 All right. Good morning everybody. We're gonna get started. So our first presenter is Victoria Holiday. She's a third year neurology resident who did her undergrad and medical school at the University of Kentucky. And then she's been doing her neuro-opth rotation with us for the last few weeks. She's gonna talk to us about a case report on idiopathic intracranial hypertension. Hi everyone. I appreciate the opportunity to speak today. And I just wanted to present with you an interesting case that I shared with Dr. Judith Warner and Dr. Arefe Adatina that I saw in clinic with them of fulminate idiopathic intracranial hypertension. So the patient that we saw was a 31 year old right handed woman who presented with the complain of headache and vision loss. And she actually presented to us on day 12 of her symptom course but I wanted to present to you the history as she gave it to us. So on day one of her presentation she woke up with a new very severe headache that she'd never experienced before. That was a severe sharp pain over her occipot as well as pressure over the forehead on both sides and was associated with phonophobia and photophobia. Now she did have a history of migraine so it didn't initially think very much of it but this seemed different. And by day of her symptoms she had developed a gray spot in her left eye that was persistent. Her headache had become worse associated now with nausea and vomiting and was fairly constant and then two days later unfortunately developed a gray spot in her right eye. The next morning she presented to the emergency department and was admitted from the emergency department after an MRI of her brain as well as an MRV of her brain that were both red as normal. She had a lumbar puncture that showed an opening pressure of greater than 55 centimeters of water and they removed about 25 cc's of CSF at that time. They sent it for standard studies including a cell count culture, grand stain, protein and glucose and all of that returned normal and she had a transient improvement in her headache for about 12 hours. She was started on acetylzolamide with the presumption that this was adiopathic intracranial hypertension and discharged home. Unfortunately that was her improvement was short lived and she continued to get worse with the gray spots in her left eye continuing to grow larger and severely obscuring her vision. She had a central blind spot that she actually reported seeing hallucinations of things like snakes and insects within that blind spot as well as blurry vision and gray spots in the contralateral eye. Then she presented to us on day 12 for evaluation. In talking with her and getting further history she did report a weight gain of approximately 30 pounds in the last year but no exposure to tetracycline or steroids, no dyplopia and no eye pain. Her physical examination at the time of her presentation was a blood pressure of 145 over 99 and a heart rate of 123. Her weight was 260 pounds which was a BMI of 28.4 putting her in the morbidly obese category and her visual acuity was 20, 25 in the left eye, I'm sorry in the right eye and 21, 25 in the left eye. She had a large APD, a 1.8 log APD in the left eye but the remainder of her neurologic exam was normal. This is a photograph of her fundus and I know it's really hard to see but this is kind of the best we could get on the day that she presented that demonstrates grade 5 papillodema and that was true for her bilaterally. She had peripapillary hemorrhages which I hope I can convince you is that dark area right there but also severe disc swelling with obscuration of all of her major vessels. She had visual field testing and this was her right eye which just showed some constriction of her visual field but in her left eye which was her more symptomatic eye, this was her visual field. Unfortunately she had a very large, she had an enlargement of her blind spot and a severely constricted visual field in the left eye. So with these findings and the acuity of her presentation we were able to arrange for her to be transferred to IMC for a lumbar drain that evening and she was continued on her diamox during her hospitalization and on day 14 of her presentation she had bilateral optic nerve sheet fenestrations. Her lumbar drain was left in post-operatively and set to 10 centimeters of water and she didn't have any CSF drainage post-operatively so neurosurgery felt that she wouldn't require any further CSF shunting procedures and on day 16 she had her lumbar drain removed and was discharged home with Acetazolamide 500 BID. She followed up with us four days post-operatively and her headaches had improved for a few days following her procedure but never quite resolved and then she was complaining more of a after the procedure by the time that she had come to follow up with us. She continued to have photophobia and phonophobia but some improvement in her vision subjectively more in the right eye than the left eye. Her physical examination on the day of her follow-up was again a high-pretensive patient with a blood pressure of 147 over 78 and a heart rate of 89. Her visual acuity was still 2025 in the right eye and it improved to 2070 in the left eye which was significantly better. She was 2125 when she initially presented. Her APD persisted with a 1.5 log in the left eye and she had bilateral improvement in her papillodema which was a stage four. This was her left eye after fenestration which showed a mild degree of improvement compared to previous but she wasn't all that severe initially. This was her left eye so if you remember I can go back. She had this severely constricted visual field initially prior to her fenestration and this was her visual field after fenestration in the left eye. So a significant improvement in her visual field following just four days immediately after a lumbar drain placement and bilateral optic nerve sheath fenestration. So this patient actually meets criteria for something called fulminate idiopathic intracranial hypertension or malignant IIH and a paper was published in Neurology in 2007 that helped to establish some diagnostic criteria for this and the patients have to meet the modified dandy criteria for IIH but also have acute onset signs and symptoms that increase intracranial pressure and less than four weeks of onset between their initial symptoms and their severe visual loss. She also had rapid worsening of visual loss over the course of a few days so really she met the last two criteria really in the first week of her presentation. This also from the same paper I just wanted to talk a little bit about the data that they had that helped them to establish this criteria and it was a retrospective review of idiopathic intracranial hypertension patients at Emory and Vanderbilt Universities and they looked at all comers over ten years at Emory and three years at Vanderbilt and really only were able to classify fourteen patients at Emory and two patients at Vanderbilt as fulminate IIH and that's between two and three percent of all patients that they investigated so fairly rare entity and then looked at several characteristics of the patients including their demographics, comorbid factors, presenting symptoms, timing between symptom onset and visual loss, timing between their symptoms and surgical treatment, and then time between ophthalmologic evaluation and surgical treatment, their CSF opening pressure, their treatment and their visual out. And what they found was that on average the interval time between their first symptom onset and their worst vision loss was between seven and 28 days. On average with traditional IIH you could expect anywhere from two to four months to be associated with vision loss but this is much quicker and our patient really fit into the lower end of that category. And all patients ended up having surgical treatment in this study which was either optic nerve sheet fenestration or some sort of CSF shunting procedure. And in the end they found out that the visual outcomes were directly related to the timeliness of their treatment. This is a slide full directly from the paper and the right hand column is visual fields before treatment, before surgical treatment and the left hand column is visual fields after surgical treatment. And the top two rows actually demonstrate patients that didn't have a significant improvement following surgical treatment but the bottom two represent more of something that our patient looked like in that they had a significant recovery of their visual field following surgical treatment. In all cases out of the 16 cases that they investigated, 14 did have some improvement in their visual symptoms but eight patients remained legally blind and so half of them still had vision at least as bad or worse as 2200 in their best eye or they had a less than 25 20% restricted visual field in their best eye. And the visual fields remained abnormal in all cases so nobody recovered 100%. The not legally blind patients had a surgery within a few hours to four days of ophthalmologic evaluation and on average that was two days. And the legally blind patients had surgery within three to 37 days which is a large spread but worked out to be on average six and a half days after ophthalmologic evaluation. So even though that's just a difference of a few days it seemed to make a big difference in terms of their visual output. In conclusion I just wanted to demonstrate a case of something that's really rare for us which is subliminal or malignant adiopathic intranial hypertension and to point out that speedy management of these patients and quick surgical treatment for these patients can make a big difference in terms of their visual outcome. And I think the paper from neurology that helped to demonstrate that and I think our patients are a good example of that. I appreciate your attention and I'm happy to take any questions. Thank you.