During this live webinar, we will define five potentially life or vision threatening conditions in neuro-ophthalmology. At the end of the webinar, you will be able to describe the key clinical features of the five conditions, as well as list the key imaging features differentiating the five conditions.
Lecturer: Dr. Andrew G. Lee, Clinical Professor of Ophthalmology, Texas, USA
Dr. Lee: Hello, and thanks to Cybersight for having me today. I’m going to be talking to you about five neuro-ophthalmic diagnoses that you cannot afford to dismiss. These are my disclosures, I do consult for the National Football League, NASA. None of these apply to the contents of these talk, however. And if you’re interested in more information on any neuro-ophthalmic topic, please come visit me to our website. So with that introduction, I will tell you that I’m not going to be discussing any off-label uses of drugs, and this is just to introduce you to where I work, which is the Texas Medical Center, and the TMC is in the foreground here, in the background is Houston, Texas, we see 7 million patients in that little triangle with 9 2,000 employees, 34 had 4,000 residents and fellows. Today I’m going to be trying to cover the five potentially life-threatening diagnoses in neuroophthalmology. Define the rule of the pupil and touch a little bit on the best dying imaging studies for the five diagnoses. So these are the five chances for you to save the life of your next neuro-ophthalmic patient with any of these conditions. Arteritis, of course, giant cell arteritis, but there are other arteritis-like disorders, apoplexy of the pituitary gland, abscesses and particular mucormycosis in a diabetic in diabetic ketoacidosis. Aneurysm is of the posterior …: So when you’re making a differential diagnosis, of course we’re all taught to think about horses, horses, and horses and not so much zebras, so these are kind of in between, they’re not mythical unicorns but they are life threatening diagnoses and the average ophthalmologist sees at least one of these conditions in their career once. So you’ll probably see all five of these things. The big one is giant cell arteritis and everyone knows what giant cell looks like, elderly patient, often female with acute onset headache, jaw claudication, temporal artery pain neck or ear pain and for us, of course the loss of vision typically due to ischemic optic neuropathy. And the serum erythrocyte sedimentation rate and c-reactive protein are markers as acute-phase markers of disease. So everyone knows that temporal arteritis affects the temporal, but we call it temporal arteritis because that’s where we get the biopsy. Anyone that has the face, the eye, the nose, the tongue, the jaw especially jaw claudication, or they are neck could still be giant cell arteritis. It’s a large vessel arteritis and that means it can affect any part of their head. If you look at the initial symptoms of giant cell arteritis, I want to call your attention to the top four. Headache and there’s nothing distinct tive about the headache. It’s not like the other headache syndromes that have diagnostic criteria. The headache in giant cell arteritis can be mild, moderate, severe, it can be in your temple or in any part of your head. If you ask your average elderly patient do they have joint pain, they’re going to say yes, the thing we want to know is proximal hip and I like to ask, hair, stair, chair, is it hard to comb your hair, get out of a chair or climb a stair and if so why? notice No. 3 here is fever of unknown origin N our hospital, FUO, fever of unknown origin is going to get the usual things: Chest x-ray, urinalysis, CBC and blood cultures. GCA can cause fever of unknown origin and the most important one is visual symptoms without loss of vision and that means transient vision loss or transient diploma should be at the top of the diagnosis. The dangerous part is of course that the eye exam is normal. The other interesting thing about this series is look at where jaw claudication is, only 2% say that’s the presenting symptom and the thing we tell you to look for, the decreased temporal artery pulse, the erythematous nodular artery, those are not even on the list for the presenting initial symptom. The initial symptoms: Headache, PMR, FUO, and transient visual loss. If you look at this British Journal of Ophthalmology from 1997, 23 people, 16% of the cohort, lost their vision. But the dangerous and disturbing thing is, look how long they had symptoms before before the diagnosis was made. They had GCA symptoms, on average, 1.3 months — months — prior to losing their vision and a third of the people had hair, chair, stair, TMR for 10 to 8 months prior to losing their vision and that No. had criteria, the thing I showed you with the transient vision loss, 65% of the people premonitory visual symptoms for 8.a 5 days. As you know, if we treat with steroids before the vision loss, it’s going to be protective. If they’ve already lost their vision, of course we still want to give the voids, but it’s going to be a lot more difficult. This is a beach in Oahu, Hawai’i. Atop the bay is this mountain where there are these three little bubbles up there and you can’t go up there. I want to tell you what happened on that beautiful Sunday at that exact spot, but in ha different year. Two army privates standing at that spot right there, detected incoming aircraft at 702 a.m. practicing with new radar equipment. Lieutenant Tyler received this message and told army privates Lockard and Elliot, don’t worry about it. Well, they should have worried, because that beautiful Sunday was a date that will live in infamy, December 7, 1941, it was in fact the first use of wartime radar in United States history. It was not an incoming flight of B-17s, it was hundreds of Japanese torpedo flights and bombers and of course that will live in infamy. This boat still lies where it sank and oil still trickles out from beneath there and Pearl Harbor. It was a beautiful Sunday when that occurred. The reason I’m telling you now is the Japanese Imperial command chose Sunday morning to strike Pearl Harbor on purpose. They had visited America before and so they knew of what the drinking habits were of the American soldier and sailor on Saturday night and where those boys would be on Sunday morning. Likewise, the giant cell arteritis, and in fact every condition on the list today is ready to launch their surprise attack on Sunday morning at 7 a.m. So it doesn’t matter whether it’s Sunday morning or Saturday night or your birthday or your anniversary, the standard of care does not change based on time of day, holiday, day of week. And you’ve got to be worried on Sunday, 7 a.m., if you see any of these conditions, but especially, elderly patient with vision loss. And so there are some things you don’t know about something you know well. You of course know giant cell arteritis causes vision loss typically from anterior ischemic optic neuropathy, but you should know also that it doesn’t have to be anterior and by anter your, we mean anterior to the posterior lamina. The doctor sees nothing, the patient sees nothing, there’s nothing to see, only the RAPD. And so that normal fundus is a very dangerous thing and of course the sign that was seen in 65% of the series, transient vision loss, or worse, transient diplopia in the elderly with a normal he exam is a premonitory symptom and that is the distinct tive history that you’re dealing with GCA. So which is worse if we see the disc is swollen, A I/O N or if the disc is not swollen, P I/O N? Of course when it’s pallid edema, like this, this is giant cell. However, it’s even more likely to be giant cell if the disc is normal. So a normal appearing disc is what we see in posterior optic neuropathy and in an elderly person that should prompt consideration for optic neuritis. And of course if it’s swollen and pale at the same time, we call that pallid edema. So when we have two ischemic lesions, two circulations involved, either choroid and retina or choroid and optic nerve, you should be thinking about giant cell arteritis. And OCT EA right now we have to still have to use the angiogram for perfusion, so you can see here in this P I/O N case, we have this massive nonperfusion of the choroid and that just doesn’t occur in the nonarteritic OIN. And of course the MRI scan of the head was normal. Why? There really isn’t any reliable MRI result in giant cell arteritis. Yes, you have to do an MRI in this case, but you are really not going to be assured by a normal MRI here. So the big red flags are: Severe vision loss, light perception or no light perception form. It’s really giant cell until proven otherwise when someone is elderly and NLP. Or LP. I would admit that person, by the way. Transient vision loss, is essentially never seen in the nonarteritic form of N I/O N. And therefore, amaurosis fugax, should or transient diplopia should really be with red flag. And hair, chair, stair, proximal hip and shoulder girdle ban with any morning symptom is a big red flag that you’re dealing with GCA. And of course, GCA can kill people. the big vessel we’re worried about is the aorta, causing aortitis and of course if you need your aorta and if you have aortitis, that definitely can kill you. Five things I want you to remember about acute visual loss in the elderly. One is giant cell arteritis and the other four are giant cell arteritis! It should be top of mind every time with elderly person with headache, and transient ischemic attack, and transient vision loss are also on the differential. you can here’s a case with a 75-year-old woman. And this was sent to me as hypertension because there were these multifocal cotton wool spots here and the disc didn’t look swollen, but the blood pressure on this side is the same as the blood pressure on that side, and sometimes people have such severe pain that they have hypertension as a result of their giant cell arteritis. So you should be. If you see multifocal cotton wool patches is multilevel ischemia and that could still be GCA. This person shows nonperfusion, not only of the disc, but the peri papillariry choroid. This suggests two circulations and that means vasculitis until proven otherwise and the most common is of course GCA. And we’re going to do the temporal artery biopsy and we’re going to be looking for these giant cells, but you don’t have to have giant cells to have giant cell arteritis, just like you don’t have to have temporal pain to have temporal art atritis. You have to look carefully at this biopsy report because what we’re looking for is disruption of the internal lamina. And lymphocytes or macrophages at that same location. Basically you don’t have to have giant cells to have giant cell arteritis. Sometimes there’s nothing seen because no artery was identified in the specimen, because you actually biopsied a vein or a piece of connective tissue. So you should be aware of these unusual ocular presentations of GCA, that’s multifocal cotton wool patches, posterior ischemic optic neuropathy. Nonembolic central retinal artery occlusion, and transient visual loss or transdiplopia. And simultaneous choroid and optic, choroid plus retina, retina plus chore choroid, any combination is giant cell until proven otherwise. So here’s a person. Of course the OCT of the nerve and the macula are going to be normal, because there’s nothing to see. Angiogram of course proving that this is a P I/O N, a posterior ischemic optic neuropathy with choroidal perfusion. And so the key thing to do in setting is perform a confrontation visual field. By the way, confrontation is this kind of field, confrontational is this kind of field, so please be confrontation, not confrontational. If you wrote unreliable visual field. You’re trying to find a bitemporal, hemi opensa. There’s no field machine in the emergency room in the hospital and so if you are seeing the patient as an emergency, you really have to know how to do a confrontational field. Severe headache, young person, bitemporal hemianopsia, … Pitt tumors are pretty common. Might be as high as 1 in 500 in elderly patients and the average ophthalmologist should see one Pitt tumor per year or you’re missing your quota. Gentleman and apoplexy is dangerous because it’s potentially life threatening. Of course it’s acute onset. You should take any acute or painful loss of vision very seriously in any age really pituitary apoplexy: The pituitary is the master gland and you can do without a lot of your hormones, but you can’t do without cortisol. Intracellular mass, this rim of hypersignal intensity is the blood from the apoplexy, but there’s other forms of apoplexy including this necrosis form, which is hypointensity and you can see here, my poor chiasm is being worn as a hot producing the bilateral visual loss. If you look at this series from Neurosurgery, 2005, 62 patients, average age 51.1 years, average time to presentation, was 14 days, so they had their headache two weeks ago, then they go to the bed, they don’t go to the distribution they wake up, they feel better, and then they call the office. 81% had no previous history of pituitary humor. And headache was the dominant symptom, but look at the visual acuity loss rate, 56%, and by temporal hemianopia, the thing you’re looking for in the field, 34%. 73% had hypopituitarism, and 8% had diabeticees insipidus and if you have DI, you might D-I-E. Apoplexy is a clinical diagnosis, it’s characterized by acute and severe, that’s what apoplexy means, not a radiographic diagnosis. We’re using the MRI to confirm what we already suspect. And so the wicked good pearl is: Please do a confrontational visual field if you’re worried about bitemporal hemianopia. Going to switch to the third case now, which is acute ophthalmoplegia in a diabetic. Now, normally a diabetic is allowed to have ophthal plegia from diabetes, small vessel, that’s a very common scenario for small vessel ischemic cranial neuropathy. However, the key differential feature is diabetic ketoacidosis, so if you have diabetes and diabetic ketoacidosis and this patient has pleat left-sided ptosis. So we’re not allowed to have a 3 and a 4. Or a 2 and a 3. Or a 3 and a 5. You must check the cranial nerves. So we’re going to be checking all the cranial nerves and an ophthalmologist should be at least able to count No. 7. You need to check cranial nerve 2, 3, 4, 5, which is the one that most people skip. 6, and bonus point for checking 7. But the key and dangerous portion here is almost complete ophthalmoplegia, so the eye is down and out, what we’re looking for is intorsion and downward gaze. A diabetic is allowed to have exactly one cranial neuropathy at a time. And once you have a combination, that really is no longer a diabetic ischemic, and more likely to be acute compression. And in a diabetic in DKA is Mucor. The life-threatening diagnosis here is uncal abscess and so here’s what it looks like in MRI. Sphenosinuss are opacified and there’s extension into the cavernous sinus producing ophthalmoplegia. we don’t want to be giving steroids to fungus. If you give steroids to fungus, you’ll get fungus among us. And even though DKA is the book answer, please don’t procrastinate on seeing acute, severe, or painful ophthalmoplegia in any of these settings: Cancer patient, long term immunosuppression, chronic renal dialysis or organ transplant survivor. From the pituitary gland, that’s apoplexy, if it goes upstairs, bitemporal hemianopsia, and then the sphenoid sinus, diabetic DKA, the fungus can jump right in the cavernous sinus, and if big red gets involved, you might die. So this is a case of mine from Iowa, she was on acute myelogenous leukemia. She had two days of erythematous swelling, the resident went up and said a little bit of swelling in the eye. Is this really orbital inflammatory pseudotumor or the painful steroid response of ophthalmoplegia syndrome? The wicked good pearl here is don’t give the diagnosis of autoimmune disease to people who don’t have an immune system if you’re immunosuppressed, you should be thinking infection, infection infection. And of course, ENT went up the nose, saw this pale, necrotic tissue and biopsied this and it proved to be fungal hyphae, mucormycosis. In the book it says look out to this black eschar thing. Please don’t wait for the black eschar thing. By the time that comes, it is game over. And you you might be asking yourself, how could we miss fungal orbital apexes on an MRI. Well, these patients are often sick and so sometimes they can’t have the contrast material because their kidneys and GFR is not good enough, and you need to have fat suppression or you can miss the lesion. These are super-dangerous cases because it’s very tempting to give them steroids and if you give steroids to fungus, it will be bad. So the MRI scan could be normal, so we recommend doing a CT scan if you’re worried about fungal disease, so we can see the sinuses and that bone and it’s just way better for seeing what we need to see. The distinctive radiographic sign on MR, however, is enhancement in the adjacent sinus, so whatever they see, radiology sees is adjacent to what you’re trying to see, that is the key feature that it might be fungal disease. So enhancement of the orbital sinus next in your optic nerve, that’s the dangerous thing that we’re looking for. And you can see in this case of aspergillosis of the orbital apex, the adjacent ethmoid sinus is also enhancing and you need to give contrast, because if you don’t give the contrast, you can’t see anything. That’s my house at night. You need to use fat suppression, because the fat is very bright on T1, so this is T1 and in fat, we kind of use the fat to tell this is a T1 signal. Fat in fact is our friend. So this is no fat suppression, no contrast yet. This is post contrast study, you can see the contrast in the nose and in the cavernous sinus. Can you tell if this nose is enhancing? No, I can’t either and the reason is the fat, who is normally your friend, is bright and so is the gadolinium and if you put white stuff on white stuff, you get white stuff. And this MRI scan might be read as normal when the real reason is hey, you didn’t give fat suppression and we can’t see the. polar bear in a snowstorm. White stuff on white stuff. You can see here the MRI scan says there’s nothing in that sinus, but the same-day CT scan shows it’s actually full of fungal hyphae, so the dark T2 signal, can mimic air and therefore CT scan is better in the acute setting for these cases. Getting to the home stretch here. This patient is a 60-year-old diabetic. You’re going to be very tempted to think this is diabetic third, diabetic third, but you have to check the pupil. So before making the diagnosis of ischemic nerve root palsy, come back in 6 weeks, we need to look at that pupil. Because an acute pupil involved third nerve palsy is an acute nerve root — you need to look for aneurysm and the MRI in the head scan was normal again and that’s because you need an A to find that A. The A we’re looking for is aneurysm, the A to find it is angiogram. We have to have some kind of A here. There is no A in MRI. CTA, MRA. Digital subtraction angiography, we have to have an A here. In the acute setting you’re going to use CT, followed by CTA and then MR followed by MA. Here’s a jalapeno pepper pressing right on the third nerve. It runs right next to the posterior communicating artery in this location and the pupry artery is on the left side. You can see that neuroophthalmology is a very small piece of the insurance claims pie. But if you look at the average malpractice settlement, neuroop is a leader here. I would say the things we’re most asked to look at are things on this list. This is an acute painful anisocoria, they’re going to be complaining about the little ptosis, so little ptosis plus little pupil is big problem. Big ptosis with big pupil is a big problem, but little pupil, and little ptosis is also a big problem. You can use drops, which will cause reversal or cocaine or all these drops they talk about in the books, but in the real world just image this patient, and the thing we’re worried about in a patient that has a Horner’s after an accident is. The wicked good pearl is: In the acute setting, just image the entire sympathetic pathway for the Horner syndrome. Yes, you can do the drops, but if you don’t have the drops, who cares, if you think it’s a Horner syndrome, it is carotid unless proven otherwise, because the pathway of the sympathetics in the eye extend posterolaterally. And the things we’re worried about are downstairs, below this line, crotic dissection and if it’s arm pain and chronic, the P anko’s tumor. I love this sign. Touching wires causes death and also a $200 fine. You’re gotping the idea that the MRI scan is not going to be helpful in a lot of conditions in this list and the reason the MRI head was normal is you didn’t image the neck. So we have to image the entire sympathetic pathway in the Horner syndrome, in the acute setting that’s CTA of the neck and you need an MRI of head and neck to follow. And what we’re looking for is this distinctive feature on the MRI of the head and neck is this hypercrescent. We’re going to call neurology, they’re going to get admitted in the hospital. I just want to emphasize the case of amaurosis fugax. This is a 70-year-old woman judge. She had a normal eye exam and she was given the diagnosis of ocular migraine. You should be very, very careful of making this diagnosis of clinical ocular migraine which I don’t think really exists. The dangerous part is normal eye exam so it’s going to be very tempting to tell this lady, look, we didn’t find anything, why don’t you call me back if it happens again, well, this is a TIA. And the altitudinal offset that’s that curtain coming down. Is there life after death? Trespass here and find out. So please don’t trespass on transient altitudinal loss. You need to call the stroke service for a stroke workup at the stroke center by a stroke doctor so that she doesn’t get a stroke. And the MRI scan ahead might show DWI abnormality, but it could be normal because it’s a TIA. It’s transient. And we have to image the neck again. And the reason is it could be ipsilateral hemodynamically carotid disease and what we’re looking for is this plaque in the neck, if the plaque flies off and goes in your head, you get transient hemi numbness or whatever TIA symptom, but if it goes in your eye, you and of course if the embolus goes into the retina, you get this cherry red spot and it’s central artery occlusion. You should know also that it can affect the vertebral system and the vertebral dissection is the posterior is and so you can either have vertebral symptoms on the ipsilateral side, the contralateral side or both. And that’s because the basilar artery backs the posterior. If you get the initial imaging study it might be normal and they have to be admitted to the stroke service for a stroke workup and stroke follow-up and that’s especially important in patients who have transient diplopia or transient bilateral vision laws. An the absence of the trauma doesn’t mean it’s not a spontaneous dissection and the dangerous thing about carotid and vertebral dissections is it can populate. The crescent sign, but instead of it being the front artery, it’s going to be the back artery, so instead of it being the carotids, it’s the two vertebral arteries and you can see the hyperintense crescent which represents the blood in the false lumen and in this particular example, the patient has a lateral medullary infarct corresponding with this dissection. Should call stroke service for both carotid and vertebral dissections, it is an emergency. So in summary, you should know now the five potentially life threatening diagnoses in neuro-op, you should be able to define the rule of the pupil but in general we’re going to recommend imaging any third nerve palsy regardless of the level of the pupil. If the pupil is not involved, it’s not an aneurysm; that’s not true at all. if the CTA is negative for any third nerve palsy, but if it’s pupil involved, you should admit to the hospital. That’s the rule of the pupil. Sometimes it’s not an imaging study like giant cell arteritis, even though you and the more dangerous thing, the MRI head scan might be normal in many of these cases either because the fungi are dark or you didn’t give contrast or you didn’t use fat suppression or you didn’t use an A to find that A. Aneurysm needs angiogram, or you imaged the wrong area, you MRIs their head when you should have been looking at their neck. I’ve showed you the key clinical and/or radiographic I feature for all of these, but really your job is not to be a neuro-ophthalmologist, you don’t have to like neuro-ophthalmology, but you have to RVSCT. Recognize, triage and refer these five conditions to the hospital. So the mean take-home message is, what does your list look like? My dad would say when he’s listen to a lecture, a good lecture is his lists list is things he needs to do differently because of that lecture, because a great lecture was a list of patients that he had to call back, because he knew that they needed something more. A bad list is if it was writing down a grocery list for the things to pick up on the way hot so what does your list look like right now? Should look like this: Acute headache, acute or transient vision loss or AION in, PRON is GCA arteritis until proven otherwise in elderly patients. Acute orbital apex syndrome especially if they are in DKA or any immunosuppressed patient should be considered to have Mucor in many proven otherwise, fungal abscess. You should be worried about acute painful, but you also have to worry about little pupil and little ptosis, that’s the Horner syndrome and that is arterial dissection, either on the carotid or vertebral side. That means stroke workup by stroke doctor at stroke center. Acute painful bitemporal anopsia apoplexy unless proven wise. Acute painful homonymous on the arterial side rather than the carotid side. The bottom line is it’s job, cataract, cataract, cataract, you have to go back and scrape that off. You don’t have to dispose of the possum, but you got to get someone else to come and take care of the possum before you continue on with cataract, cataract, cataract, glaucoma. It’s your job, recognize, triage, refer. And I thank you for your time and attention and I believe we’re going to have time for some questions and answers in a little bit. But I just want to end with this last little thing because we have a little bit of time which is a philosophical question. Why are here with me at 7 a.m. in the morning Central, because you believe that you make a difference in this planet. I want to tell you the story of my own life, which is on July 20th, 1969, I was 5 years old when this was on our TV careen screen. It said this live from the moon, Houston, my home town was the first word spoken that day, tranquility base here. Houston, we have a problem. They made it into a movie called Apollo 13 with Tom Hanks, that’s me with commander Lovell, this is the problem. Half the spacecraft was lost. An explosion occurred, blew off half the spacecraft. That’s a big problem. Houston, we have a big problem. That’s my daughter Rachel a long time ago, she’s holding up three fingers to indicate it takes three fuel cells to return home from space. They did not call up to Jim Lovell, they said this: Failure is not an option. That’s my daughter. She’s in college now. She’s standing next to actual human control computer tower. That was on my TV screen live. Now it liches in the Smithsonian and you can stand right next to it. Most of the computing power to put man on the moon and to bring home Apollo 13. It is human brain power, it is why you’re here today. It is human brain power that drives everything that we do. And that is the message of today’s talk. Today I get to fulfill my childhood dream and work with real rocket scientists at NASA, Johnson Space Center, Houston, Texas, that’s what I was thinking on July 20, 1969. Instead, I became this, and I truly believe that one person can make a difference, but that everyone should try. John F. Kennedy. So I thank you for your attention and I think we have time now to move to our questions and I’ll see if Lawrence can make that happen. Lawrence: Perfect, thank you, Dr. Lee, if you open up the Q & A we have I believe 11 questions at the moment. >> Dr. Lee: All right, can you repeat the five diagnoses? Arteritis, abscess, aneurysm, apoplexy, arterial dissection, >> Question formal verification 2, so if you have an elderly patient who reports a TIA and you don’t see a plaque — no, if you have a TIA, you should send the person to the emergency room for a stroke workup by the stroke doctor at the stroke center, because it might become a stroke. If you want, you can order a stat ESR and CRP, but really, they gotta go to the hospital. Is there a protocol for pharmacologic thromboulitis? Yes, if they’re candidates for TPA, the thrombolytic therapy, they can receive this therapy, that’s not your call. Your call is to make the call to the person that makes that call and that person is not you, it is stroke neurology at the stroke center by the stroke doctor. You can give TPA but only if you’re in the window and most of the patients when with vision loss don’t make it to the hospital in time to have a TPA. But that’s not your call. So the sedimentation rate just increases your likelihood ratio he, pretest probability of disease is what drives the decisionmaking, however. And if you have a pretest that it is GCA of 90%. Headache, pallid edema, it doesn’t matter if the sed rate is normal. We need all three, sed rate, platelet count and … So if a patient has a fixed and dilated pupil, can you still test for an RAPD. You need two eyes, but only one working pupil to detect an RAPD and that thing is called a reverse RAPD. So if the pupil is fixed and dilated and you swing the light from the left eye to the right. The left pupil, the uninvolved pupil will dilate when you swing from the left to the right, that’s called a reverse RAPD. And if we see a reverse RAPD and a third. That’s orbital apex or apoplexy. There are a number of questions here about show us the list again, and you can just go and this talk was recorded. I know that I speak relatively quickly, but you can just watch it again if you want. And if there’s — a lot of these questions, actually, are on our YouTube site, can you search the YouTube site and it will answer a lot of these questions. So carotid Doppler, should it be done by radiologist or cardiologist? That again is not your call. So carotid Doppler is usually done in the hospital. It’s not your decision to decide who does it. Can a longstanding pupil involved third nerve palsy — so that is the same, even though it’s one month back, it still could be aneurysm, that still needs the same workup. Can you explain about migraine and visual problems? Yeah, migraine normally causes bilateral simultaneous transient visual phenomenon. It’s often a squiggly line, with scintillation, either color or flashing. It lasts 20 minutes and then the headache has to phenomenon. The reason I don’t like the ocular migraine or the retinal migraine is because it’s not bilateral. We usually call those unilateral cases, that we used to call migraine retino vasospasm. You have to have migraine to have retinal migraine. Is there a role with … — (reading fast) yes, you need to do IV steroids not only to protect their eye. The IV steroid is to protect them from the other large vessel vasculitis problems including aortitis, so it’s not just about the eye. Next question. Yes, so any patient who has high myopia, you’re going to want to do a baseline exam so you can document exactly that that nerve looked like. There are a number of choices h ultrasounds for calcified Drusen, but OCT is really great for Drusen, too, it shows a hyperreflective core with a hyperreflective rim. OCT can also display the main symptoms of typical papilla edema. Your main role is to document, document, document. You’ll have a clear record that it is pseudopapilla edema. Do we have a blood test for Mucor mycosis? No, there’s no serological test for the Mucor. It’s a biopsy disease. So ENT has to do the biopsy to look for the fungal hyphae. If we have nonseptate hyphae, you really should be thinking about Mucor. The patient with a temporal artery biopsy is performed by an ophthalmologist or neuro-ophthalmologist, can you share any videos? There are other sites that have the whole biopsy procedure on video and so that’s one of the great things about the internet. You can just go and watch these videos. How do you diagnose ophthalmic artery occlusion, it looks like a central artery occlusion, but in CIOAO, you have a cherry red spot. In one of the clues that if it’s an ophthalmic artery occlusion instead of central artery occlusion. The cherry red spot is actually the normal part of the fundus. The other thing with ophthalmic artery occlusions is the ophthalmic provides the motor supply they might have full-on objecting already ischemia, cataract, neovascularization, so these are the things we worry about with more proximal disease like the ophthalmic artery occlusion. That’s akin to the ophthalmic artery questions. It’s uncommon, but it’s also a symptom and sign of GCA. So any ischemia whether it’s in the front of the eye, anterior, middle of the eye, or back of the eye, or behind the eye, all are GCA. Can we diagnosis GCA without a biopsy? You can, but the temporal artery biopsy is the gold standard for giant cell arteritis, and I would prefer that you have a biopsy proven disease, because patients that are treated with steroids often develop side effects, especially elderly patients and some of those sigh effects are potentially life threatening, so it’s much harder to explain to the patient and their family about the side effects if you don’t have a biopsy. Also it keeps from now, when do we get away from not doing a biopsy? Well, the patient is not a surgical candidate or they refuse to have a biopsy. If it showed the halo sign in an ultrasound, then maybe you could get away without the biopsy. One day the ultrasound can be good enough to get rid of the biopsy, but right now the biopsy is still the gold standard. A patient with a dilated pupil post trauma for one to two weeks, no requireis trauma, no headache, should I suspect the patient for PCA aneurysm? An isolated fixed and dilated pupil is almost never an aneurysm. So what you’re looking for is third nerve palsy signs, ptosis and motility deficit. If it’s just a dilated pupil alone in isolation, that’s almost never a third nerve palsy. What you should be thinking about is pharmacologic dilation or something wrong with your Iris, and so that is where you could do the topical testing with pyelocarpine, 1/10th dilute pilocarpine, you’re going to be looking for vermiform movements, sector involvement. And in trauma cases they can definitely occur because the ganglion can be damaged but you better make sure there’s no ptosis or motility deficit. The other is something is wrong with the Iris itself and that’s 1% pilocarpine and if it doesn’t dilate the pupil, then you know it’s pharmacologically blocked or not third nerve palsy. Can it present as blur or total vision loss? So amaurosis fugax miens means blindness, and it means fleeting, so it’s a fleeting blindness, it doesn’t have anything to do with the severity. However, the more severe it is, the less likely it is to be something benign, so things like dry eye can cause transient visual blur. That’s not amaurosis tag ax fugueax. Is there any correlation between optic disc Drusen and migraine? Not directly, but patients with migraine headache often have Drusen as a co-morbidity because both conditions are so common. I don’t think it’s a causal association, they just are both common things. After long-term steroids do they have to take low-dose steroids for maintenance? The answer is yes. I Most patients are on steroids for months to years. The average duration for us is usually between 18 and 24 months. We usually try to switch to a steroid-sparing regimen if they get any significant side effects and the most promising one is Tosolizumab which is an interleukin 6 blocker. So I would recommend all of these patients get seen with a doctor who can help you with managing the osteoporosis and steroid-related side effects. (Question) When you have that combination of things where you have retinal fluid serous detachments and disc edema in the setting of preeclampsia, that’s almost always hypertensive. So there’s grades of the hypertensive. Grade 1, Grade 2, Grade 3 which is the hemorrhages, the exudates and can cause serous detachment and grade 4 is the disc edema. So the combinations. That’s eclampsia, in a pregnant patient, in a non-pregnant patient that’s usually hypertension. And that’s hypertensive emergency so if they have elevated blood pressure with Grade 3 or grade 4, including the disc edema, that’s an admission to the hospital. So that’s another one of these admit to the hospital things. IIH, can it be cured? No. Instead of performing an LP, are there other things to use to check the ICP when there’s papilla edema, there are things that have been proposed but the only way to is to mesh the opening pressure. I personally feel that all IIH patients need to have a lumbar puncture. What is the role of orbital Doppler in TIA, how accurate is it? So orbital Doppler is fraught with all sorts of problems, so as opposed to carotid Doppler, we have Direct Access to the carotid and it’s relatively clear that you can get clear diagnostics. Get to go the it’s very difficult to use orbital Doppler in TIA. Transcranial Doppler, however, can be used. So the Doppler of the head and carotid are good, the orbital one, iffy. How often do you observe mandibular claudication? That’s jaw claudication, that’s a common symptom at final diagnosis, but it’s a very rear initial symptom. It’s characterized by pain when you chew. If it’s present, it’s highly suggestive of giant cell, so it’s a very specific sign, but its sensitivity is terrible. So you cannot rely upon the absence of mandibular or jaw claudication to exclude giant cell. How do you further workup suspected retro bulbar optic neuritis is an inflammatory optic neuropathy and 95% of the time, the nerve is enhancing. The most common reasons for the MRI to be normal are, you didn’t do enough contrast, you didn’t do fat suppression or you didn’t image the orbit. So by normal MRI it really needs to be — even if it’s negative, 4% of the cases still have retro optic bulbar neuritis. If you think it’s retro bulbar optic neuritis, it’s great if you see enhancement, but if you don’t see enhancement, that doesn’t rule it out. 20 to 25% of those patients actually do have MS even though they have a normal MRI at time 0. You still have to think about MS, the MS mimics, and the MS-like diseases. What kind of Doppler can you use to — you can try to use the Doppler again on the ophthalmic artery occlusions but really it’s a clinical diagnosis, some of those patients have to have conventional angiography, the ophthalmic artery is very small, so CTA, MRA, you can try. It’s a clinical thing. It’s hard to use the angiogram for that. If you’re going to do an angiogram, fluorescein angiogram. How do you differentiate if it from retinal migraine … So if it quacks like a duck and flies like a duck, and looks like a duck, it’s probably a duck. So it quacks like this, it’s migraine, scintillating fortifications, bilateral, moves across your field, buildup, starts small, gets bigger, 20 minutes and then headache follows. If you have that, it’s migraine, it’s the unilateral ones that are much more dangerous and if you don’t have the scintillation, skin toma, the fortification, you have to do a visual field on this person a mazingly the more times it happens without a residual, the less likely it is to be something bad. So if you have hundreds of episodes that are stereotyped and been there for years, yes you can do the workup, but a young female it’s extremely unlikely you’re going to find a cause, and actually the more times it happens and the more stereotyped and the more frequently it happens. The hormones in a fertile female matter a little bit. You should think about birth control pill, you should communicate these results to the PCP. Lawrence, should we continue or should we wrap it up? >> If you have a few more minutes, there are some questions, but feel free to wrap up whenever you need to leave. >> Dr. Lee: All right, we’ll try to do these. You should test their side vision, because the Humphrey 30-2 only tests the central 30 degrees, so you have to do a confrontational field. The Humphrey doesn’t have to do a 30-degree field. It can do bigger fields. If you really feel it’s a real field defect and it’s in the temporal crescent, that needs to be imaged. Can giant cell occur suddenly? Yes it can occur often suddenly, that’s the normal, acute, but it can also be subacute and chronic. Herpes zoster can cause ophthalmological plegia, third, fourth, sixth, it doesn’t have to be — any cranial nerve. Can scintillatingsy toma cannot last for a week. You have to work that up. However, migraine can be status migrainosis, which means it persists. You really need to make sure it’s not seizure and status epilepticus and some of those patients of course, are non-organic. Please throw some light on the Alice in Wonderland syndrome. So Alice in Wonderland is a book and Alice goes down the rabbit hole and she thinks things are bigger and smaller and she meets some wacky people and the mad hatter. So patients with almigraine often have Alice in wonderland symptoms. Making sure they’re not on hallucinogens and other things, because maybe Alice was on drugs. If it person has amaurosis fugax amaurosis minds blindness, fugax means fleeting. Should we believe that IV methylprednisolone has no role in treatment. It should be given to patients who have vision loss because you’re really can’t tell how much they’re going to get back. There’s never been a head to head trial, but pallid disc edema may recover, still, so we’re trying to reduce the ischemic event and also if you had giant cell, you’d probably want IV methylprednisolone. So a monocular temporal hemianopia, that thing is called the junctional skittoma of — it can occur transiently, but it’s kind of rare. You need to make sure it’s not the temporal crescent, which is in your occipital lobe, rather than temporal hemianopia. You can do an LP as many times as you want, but you won’t have many patients because after about the 5th time they’re not going to want to come backnym. If you need to keep doing LP you need to think about doing — the one time when we do serial LPs is in pregnant ladies who don’t want to have surgery. It’s not really the kinking that causes the problem. If the doleo could he cook attic artery. You really have to have a lot of mass effect. So it’s not the kinking, it’s the mass effect. Differentiate between foster Kennedy and pseudofoster Kennedy. So foster Kennedy is — the most common cause of the true foster Kennedy syndrome is a mass fundal olfactory, however, if you do an imaging study and it’s negative, pseudoFoster Kennedy just means — and then developed an acute — and the most common cause of that is nonarteritic anterior ischemic optic neuropathy. Three episodes of unilateral painless loss, nonscintillating lasted a minute, otherwise healthy young male, yeah, so that’s the appropriate workup. You do need to work it up. But after 30 or 100 such episodes, no more workup. so I think, Lawrence, we made it through all the questions.