This live webinar identifies at least 3 uncommon pathognomonic patterns of eye movements. Appropriate testing for these entities are also discussed.

Lecturer: Dr. Karl Golnik, Chairman, Department of Ophthalmology, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA

Transcript

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DR GOLNIK: Greetings. This is Dr. Karl Golnik, here for another neuro-ophthalmology Cybersight webinar. For me, it’s morning time. So good morning, good day, whatever time it may be for you. You’ll notice perhaps, since I think my last webinar, I may have lost my beard, so I’ll have to send them another photo. I look much younger without the beard. I’m sure everyone would agree. So today we’re gonna talk about wacky eye movements, weird eye movements. Are they deadly, debilitating, or just dubious? And so we’re gonna run through mostly cases. In the past neuro-ophthalmology webinars, which have been recorded and are on Cybersight, we talked about a couple of — I think in a couple different webinars — about different types of common eye movement abnormalities. So cranial nerve palsies and myasthenia and so on. This talk is sort of more… Maybe a little bit more advanced. I should say probably conditions that are not quite so common. So that said, you’ll probably see some of these things as you go through your practice of ophthalmology. So my objectives for the talk are that we will, when we’re done, you’ll be able to identify at least three uncommon pathognomonic patterns of movements, and number two, determine appropriate testing for the entities that we’re going to be looking at, mostly through videos. And so we will start with this gentleman, who’s asking me if he’s going to be on YouTube. This is a fairly recent video, in a fellow with double vision. And you can see that he is trying to look to the right here. And I’m gonna ask you a multiple choice question in a moment. Here he is in left gaze. Upgaze. And downgaze. So let’s watch his eye movements again. So here he is looking to the right. And left. And the video is looping. So he’s starting at the beginning. I’ll show you one more time. And I’m gonna ask you the question on the next slide, and then we’ll come back to the video and look and see. So here we are again. Right gaze. Left gaze. Upgaze. And down gaze. All right. So let’s move to our question. So you can vote on this with your keyboards, your laptops. What do you think this is called? Is this called a Weber syndrome, a one and a half syndrome, Parinaud syndrome, or Wallenberg syndrome? I’m gonna give everybody a moment to vote. I asked them not to let everyone see how people are voting while you’re voting, so you won’t be influenced. If there’s a predominantly popular choice. So we’ll give you another 10 seconds or so, and then we’ll show the results of the polling. Five, four, three, two, one. Can we show the results? Are people voting? Here we go! All right. So we do have one most popular vote. About half the people, a little bit more than half, said this is a one and a half syndrome. And you — majority — you are correct. So let’s remove that polling and look back and figure this out. So here’s our guy again. And hopefully the video will play again. And so here he is. When he looks to the right, you’ll notice that the left eye does not move to the right, but the right eye does. But there is this funny nystagmus-type movement, so-called abducting nystagmus. So this alone represents a left INO, a left intranuclear ophthalmoplegia. And when he looks to the left, his eyes move slightly to the left, symmetrically. He has an almost complete left gaze palsy. So a left INO, and the video is looping. A left INO and a left gaze palsy. So when we use the term “gaze palsy”, if neither eye moves in a certain direction… So left INO, left gaze palsy. This is called a one and a half. The left gaze palsy is the one, and the left INO is the half. So a one and a half. And this can be due to fairly small lesions. So here we are. Here’s a one and a half. It could be deadly, because, of course, if you have a bad problem in your pons, then you could die. If there were a big stroke or something. It could be debilitating. Because this guy did have a stroke, in fact. And he is somewhat debilitated, because of this eye movement problem. So deadly… If there were, let’s say, this was due to some sort of a glioma, an intrinsic pontine tumor, that could be deadly eventually, but certainly it could at least be debilitating. It could get better, or it may not, after a stroke. But let’s look at the anatomy. So here is a schematic cross section through the pons. And you’ll see here we have the 6th nerve nucleus. We have the medial longitudinal fasciculi. So if we call this the left — so in this gentleman’s case, if there’s a lesion here of the MLF, and of PPRF, the horizontal gaze center, this gives you the INO. That’s the half. This would give you the one. The gaze palsy. So a lesion that looks like this — pretty small. Or remember that the interneurons that run from the PPRF, from the horizontal gaze center, run through the 6th nerve nucleus. So a nuclear 6th nerve lesion, plus affecting a bit of that adjacent MLF could give you the same picture of the so-called one and a half syndrome. Now, there’s something else that sometimes comes up, and it’s called an 8 and a half. And so as you might imagine, an 8 and a half is our original one and a half, plus a problem with the 7th nerve. So here is labeled — here’s the motor nucleus of the 7th nerve. So you can imagine that if the lesion were a little bit bigger, and it affected the MLF, and the horizontal gaze center, and the 7th nerve, now you would have the eye movements we just saw, plus the person would have an ipsilateral 7th nerve palsy, so a facial weakness, and that would be called an 8 and a half. So I don’t have a good video of an 8 and a half. So that’s weird, wacky eye movements number one. And remember, you can type in questions in the chat box any time. I will not see those questions until the end of the webinar, but we’ll try to save five or ten minutes at the end so that I can answer any of the questions. So type them in whenever you want. You don’t have to wait ’til the end. So this is a fellow — I’m gonna show you his video. Let me just tell you his story briefly. He’s a young guy who, about five, six years earlier, was found to have a posterior fossa tumor. Benign tumor. But he was treated with surgery and with radiation. And this left him with a mild to moderate left 6th nerve palsy. So here are his baseline eye movements. And you can see that he’s got a left 6th. So he’s a little esodeviation. A little esotropia. And he looks up and down and right just fine — but watch when he looks to the left. So you can see the moderate left abduction deficit. This is his baseline. He says yes, I have double vision. It’s primarily when I look to the left. I’m just gonna run the video again while I’m talking. Primarily when I look to the left. He has a little bit of a head turn. He doesn’t really want eye muscle surgery. And basically he’s content. The problem is: Recently, over the last some months, he says… You know, sometimes my double vision reverses. Reverses? What do you mean? He says you know, my eyes have a tendency to cross inward relative to one another. But sometimes they go outward! I said… Outward? He said yeah. They’re deviated outward. And I could tell that my double vision has reversed. I said… Okay. Does it happen at any particular time? Well, it seems if I’m exercising or if I’m active or upset or something like that. I said… Well, let’s take a look. I don’t know if you can see my video on my screen. So I said… Let’s take a look. Will it happen if you’re just sitting here? He said no. I have to be exercising. So we went out into the stairwell, where we had several flights of stairs. And I said… Okay, run up and down the stairs. So he ran up and down the stairs, he ran up and down the stairs, nothing happens, he ran up and down the stairs, and finally I said… All right. One more. Up and down a few more times. And here’s the way he looked after running up and down the stairs. Now he has an adduction deficit! Look at this. And his abduction is good. Look at that! He’s exotropic here. And we’re watching realtime video. Look. He’s XT. And look at the striking Ad-duction deficit. Good ab-duction. But watch what happens. This is over realtime. He says… This is typical. It’ll happen, and then it’ll go away over a minute. So now you see his ad-duction deficit is decreasing. And his ab-duction deficit is coming back. And eventually, he’ll be back to his baseline. So this is unedited, time-wise. Now his ad-duction looks pretty good. And his ab-duction… I think we’ll show it one more time. Ad-duction, normal. Ab-duction… Back to baseline! Ha-ha! So… The question is: What is going on? This is kind of wacky. And we’re gonna have this be a polling slide for you guys. So what’s going on here? Is this myasthenia gravis? Is this a transient cranial nerve palsy? Is this something called ocular neuromyotonia? Or I have no idea what this is? And we’ll let people vote. I’ll give you a few seconds here. All right. So we have kind of a mixed bag. The majority of people, 42%, think this is ocular neuromyotonia. About a third think myasthenia. Some of you are very honest, saying no idea. And a few think transient cranial nerve palsy. So this is indeed, again — the majority is correct. We can get rid of the polling. This is a condition called ocular neuromyotonia. So ocular neuromyotonia occurs most commonly after skull-based radiation. So posterior fossa, cavernous sinus radiation. And usually people have a cranial nerve palsy. It can be III, it can be IV, it can be VI. I’ve never seen IV. I’ve seen III. In fact, I’m gonna show you a video of III in a moment. So it can be III, IV, or VI. And usually what happens — that some years after the treatment, the radiation, and the person usually has a mild or moderate deficit that has persisted. But once in a while, instead of that nerve working too little, it works too much. And when it works too much, if it’s the 6th nerve, what happens? You get this constant firing of the nerve. So the abduction improves, but now there’s a restrictive process. And the eye will not ad-duct. It’s not because there’s paralysis of the medial rectus. It’s because the lateral rectus muscle is tight. The 6th nerve keeps firing, firing, firing. There’s different theories about what causes this. One of them is called ephaptic transmission. Some sort of cross talk, where there’s this kind of loop, where the nerve just fires, fires, fires. It works normally, but it prevents the yoke muscle — in this case, the medial rectus muscle — from pulling the eye inward, because the lateral rectus is tonically firing, because the 6th nerve is repetitively firing. And so that explains this deficit. And then after a short time, usually typically what you’re seeing in the video, a minute or so, that 6th nerve gradually stops firing, goes back to its baseline, partial paralysis, and you see his usual baseline. So this is ocular neuromyotonia. Usually after some sort of skull-based posterior fossa radiation. Rarely reported in association with other conditions. Even thyroid eye disease — I really don’t understand that. I personally have not seen it. So this has left 6th nerve ocular neuromyotonia. Let’s look at a different patient. So here I wrote “prior radiation, rarely vascular compression, even thyroid eye disease”. It is debilitating. It’s actually treated. I didn’t mention. You can treat this. I treat this with oxcarbazepine. As opposed to carbamazepine, which requires blood monitoring. So oxcarbazepine works very well. I don’t see this much. I’m told that in Asia, where there’s a lot of nasopharyngeal cancer and skull-based radiation, neuro-ophthalmologists at least will see it fairly frequently. I’ve had great results with oxcarbazepine, also known as Trileptal in the United States. And both this gentleman and another patient of mine, whenever they run out of their prescriptions, call me, and say: Yeah, I ran out of my prescription. I stopped taking it. Within days, it started up again. Please refill my prescription for the medication. So here’s another patient, who has, again, a history of a tumor, many years ago. He is cognitively not that with it. He actually has no complaints at all. But his caregivers complain that sometimes his right eye turns upward and inward in the eye socket, and stays there for a short time. So let’s look at his baseline video. And here he is. You can see that he has an old partial right 3rd nerve palsy. He’s got a little bit of ptosis. His eye doesn’t move down fully, doesn’t move up fully. It abducts okay. He’s not really looking all the way over to the side. There he goes. That’s a little bit better. And it adducts okay. But up and down, not normal. So let me just show that one more time. So abduction, not bad. Elevation is not normal. Depression is not completely normal. Adduction may be slightly decreased. There’s the depression. You can see the deficit. A little bit of ptosis. So here’s what happens on occasion. And I made this happen by having him try to look up and in. And then, when I did, and I said… Okay, now look straight ahead… Here’s how he looks. So let’s look at the other video. Now look at his eye. So it’s up and in. And when he looks to the right, look. It won’t move down and it won’t abduct. Look. Because he has third nerve ocular neuromyotonia. In particular, with his medial rectus and superior rectus. So now his eye is restricted up and in. It won’t abduct, because the medial rectus keeps firing, because part of the 3rd nerve is firing. And it won’t go downward, because of the superior rectus. So this is 3rd nerve ocular neuromyotonia. And again, associated with prior radiation, possibly vascular compression, thyroid eye disease. It’s debilitating. It’s not deadly. It’s after, usually, treatment of a tumor with radiation. Okay. Any questions? Remember, go ahead and feel free to ask them. So let’s look at this fellow. He’s actually an inpatient. And he has these wacky eye movements. He’s lying down. He doesn’t want to sit up, because he’s very offbalance and has vertigo. Just feels crappy. Interestingly, he did have an upper respiratory infection. Flu-type symptoms. A week or two ago. You can see his eye movements. I’ll run that again. And I’m gonna ask a question, a multiple choice question, about what you think. So… Let’s go to the question. So this is a different kind of a question. Sort of assuming you might know what he’s got. And the question is: If this was an 8-year-old — now, he wasn’t. He was a 23-year-old, I think. But if you saw these eye movements in an 8-year-old, what would the appropriate evaluation be? Would it be an MRI of the head? Lumbar puncture, acetylcholine receptor antibodies, or urine catecholamines? This is a little different question. It presumes you might know what these eye movements are. And then, in an 8-year-old, what would the appropriate evaluation be. All right. So we had no votes for the middle two choices. MRI of the head or urine catecholamines. So in an eight-year-old, honestly, probably both these things would be done. The most important thing would be the urine catecholamines, however. Go ahead and remove that polling. Because this is a form of ocular neuromyotonia. It was his abnormal eye movements — and I might as well move ahead. We’ll just play the video again. His abnormal eye movements are conjugate. Right? Both eyes are doing the same thing. They’re primarily horizontal. There’s probably a little bit of vertical component in there. If it’s straight horizontal, you can call it ocular flutter. But there’s a little bit of vertical from time to time. So this is a form of ocular neuromyotonia. Now, in a 23-year-old guy, he did not have neuroblastoma, which is what we’re concerned about in an 8-year-old. So an 8-year-old with neuroblastoma — we’re concerned… Excuse me. An 8-year-old with opsoclonus — we’re gonna be most concerned that they have neuroblastoma, until proven otherwise. And the neuroblastoma is not necessarily in the head. Right? They’re probably gonna end up with a whole body scan, because they could have metastatic neuroblastoma. But they could also have a very small neuroblastoma, because opsoclonus is a paraneoplastic phenomenon. Paraneoplastic phenomenon. Which is a remote effect of the cancer. So antibodies are formed fighting the cancer, but they attack part of the brain. So the MRI of the brain could well be normal. In fact, patients who present with opsoclonus and are found to have neuroblastoma have a better prognosis than patients who don’t develop the opsoclonus, because the tumor is often found much earlier, because of these wacky eye movements. So in this fellow, it wasn’t deadly. It was debilitating while he had it. But it was actually related to his previous viral infection, which sometimes we’ll see. So this is opsoclonus. Sometimes you’ll see a triad of opsoclonus, myoclonus, and ataxia. Neuroblastoma, until proven otherwise, in a kid. It can be postinfectious, as it was in this fellow. So opsoclonus sometimes with myoclonus and ataxia. And it may be deadly, if it’s a kid with neuroblastoma. It can definitely be deadly. In this 23-year-old, it was debilitating. It gradually resolved spontaneously with no treatment, over about three to four weeks. All right. We’ll move ahead. More wacky eye movements. So this one may not be that wacky, but we’ll see. Let’s play the video. See, she’s got a patch on her forehead there, because she has double vision. And so I’m gonna ask you what you think about these eye movements. If you have a diagnosis, that may be a little bit tough and not fair, because I’m really just showing you her eye movements. Not her alignment measurements. I can tell you a little bit more about her history. Let me play the video again. This should all be looping ’til I stop it. And that is… She has double vision, obviously. But interestingly, her double vision is only when she looks to the left and the right. When she looks straight ahead, her vision is blurry. It’s not double. But when she covers either eye, straight ahead, her vision is fine. But with both eyes open, it’s blurry. And that’s the story. So she likes to wear the patch straight ahead, primary position, not because of double vision straight ahead. She has double vision in left and right gaze. But because of blurry vision in primary position. So we’re gonna ask a question here. And I think this is the question: What’s the diagnosis? So does she have bilateral 4th nerve palsies? Does she have myasthenia? Does she have thyroid eye disease? Or none of the above? All right. So the majority of people thought bilateral 4th nerve palsies. There are a few votes for all of the other categories. Indeed, this patient does have bilateral 4th nerve palsies. We can get rid of the poll. And let’s look at her eye movements again. And so the key thing about bilateral 4ths is that you’re looking — and of course, I think if you were being tested or if you had this patient in your exam room, you would be looking for what we call “shifting hyperdeviation”. So when she looks to the right, she has a left hyper. Especially when she looks down and to the right. So down and to the left, she has a right hyper. And again, you can quickly do your cover-uncover testing to show this. So right hyper in down and left, left hyper in down and right. Shifting hypers. Now, she does not have double vision in primary position. Yet she has bilateral 4th nerve palsy. She does not have double vision, but she has very blurry vision. So why is that? Well, obviously if you have a unilateral 4th nerve palsy, what happens to the eye with the palsy? It moves up and it extorts. Right? Because the 4th nerve and the superior oblique muscle are intorters. So in primary position, you’re gonna have a hyperdeviation, and an excyclodeviation. But if you have symmetric bilateral 4ths, now both eyes move up. So you eliminate — you subtract the vertical component, and it may be that there is no vertical misalignment. But what happens to the excyclotorsion? The excyclotorsion adds. So instead of having a few degrees of excyclotorsion in primary, now you have two times a few degrees. So the person has blurry vision, because they’re seeing two images that are superimposed, but excyclotorted, relative to one another. When you close either eye, your brain will right the image, and you will have clear vision. So bilateral 4ths, shifting hypers, and a lot of excyclotorsion. You would measure that with the double Maddox rod. If you don’t have a double Maddox rod and just a single Maddox rod, you can put the single Maddox rod in front of one eye. You can ask them to look at the eye chart at the end of the room, that has a horizontal edge, straight edge, and ask them to look at your light to see the red line of light in the Maddox rod. And then compare that red line of light to the horizontal edge of the eye chart. So that has been shown to be effective. And see if there is a lot of excyclotorsion. So bilateral 6th. Or excuse me, bilateral 4th nerve palsies. And this is usually debilitating. It’s usually not deadly. And it’s certainly not dubious. All right. And this person — the underlying etiology by far, the most common reason to see this scenario, is closed head injury. The 4th nerves are long, thin, and come out the back of your brain stem. Right? So they’re easily shaken up from a closed head injury. Usually improve. Not always, but usually improve spontaneously. All right. Let’s move on to another topic. This young woman… She’s not that young. Younger than I am, though. She was in a motor vehicle accident. And she got double vision. Interestingly, she did not have any injuries to herself at all. But her husband, who was the passenger, died. And we’re gonna look at her eye movements. You can see that she is… I’m trying to get her to look at a target. She’s esotropic, clearly, here. Oh, now she’s really esotropic. And when she looks to the right or left, she can do it. She’s got some kind of funny-looking movements. And sometimes it almost looks like the abducting eye isn’t abducting quite… Like right there. Look. And then if you make her look… Oh, look how the eye gradually does come out. But initially, it sort of stops. So let’s look at this again. So she’s eso. But she gets even more eso. So watch when I start screwing around with her eyes. She develops a larger esotropia. A larger… Watch. Watch when I remove my hand. Whoa. And at times it seems like she has abduction deficits, but then they’re not sustained. So… Interesting story. Let’s see what the audience thinks. What is the diagnosis? This is another audience poll. Is it bilateral 6th nerve palsies? Is it myasthenia? Is it thyroid eye disease? Or is it spasm of the near reflex? All right. So we have two popular votes. One is spasm of the near reflex. One is bilateral 6th. So sometimes this can… This is indeed spasm of the near reflex. Sometimes this can be confused with bilateral 6th nerve palsies. We can get rid of that. Let’s look at the video. And the reason it might be confused is because you can see what look like these 6th nerve palsies, but if you really make them look to the left, and you see that left abduction deficit, then keep them in sustained left gaze, usually that eye will gradually go out to the left. So it can look like an abduction deficit, but it doesn’t stay there. So if you really had bilateral 6th nerve palsies or unilateral 6th nerve palsies, the eye is just not gonna move there, no matter what you do. But with spasm of the near reflex, it usually gradually will. One of the key things you want to look for, for spasm of the near reflex — of course, it may be variable. It may be constant. But look at the pupils. The pupils should be miotic, right? Because part of the near reflex is miosis. And if we watch her pupils, you can see they’re pretty small to begin with. But when she’s really eso, the pupils are a little bit smaller. And if you can break her, which is what I’m trying to do now, the pupils get a little bigger. But watch when she gets really esotropic, when I cover this eye. And now she’s looking straight ahead. Look at how small those pupils are. Wow. So she’s got more spasm of the near reflex. I was trying to break her down, to see if I could get rid of the spasm, to show that her pupils are even bigger. But you want to look closely at the pupils if someone has intermittent — they look like this intermittently. Because when they’re eso, they’ll be miotic. And that’s a clue that this is spasm of the near reflex. So we don’t really know what causes this. I mean, the classic story is a college student studying all night long. They get stuck. You can put atropine in the eyes, try to relax their accommodation, and maybe that’ll break the spasm. But it can be a problem. I’ve seen people who have had this for years, and we’ve tried everything. Plus lenses. We tried everything we can do. Cannot get rid of it. So it can really be a problem. But we think it’s more of a psychiatric sort of a thing than anything else. She had it, we think, of course, because — not the head trauma. She didn’t have head trauma. But her husband died in the accident, which obviously caused a lot of psychological trauma, and we think that’s the case. And it did gradually, in her case, go away. So this is spasm of the near reflex. And this is debilitating. It can be debilitating. But you can try to treat this with cycloplegic… I’m not sure about psychiatric therapy. We talk about that if it’s really persisting. There was a report, and I put this on the slide. A refractive lens exchange. So actually, surgery. And the report was interesting, because it sort of started by saying how great this worked, but then it said… After a year, it came right back, even though the refractive lens was still in the eye, obviously. So I don’t recommend refractive lens exchange. So here’s another patient who’s really asymptomatic. And watch her eye movements. They seem pretty good here. Oh! What’s going on here? So this person is asymptomatic. And this is my secretary. She said… Oh, I can move one eye at a time. I said… Let me see that! So what is she doing? She is invoking her near reflex to spasm her convergence. And when she does that… Let’s see if we can see her pupils. Watch her pupils get smaller. A little bit smaller. There they get bigger. So this is a party trick. So this is something that people can do… I don’t know. Probably some of you can do this. I can’t. This is a party trick. And it’s not something to worry about, obviously. All right. Dubious! This is dubious. It’s not debilitating and it’s not deadly. Party trick. All right. So here’s a guy with double vision. And he’s in his 50s. He has known gastric cancer. And of course, because of that, he got imaged when he started complaining of double vision. He had imaging done. And the imaging… He brought the CD, because one of the things as a neuro-ophthalmologist, I always do is: I want to see the pictures. I don’t trust the reports. In fact, one of the definitions of a neuro-ophthalmologist is a physician who makes a diagnosis based on MRIs that were previously read as normal. So his MRI indeed was read as normal. And you can see his eye movements. And his left eye doesn’t move up very well. I took the video, I think, and I didn’t do a great job. He actually had… The biggest problem was with his left eye moving up and in. And I thought this looked like… Almost like what looked like an acquired Brown syndrome. So a Brown’s tendon sheath syndrome is when the eye in particular won’t move up in adduction. I’m not showing you that very well. You can see the big vertical misalignment when he looks to the right. But he had a striking deficit in elevation that was worse in up and in than it was in up and out. And so I thought he had this sort of Brown syndrome, and I said… Jeez, I’m surprised the imaging is normal. Let’s look at the MRI. And here’s the… Sorry. Here’s the MRI. So here’s the “normal” scan. Now, this MRI was done not at my university. Not read by one of our good radiologists. But it was done at a community hospital, and it was an MRI of the brain. Because the patient has double vision. And so oftentimes I’ll see people… They’ll see their family doctor, they’ll see the neurologist, and they’ll say: I’ve got double vision. So guess what? We get an MRI of the brain. Well, then the radiologist looks at the brain, but they don’t necessarily look at the orbit. And so I routinely will see people with thyroid eye disease, double vision, get a scan, normal. But they don’t look at the orbit. Now, this guy didn’t have thyroid eye disease. What he has is this very large superior oblique muscle. And this was a metastasis from his gastric cancer to the superior oblique muscle. Which indeed killed him. So this, in this case, was deadly. But that’s why you look at the films! That’s why you look at the MRIs or the CAT scans. Because they looked at his brain, and sure enough, the brain was normal. But the orbit was not. And this large superior oblique muscle was giving him this acquired Brown syndrome. So unfortunately, in his case, this was deadly. Okay. All right. Here’s a 75-year-old woman. Saw me on her way home from the hospital. With double vision. Here are her eye movements. This is up. Right. Left. Down. Want to see that again? Up. Hold on. I’ll show it again. So up. Right. Left. And down. Okay. Those are her eye movements. Fairly straightforward. Her pupils were normal. Her lids — maybe a little droopy. So what do you think? Is this CPEO? Is this the Guillain-Barre? Or a variety of that? Is it myasthenia gravis? Or thyroid eye disease? And it probably wasn’t fair, because I didn’t tell you the history. All I told you was that she had double vision. So we’ll see what you say, and then we can talk why the history might help, depending on how you vote. Ten seconds. Five, four, three, two, one, go ahead. Let’s show the results. All right. So yeah, a lot of you said CPEO. So the part of the history I left out, which was not fair, was that this happened two weeks ago. And so clearly you wouldn’t expect CPEO in a 75-year-old to start — to be symptomatic two weeks ago. Interestingly, CPEO doesn’t usually cause double vision either. Although her eyes did not move at all, and clearly CPEO is in the differential diagnosis. As is myasthenia gravis. I suppose thyroid eye disease could be in the differential, although I would put that at… I’ve never seen that. But I put that a distant fourth. She in fact had a two-week history. And her history was that she’s a healthy 75-year-old, and she had some sort of a GI problem, gastrointestinal problem, a little diarrhea, for a couple days. That got better. But then about a week after that, she developed double vision. And they did all sorts of testing, including acetylcholine receptor antibodies, Tensilon testing, MRIs, you name it. Everything was normal. They did, to their credit, send a blood test that — it took a little while for the result to come back. And the result did come back with anti-GQ1b antibodies greater than 1 to 10-24. And this is also known as the Miller-Fisher variant of Guillain-Barre. So there’s usually some sort of viral prodrome, but in this scenario, the eye part of it is the prominent part. Not the typical Guillain-Barre. So that’s the Miller-Fisher variant. And these anti-GQ1b antibodies have a predilection for the intranodal areas on oculomotor cranial nerves. So you can see any degree of ophthalmoplegia. What I’m showing you obviously is about as bad as it gets. Her eyes weren’t moving. She was slightly esotropic, which is why she had diplopia. But you can see this triad of ophthalmoplegia, areflexia, and ataxia. She really had very little ataxia. She did have some areflexia. So she had this Miller-Fisher variant. This usually is not debilitating — deadly. Because it’s more eye stuff than typical Guillain-Barre. But it can, obviously, be debilitating. In her case, she received intravenous immunoglobulin and was followed. I saw her six weeks later, and I told her — usually this gets better. And she came back in six weeks, and she said: You lied to me! I am no better. And here are her eye movements. Fortunately her daughter was with her both times and said: Mom! Your eyes are moving way better! But why is she no better? Because she still has a little esotropia. And sure enough, six weeks later — so three months after the start — she did have resolution of the small amount of esotropia. You can see that her eyes are obviously moving much, much better, six weeks after the start of this, and after the intravenous immunoglobulin. So the Miller-Fisher variant, anti-GQ1b antibodies. All right. Here’s a patient who has a very… To me, almost pathognomonic complaint. And the complaint is that intermittent oscillopsia. Now, they don’t say oscillopsia. But they say… Every once in a while, with my left eye only, or my right eye only, everything I’m looking at moves. Or it shimmers. I close my eye in question, and the other eye is fine. Everything looks totally normal. But when I close the good eye, everything I’m looking at is moving. Usually sort of up and down or sideways. Or obliquely. Obliquely. And here are what the eye movements look like. And this is all you see on exam. So you want to look at the slit lamp. You want to look… You can see some of it. But really the best thing to do, which I’ll do in a moment, is to look at the nasal-conjunctival vessels. You can see a little bit of movement of this iris right now. And let’s try to focus on those… See that movement? We’ll get better focus in a second. There we go. You see that little bit of movement there? That’s it. All you see. And it’s unilateral. So the question is: What is this? Unilateral. Is this opsoclonus? Is this superior oblique myokymia? Downbeat nystagmus? Or 4th nerve palsy? So most of you, 82%, got it right. So none of these other things… Opsoclonus is gonna be conjugate. Downbeat nystagmus is gonna be conjugate. So those are not good answers. Because this, I said, clearly, was unilateral. 4th nerve palsy is not gonna give you this movement of the eye. Superior oblique myokymia — myokymia means the muscle is firing when it shouldn’t, basically. And we think it’s probably not a problem with the muscle. It’s probably a problem with the nerve. The 4th nerve, sending an inappropriate signal. So sort of the opposite of 4th nerve palsy. A little bit too much. Kind of like hemifacial spasm. When there’s a little blood vessel irritating the 7th nerve. You can see inappropriate facial twitching, spasming, because the 7th nerve fires when it shouldn’t. We think for the superior oblique myokymia, it’s similar to that. I’ll show it again. You’re looking for small intorsive movements. So this is the right eye. We’re looking at the nasal conjunctiva. But that’s all you see. And these patients often come in and say — I’ve had this happen once in a while over the years. I go to doctors, they can’t see anything. Even when it’s happening, they say everything looks fine. They just want to be reassured, sometimes. But sometimes not. Sometimes it really can be debilitating, as you might imagine. The treatment strategy for this is… Oops. I don’t have the treatment strategy. Beta blocking drops. I usually start with just a drop once or twice a day. Of Betoptic, Timoptic. Once or twice a day. That works for some people. But not a lot of people. But it’s easy. If that doesn’t work, you can try oral beta blockers. If that doesn’t work, something like Tegretol, or carbamazepine, or gabapentin have been tried. And there’s even been eye muscle surgery done, and one or two reports of neurosurgical microvascular decompression of the 4th nerve. Finding a little blood vessel, just like with hemifacial spasm and the 7th nerve. So definitely can be debilitating.

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April 24, 2018

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