of the important overlap of systemic symptoms or conditions that often present with neuro-ophthalmic signs. And particularly older patients of course are at more risk of having a stroke and for presenting with ophthalmic manifestations of arteritis, for having tumors with progressive vision loss and for having vascular lesions, particularly aneurysms that could present with double vision or other cranial nerve palsy.

So if you can't figure it out and you can't see what the patient has or it's not a very clear manifestation of something you encounter, for instance, the overlap between someone being born with an eye muscle problem and just developing a decompensation versus someone acutely having a stroke and having a similar presentation. So if you're not comfortable, then think about things you haven't seen before and particularly elderly patients and patients with pain that would put up a red herring that you should think about more workup, additional help.

Dr Shira Simon: I was just going to add to that, often there will be more than one sign. And when there's more than one sign, that gets a little bit worrisome. For instance, if someone has pain in their eye, the vision's going down and maybe they'd noticed tingling in their arm or leg, then that could tip us off to something like optic neuritis or multiple sclerosis.

If someone has a new drooping of their eyelid and then says, "Oh, you know, my pupils are different sizes," or "I recently had a chiropractic manipulation," we worry about Horner's syndrome. So it's kind of, you know, putting the puzzle together, but also sometimes it's, "I had imaging, it was normal, but the vision keeps on declining." We've seen many of those cases where we just have to review the imaging and there's some subtle finding that's actually abnormal and that's where our role comes in to try to identify what it is and get them treated in the correct direction.

Melanie: Such vital information that you're both giving here today. Really important. So, Dr. Simon, what are your biggest concerns in patients with acute vision loss? What are you looking for?

Dr Shira Simon: So as Dr. Volpe said, sort of the biggest emergency, one of the biggest emergencies in neuro-ophthalmology is giant cell arteritis, especially elderly community can have progressive permanent vision loss and other manifestations throughout the body, that is sort of a life or death diagnosis that we do have to catch. Similarly, I'm always worried about something in the brain, an aneurysm, a mass. And then the more nuanced findings that we still see a lot are other systemic things. So other autoimmune conditions when cancer can infiltrate in different ways, if it's like lymphoma, leukemia. You know, it's sort of piecing it all together if it's vision, but also figuring out what other organs might be affected.

Dr Nicholas Volpe: And I would also add that in this group of patients, although these will overlap and it's not a hard diagnosis to make, but ophthalmologists, optometrists, others will encounter patients with acute strokes, if you will, or artery occlusions in their eye. And that group of patients have recognizable examination and should be treated like anybody else who's had a stroke, mostly doing things to prevent the risk of additional stroke. So we are now very aggressive about managing patients, both with transient monocular vision loss that may come from an embolism or an impending stroke or an actual stroke in the eye, such as a retinal artery occlusion.

And then we have lot of unfortunate and advanced causes of vision loss, for instance, ischemic optic neuropathy, where there isn't much you can do, but certainly recognizing that condition, making sure it's not from temporal arteritis, making sure this is not a cancer patient that's mimicking an ischemic optic neuropathy. All of those things are in patients who are older and have these underlying systemic conditions or risk factors.

Melanie: So then, Dr. Volpe, how about double vision? Is that a setting in which catastrophes can potentially be avoided? What would you like other providers to know about that?

Dr Nicholas Volpe: So double vision is perhaps the most commonly encountered neuro-ophthalmic symptom. It's also the one that quite frankly most non-experts are not familiar with because in order to have the experience and the exam expertise to examine these patients, you have to do it on a fairly regular basis. And I'll point out that the vast majority of patients that have double vision have fairly innocent and/or benign causes that don't require urgent or emergent evaluation.

But in general, probably the most important are patients that are presenting with some form of what's called a third nerve palsy. The third nerve palsy patients are often patients that are presenting with aneurysms that are at risk of bleeding. So anyone deemed with third nerve palsy should literally be sent to an emergency room.

The other cranial nerve palsies, those are a little bit less common in terms of having urgent or important causes. But generally, we recommend that all patients with cranial nerve palsies have some type of MRI scan or neuroimaging to exclude compressive mass or vascular lesions.

Then there's a host of other patients that have fairly innocent because of double vision, for instance, thyroid eye disease, which is innocent as long as it's only double vision. But if it's causing compression of the optic nerve and vision loss, much more serious and certainly a catastrophe that needs to be avoided.

A condition called myasthenia gravis, which often presents with variable double vision and drooping eyelid.=, Well, that's never really an urgent or catastrophe issue unless of course the patient also has what are called bulbar symptoms or difficulty swallowing, chewing, breathing, things that would literally represent potentially life-threatening manifestations of myasthenia.

Once again, giant cell arteritis creeps into the differential diagnosis of elderly patients with double vision. And just to go back to what Dr. Simon pointed out, basically anybody that has any type of systemic cancer that is presenting with a neuro-ophthalmic sign, the examining physician should assume that the presentation from a neuro-ophthalmic perspective is related to the cancer, its treatment, its spread.

Melanie: So then, Dr. Simon, and along those lines, do patients ever show up to an ophthalmologist with neuro-ophthalmic signs, having recently had a stroke? And why is recognizing this scenario so important?

Dr Shira Simon: Yeah, absolutely. It can be, as Dr. Volpe was alluding to sort of a local stroke, so an ischemic optic neuropathy or amaurosis fugax where they'll just have a fleeting curtain in their vision and then the vision comes back. But either way, that does tip us off to looking into other systemic vascular risk factors that might put them at risk for more systemic strokes. So when it's "just in the eye," we still do really sound the alarms because we want to make sure that we've looked to make sure their high blood pressure or high cholesterol or diabetes or even sleep apnea has been adequately addressed, diagnosed and treated.

But we also certainly see people after a stroke. Sometimes it could be that they have a facial palsy from the stroke. It also could be that they have what we call homonymous hemianopia or some discrete visual field cut that's the same in both eye after some usually posterior stroke. And then we do have to caution them about driving, figure out how that's affecting their quality of daily living, their activities of daily living and similarly.

Dr Nicholas Volpe: To emphasize what Dr. Simon said about sort of the strokes that are lurking around in practice that people that are passed out on the floor with an arm that doesn't move, but it's just somebody that has lost the upper corner right vision in both eyes and describes it as vision loss in one eye. And if an examining physician doesn't detect it as a hemianopia, then they potentially miss a stroke. And there are is just lot of evidence suggests that those patients, the most important thing we can do is sort of plug them into stroke protocols in which neurologists expert in this area can evaluate them for risk factors to prevent subsequent strokes.

So really recognizing that, "Hey, this isn't straightforward. This could be a stroke. This could be in the brainstem. This could be an artery occlusion. This could be amaurosis fugax," all of those things should come to the forefront and recognize as possible strokes.

Dr Shira Simon: Right. And also as part of the workup, just to add on, you know, a lot of times they'll be looking at the carotid arteries with an echo or a CTA, MRA, and then also an echocardiogram of the heart, just seeing if there's some cardioembolic cause.

Melanie: Well, thank you for really giving us that comprehensive discussion there. So now, I would like each of you to tell us a story from your practice in which you were glad you acted and maybe saved a life and avoided a catastrophe. So, Dr. Volpe, why don't you go first? Tell us something that happened with you that you were really excited about the way that you practiced your profession.

Dr Nicholas Volpe: I recall a patient who was elderly, who had what we call vascular risk factors that presented with what I mentioned earlier, a third nerve palsy. And she was sort of old enough to have this on an innocent basis or what we call an age-related or vasculopathic palsy. But I sort of followed the protocol and said, "You know what? This patient needs to have an MRI scan" because sometimes these patients can have things that younger people are supposed to get and it's not just a simple vascular palsy.

And sure enough, after we did the MRI scan, it turned out that this patient in her 70s had a large aneurysm and she was admitted to the hospital. And while she was in the hospital, that aneurysm did start to bleed and they were able to treat her emergently and save her life as opposed to if I had sent her home, assuming it was just an age-related palsy. She might've indeed had not such a great outcome if that happened while she was at home.

Melanie: And Dr. Simon, I want to get to you too, but Dr. Volpe, is there sometimes a hesitancy on physicians to run those tests as you just described? Because, you know, there's always this controversy about running too many tests, but yet what you just described was vital and important.

Dr Nicholas Volpe: Well, of course that comes to the art of medicine and we fortunately practice in an environment in which many of these tests are available. And when in doubt, non-experts should seek the help of emergency rooms and other experts where workups may be overdone, but they can be expedited and sort of played against worst case scenario. And that's what neuro-ophthalmologists are sort of best at, is sort of sorting through the things that are pseudo-emergencies versus the true emergencies and that's where we can really come in handy. And if not, then working with neurologists and emergency rooms is the next best step. But no one would ever think ordering an MRI scan, for instance, in somebody with the acute onset of double vision or ordering a sedimentation rate, which is the test for temporal arteritis, would be over-testing in any cirumstance.

Melanie: What a great point you make. And Dr. Simon, tell us a story from your practice in which you were really glad that you acted and maybe saved a life.

Dr Shira Simon: Sure thing. It's a hard act to follow after Dr. Volpe. There's a patient in particular, a younger woman who comes to mind who came to see me because her significant other forced her to. And she told me that she just had a lazy eye or amblyopia in one eye that was longstanding. And she thought she needed new glasses, but the optometrist has sent her to ophthalmology.

And I took a look at her "lazy eye". I actually had the nerve was pale. And then it just got me wondering, you know, I said, "Have you had a scan before?" She said, "No." Ever since she can remember, that eye has sort of been the lazy eye. And we ended up scanning and she had a very large tumor that was pushing on that right optic nerve and then was starting to get the left nerve, which is why she was noticing some vision changes, which is not an uncommon thing as the patients will say they're losing vision. And sometimes the first eye already has lost vision. They start to notice it in the second eye.

So in this case, it was a very large tumor that was about to compress other vital structures, but thankfully neurosurgery did an expert job, removed it, and actually she even regained some of her vision because we still caught it in that window. So that was a very fulfilling thing where obviously it's the whole Northwestern team that really rallied to save her and improve her vision and save her from having other complications.

Dr Nicholas Volpe: That's a spectacular example of what neuro-ophthalmologists do. You know, we are really good at sort of fielding the things that don't make sense. And it's great that the ophthalmologist that saw that patient and sent her to Dr. Simon recognized that something's not adding up and there shouldn't be this sign or that sign in a patient who just has amblyopia. And you connect the dots and, in retrospect, you might say, "Oh, of course it had to be a tumor because it was a pale nerve and it was affecting both eyes." But we encounter those patients all the time unfortunately, that sort of bounce around, don't get the right answer and get under-worked up when there are these very serious underlying pathologies that have life-changing implications

Melanie: What an exciting field that you are both in. So Dr. Volpe, and I want to give you each a chance for a final thought here. Can you describe for other physicians some common pseudo-emergencies where a fairly compelling finding likely has a benign etiology?

Dr Nicholas Volpe: Yeah. For better or worse, despite the excitement of the last 10 minutes, the vast majority of even the symptoms and signs that we introduced at the beginning of the discussion have benign explanations or not critical issues. As I mentioned, the eyes being misaligned or having double vision is incredibly common as a leftover problem from childhood or a weak muscle. It's common in thyroid disease. It's common in patients that are actually born with things that don't develop symptoms until middle life.

Drooping eyelids are another example of an Incredibly common manifestation of the passage of time, as opposed to a true new problem or an emergency. Even an isolated dilated pupil, which really gets people excited because that's the early manifestation of a third nerve palsy, as long as the patient's eyes moves, then most commonly an isolated dilated pupil is just because somebody got something in their eye that dilated their pupil, which is clearly not an emergency. So we encounter these things all the time and I'm sure Shira also has some examples of things that, you know, "Can you see this patient? You got to see this patient. It's critical," and it turns out to be not so critical.

Dr Shira Simon: Yeah, it's always a relief. Because obviously, when people come in with double vision or vision loss or a dilated pupil, we always worry because our mind goes to worst case scenario. But like Dr. Volpe was saying, if there's a dilated pupil, other things that sometimes happen is just one pupil's a little bit bigger than the other, but they are maybe born that way and they just started to notice it. So it could be a physiologic anisocoria.

There's an entity called benign episodic unilateral mydriasis where someone has bad headaches, their pupils might just get dilated episodically. but it's benign as the name implies. Other more nuanced things that we can see is when people are sent in for optic disc swelling. Sometimes that's just physiologically how their nerve was built because of optic disc drusen and we can evaluate for that and see.

A lot of times there is a certain demographic of younger women that we worry about pseudotumor cerebri or idiopathic intercranial hypertension. And we are often the deciding factor if clinically they're having worse headaches, perhaps they started birth control or had a little bit of weight gain, they tend to fit the demographic. But then when we look in their eyes and they have perfect vision and they don't have any swelling of their nerves, that's often a very reassuring sign.

So thankfully, there are some sort of simple, very reassuring cases that we do encounter every day in addition to some of these catastrophes that, you know, we also have gotten very accustomed to managing.

Dr Nicholas Volpe: I'll point out what might not seem like it's a benign condition as an isolated symptom without any other problems with vision or eye movements, there are no important or urgent causes for pain. So, although lots of headaches and things are referred to the region around the eye and a lot of surface disease that affects the cornea and, of course, high pressure in your eye, those are all causes potentially of eye pain. But for better or worse, we tend to see a lot of patients with unexplained eye pain. And there's often not a great explanation beyond it being part of a headache syndrome.

Dr Shira Simon: Yeah. And thankfully, it's very rarely tied with any form of vision loss. As Dr. Volpe said, as long as there are no other symptoms and just eye pain, we're almost always reassuring the patient that's benign.

Melanie: So Dr. Simon, I'd really like to jump back to the exciting part for a minute as Dr. Volpe, we discussed these pseudo-emergencies with benign etiologies. Are there particular high stakes diagnoses you might encounter while seeing inpatient ophthalmology consults? And while you're telling us that, wrap it up for us with what you would like referring providers to know about this field of neuro-ophthalmology.

Dr Shira Simon: Absolutely. Thank you so much. So a lot of the things that we touched on, we can see on the inpatient setting. Commonly, if a patient starts complaining of double vision, it could be that they have dry eyes as an inpatient, but it also could be that they have an acute cranial nerve palsy and needs to have imaging, because there's a re-extension of bleeding in the brain or tumor, or they might just have a cranial nerve palsy because of a ischemic event.

Giant cell arteritis, for sure, we always have to have in our differential systemic manifestations of disease. So if there's leukemia lymphoma, sometimes we can find that on exam.

As Dr. Volpe said, if there was a field cut, they say, "Suddenly, I can't see the right side of my vision," it actually might be the right side of their vision in both eyes. And that would be concerning for an acute stroke.

So in general, when I think about what I want referring providers to know is that we're very accessible. We're here because this is literally our specialty. So if anything doesn't seem to be fitting and somebody has some unexplained vision problem that people can't explain, like that's what we're here for and we're always happy to fill the phone call, see a patient, whatever it is, because this is the stuff that excites us. And also we usually have very good seamless pathways to get these patients to where they need to be.

Melanie: Dr. Volpe, any final thoughts?

Dr Nicholas Volpe: Yeah, I think I can tie those two things together, and thanks again for having me. You know, in terms of inpatient consults, I think the thing that is always most alarming or rewarding. I'm not sure what the right word is when I see an inpatient consult, it's how remarkable it is how far a patient could get with a symptom and have not had anyone carefully examine their eye.

There are patients that are evaluated for vision loss and they have a bad cataract or something that's pseudo-sudden. So the great power of neuro-ophthalmologists is that they're big thinkers about brain and systemic disease, but we're also really good at examining eyes and taking histories and putting together the story to know whether it's something bad or something good or something that's about to lead to a catastrophe or we'll know the test to order.

So as thinkers and the doctors that really are interested in understanding the subtleties of a history and are good at examining eyes, as well as interpreting neuro-imaging studies and understanding the systemic implications of various, presentations and various diseases that puts neuro-ophthalmology at the forefront of evaluating one of the most compelling patients who present with vision symptoms.

Melanie: And Northwestern is leading that charge. Thank you both so much for joining us today. What an interesting interview and episode this was.

To refer your patient or for more information, please visit our website at breakthroughsforphysicians.nm.org/ophthalmology to get connected with one of our providers. That concludes this episode of Better Edge, a Northwestern Medicine podcast for physicians. I'm Melanie Cole. Thanks so much for listening.