 I'm gonna keep this fairly short, but this is one of our neuroothemology presentations that we are required to do as residents, and so I believe this would be my last, correct? Yay, good. I'm really excited, actually, to present this particular case because it is neuroothemology related, but it was kind of the residents putting their heads together to figure this out in our general clinic at the VA, so I'm pretty proud of this. So I entitle this diagnostic confusion, and you'll see in a second why I entitle it that way. So this was a 68-year-old male veteran who, again, was seen at the VA and general clinic, first presenting with double vision and a little bit after the onset of that with the droopy eyelid. As far as the history, two weeks prior to his presentation, he had the sudden onset of binocular diplopia, which he described as constant, and then a week after that, the droopy left upper eyelid started to bother him. On the day of exam, he mentioned that the diplopia itself had gotten worse where the images were described as being farther apart from each other than they had previously been. He had some significant things on his past medical history, namely diabetes, hypertension, cornea artery disease, sleep apnea, some thyroid issues as well, hyperparathyroid as well, and he also had metastatic renal cancer for which he had an nephrectomy performed as well as an adrenal ectomy. He had also had a significant heart surgery in the past as well. Regarding his diabetes, he did have proliferative diabetic retinopathy, status post, PRP, focal laser as well. He also had both eyes vitrectomized. He had cataract surgery done in both eyes and a yag in the right eye. Social history, being in the military, he was exposed to Asian orange, but he did not plan to have any tobacco, alcohol, or drug use in the past. He was on insulin and synthroid. As far as his review of symptoms of simply he denied any dysphagia in the past or any breathing problems, dysarthria, or muscle weakness, these were some of the specific questions that we were trying to ascertain or answers that we were trying to ascertain in our exam with him. Vision-wise, he had very poor vision in both eyes, as you can see there, and that was basically due to his proliferative diabetic retinopathy. Pupils, he did not have an afferent pupil. People are a defect and there was no anisecoria either. His extra octer movements, as is shown here, showed decreased superduction of the right eye, but similar in all other directions. Because of his poor visual acuity, we were not able to do a cross-cover or alternate cover testing on him. So instead, we did a single-matics rod just to try to pull out some of the subtleties in this. We noted that he had, in primary gaze, a right hyperotropia, which improved in left gaze, and were similar in both head tilts to either side. But in up gaze, it seemed to change to a left hyper. And so, right there, I was pretty confused in trying to figure out what's going on with his extra octer movements. He also has a droopy eyelid. So we did measurements of his ptosis. I have a picture that I'll show you in just a second. He had left ptosis. And you can see the differences in all of our eyelid measurements that the ptosis was significant on the left side. We also tested a Kogan's lid twitch, which was negative. There was no fatigability on exam either. On slatinum examination, we didn't know that he had posterior chamber IOLs placed. And on dilated fundus exam, also we saw the PRP, the laser scars and other focal scars. So as far as our differential diagnosis, just real quickly, we thought that it wasn't significant or kind of pointing towards a fourth cranial nerve palsy, but it might be a pupil sparing third nerve palsy possibly with his diabetes history, Graves disease. We know that he did have some thyroid issues. Mycenae gravis was also on the differential and a skew deviation. So this is a picture of him, as you can see, significant droopy upper lid on the left side. And so trying to put all this together and summarize it, we noted that this patient had a sudden onset of changes, both with diplopia as well as the lid changes that seemed to fluctuate a little bit, becoming more constant over time, diplopia getting worse over time as well. He didn't seem to have any proptosis either on exam, but so what we decided to do was an ice test on him. He said, well, let's just try it and see what happens. So this is pre-ice test and two minutes after applying the ice, that's what he looked like. That's pretty significant change and we're all pretty excited about that. I don't think I've ever seen an ice test as positive as this, but again, I haven't done a whole lot of ice tests either in the past, but this was literally pointing to the one differential diagnosis of myocinogravus. And so that became the top of our list and as a result, we did send it for a lab testing. Specifically, we looked at the acetylcholine receptor antibodies, which was elevated, as you can see in the parentheses, that would be the normal range, so it was 1.73. We also tested his thyroid panel, which all seemed to be within the normal range. And as per protocol, when you're trying to diagnose or look into or evaluate myocinogravus, we also sent him for a CT looking for a thymoma, which was negative on imaging. So just a brief overview. This was a great case for the residents to kind of confirm what we have learned about dealing with myocinogravus. It is an autoimmune disorder, the hallmark of which is weakness that seems to improve with rest, and pathophysiology wise, it is due to immune complexes that block the acetylcholine receptor, decreasing the neurotransmission at the neuromuscular junction. 90% of patients will present with ocular problems and so it's not uncommon for ophthalmologists to be involved right at the onset and maybe even do come up with the initial diagnosis. Tosis is the most common sign, but dyplopia is also fairly common as well. The dyplopia issue can mimic a number of different problems. It can mimic any of the cranial nerves, more commonly a sixth nerve palsy or partial pupil sprain third. It can also mimic an INL or a total ophthalmoplesia, as well as gaze palsies or it might even mimic an isolated muscle palsy like an inferior oblique. Obicularis oculi weakness can also be found on exam, but it will not have or involve pupils. As we all know myocinogravus is not always just an ocular type of disease. Systemic findings can also be involved including weakness in other muscle groups in the body. The muscle is used in chewing, neck, trunk, even limbs. Dysphagia, hoarseness, dysarthria and dyspnea can also be indications of systemic involvement as well. Thyroid eye disease can also be associated with myocinogravus in about 5% of these patients. So as you can see with exam findings the way the patient may present, it can often lead to confusion in trying to sort out what is true, what might be actually related to myocinogravus or possibly something else. But again the hallmark that we noted in our case and we should keep in mind is that there is fluctuation and fatigability involved. So on exam we need to always check pupils and make sure that it is not involving pupils. If the pupils are involved we need to start thinking about something else on our differential rather than myocinogravus. Eyelid measurements and proptosis as we looked into extraocular movements, trying to get a pattern if there is such a pattern. Testing the strength of the auricularis oculi muscles having the patient squeeze really tight and trying to actually open up manually their eyelids. Testing for a Colgan's lid twitch. I think many of us have done this and basically it's just eliciting a saccade from down gaze to primary gaze or up gaze to see if there's an overshoot of the upper tautic eyelid. After which you'll notice that the lid will kind of settle back down to its tautic state. Fatigability can also be tested in a clinic where you'll have the patient and sustained up gaze and you'll notice that the tautic lid will become more tautic. Enhancement of the ptosis as well. If you lift the more tautic eyelid manually, you'll notice that the other eyelid will actually drop and that is in accordance to Herring's law. You can also do these other tests in the clinic, namely a Tensilon test. We weren't about to do this at the VA. I'm glad we didn't but you can administer a Grygeophonium by IV. You need to make sure that you have atropin on board because it can cause bradycardia and other issues. But you can also do a sleep test which again is a very easy test to do. You have the patient sleep for about 30 minutes and see if there are tautosis or extra octor movements improved. And as we did in our clinic, the ice test. Very easy test to do. It only takes about two minutes and you can see the effect that we got in this patient. Lab test. The binding antibody is the more common test to do. It's found in about 90% of patients with general myosinographis and 50% with ocular myosinographis. You can also test for the blocking antibody as well as the modulating antibody. You can also test for the musk antibody. And usually that is done if the suspicion is still high but the other antibodies are negative. And as we did, we should also test for thyroid as well because of the possible association. Single fiber EMG can also be done. It's the most sensitive for myosinographis and you would see a characteristic decremental response with repeated stimuli as well as doing a CT scan for a thymoma which can occur in about 10% of patients. Treatment-wise, our patient ended up being put on, messed it on, but you can also use corticosteroids and other immunosuppressant medications for the eye problems, prisms, patching, tostis crutches. I've never seen those but I've seen pictures of them. They seem to help with the droopy eyelid. And even strabizma surgery can be offered if needed. A thymectomy if a thymoma is present also makes sense. Short-term treatment would include IVIG and plasma peresis. Now, about 85% of patients with just ocular symptoms will tend to develop systemic findings over two years. But as we did with our patient, we got our patient involved in neurology as well as neurothemology, plugged him into the clinic and he also was sent to the neuromuscular clinic for further management as well. I just wanted to thank Dr. Warner and Grant Morcetti who we work together on this case. Thank you very much. Any questions? It's a good review. Thank you.