 Okay, it's after 8, so I think we should probably get going. Welcome to neuro-ophthalmology grand rounds, I guess is what it's called, and I think there'll be a little influx of people coming from FEC, it's running over a little bit. I think that we're currently complaining about the EMR, so yay. And I want to welcome everybody and this sort of an interactive forum. We have three presentations today. The first is, I think is on the list was Megan, I mean Sarah, are you up for Sarah? Yeah. Yeah, Sarah Stone is going to be presenting a case of neuroratinitis that we happened upon, just a great case with a nice differential diagnosis and an actual diagnosis which of course is always a happy day in neuro-ophthalmology. Thank you Sarah. So, Sarah Stone is a neurology resident, PGY-4. Thank you. All right. Good morning everyone, again my name is Sarah Stone, I'm a PGY-4 adult neurology resident and I'm going to be talking about neuroratinitis today. So we'll start with a case. This is a case we saw in neuro-ophthalmology clinic, a 43-year-old woman presented to a neuro-ophthalo clinic for evaluation of vision loss in her left eye. She initially reported that she was on a business trip in Seattle when she awoke with blurring of her central vision. She thought it was related to her contact lenses, so she removed her contact lenses and sort of watched things for about a day but her symptoms were persistent and then ultimately she described sort of a ring in her central vision that had a grayish or purple hue with some flashing lights and a sensation of movement within that ring. She was evaluated by a local optometrist in Seattle and was told that she had optic nerve swelling and was started on treatment for this. On additional history she noted that she carried a diagnosis of obstructive sleep apnea as well as headaches with rare migraines. For surgical history she'd had three cesarean sections and a laparoscopic surgery for ovarian torsion during which her appendix was also removed. She had no significant neurologic or ophthalmologic family history. She lives in Ogden, Utah with her husband and three children. She works as a software salesperson and denies any use of tobacco alcohol or elicits. She doesn't take any regular medications and she did endorse an allergy to penicillins. On some additional questioning she recalled that about three to four months prior to her symptom onset she had been traveling to coastal California with her family on a camping trip and they had come across a stray cat which they adopted and brought home with them. About five weeks prior to presentation she remembered that she had had about a 24 hour febrile illness which sort of just self resolved and she didn't have any complications related to that. Then about two weeks prior to presentation she noted that the family dogs had developed fleas and they were working vigorously with their vet to take care of these fleas. On that clinic visit her initial exam was notable for 20-20 vision in the right eye and 20-50 vision in the left eye. She had a 0.6 log relative a-fair and pupillary defect on the left. She described a central scatoma with E.M.'s lurker testing and was only able to recall correctly one of 11 Ishihara plates on the left eye. Her slit lamp exam was notable for a normal anterior chamber without any cells and her fundoscopic exam she showed 3 plus optic disc swelling on the left and a boggy appearing immaculate with a normal fundoscopic exam on the right. Additional testing in clinic that day included Humphrey visual fields and OCT-RNFL and OCT of the macula. Her visual fields are shown here and you can see that she has central scatoma on the left sorry it's not labeled left and right but that's the left up there. And then her OCT-RNFL showed diffuse thickening on the left eye and her macular OCT showed loss of foveal contour with subretinal fluid and intra-retinal deposits. She also had an MRI brain in orbits that was contrasted and normal and her laboratory testing included toxoplasma and Lyme serologies, RPR, quantifier on gold and ACE which were negative and then she also had Bartonella Hensley serologies which were positive. The treatment that she had received in Seattle was Doxycycline 100 milligrams twice daily for an intended duration of 4 weeks and then we also prescribed her Apprentice-Ontaper for her to initiate in the event that she had subjective worsening of her vision and she did take these prior to her follow-up visit which was 2 weeks. At her 2 week follow-up exam she noted subjective improvement in her acuity in the left eye and this was confirmed objectively now with 2030 vision in the left eye and an unchanged normal vision in the right eye. Her OCT-RNFL showed her visual field showed resolution of her central scatoma in the left eye and then her OCT-RNFL and OCT of the macula were also quite improved and then on her fendoscopic exam she had some minimal optic disc swelling superiorly and a well-formed macular star. This is not her funnestota, sorry. So this was all consistent with the diagnosis of Bartonella neuroventinitis so I'm just going to spend a little bit of time talking about this entity. It was first described by Theodore Lieber in 1916 who coined the term stellate maculopathy based on the star-shaped appearance of the macula in these patients and the pathophysiology was later better characterized by Donald Gass in 1977 who used fluorescein angiography to show that initially there's actually increased vascular permeability at the optic disc and a secondary swelling that occurs at the macula. He was the person who coined the term neuroventinitis to describe this constellation of findings. Optic disc edema with a macular star is not specific to neuroventinitis although that's what we think of. It can also be seen with other ophthalmologic conditions including hypertensive retinopathy, papillodema, anterior ischemic optic neuropathy, diabetic papillopathy and juxtapapillary tumors as well as some toxic exposures and in these cases the clinician has to use details from history, physical exam and other diagnostic testing to delineate these diagnoses from neuroventinitis. Neuroventinitis mostly occurs in young adults who've had a preceding viral illness often respiratory in nature. For the most part vision changes are painless although some patients do report some retro bulbar discomfort. Typically the processes unilateral, the bilateral cases have been described and in fact there are cases of subclinical involvement of a fellow eye in these patients. They have reduced visual acuity with a central or a psychocentral scatoma. Their relative apherin pupillary defect is variable and may depend a little bit on the timing of the patient's presentation. Optic dyskidema occurs early in the disease course with the evolution of a macular star sort of throughout the disease course occurring a little bit later and then visual prognosis is quite favorable on these patients even without treatment. While infection typically comes to mind as a common cause of neuroventinitis other ideologies have been discovered. So the infectious causes include bacterial, viral, protozoan and fungal microorganisms. However, neuroventinitis has also been described in association with a variety of inflammatory conditions including sarcoidosis, inflammatory bowel disease, polyarteritis, nodosa and then some other autoimmune syndromes that also involve the eye like urban, idiopathic retinal vasculitis and neuroventinitis and tenu syndrome or tubular interstitial nephritis and uveitis. In cases where no cause can be identified, neuroventinitis is termed idiopathic and in the- What percent of all cases are considered idiopathic? I'm not sure. We're talking 10% half, 60% percent. I'd say about half, idiopathic. Anybody from retina? We all know what idiopathic means, don't we? No, no, no. We're idiots. Show them. I mean, obviously, no. Okay. So the recurrent idiopathic neuroventinitis has gained some interest in the literature. There was a series of seven patients reported by Perman et al. who looked at these cases and they proposed a possible kind of autoimmune disc vasculitis as the etiology and recommended consideration for chronic immunosuppression in those cases. So as you alluded to, not all idiopathic cases are idiopathic. So the most common identifiable cause of neuroventinitis is disseminated cat scratch disease. You may recall from medical school that cat scratch disease starts with a bite or a scratch from a cat. That leads to kind of a cellulitis in regional lymph adenopathy, occasionally lymph adenitis. The disease can become disseminated in which case patients will experience fevers, chills, myelogias, arthralogias, and then they can even develop things like endocarditis, pneumonia, osteomyelitis. Ocular Bartonella is not limited just to neuroventinitis. Other ophthalmologic conditions have been seen with oculobartonella and these include uveitis, papillitis, coroiditis, endophthalmitis, optic disc granulomas, branch retinal arterial and venous occlusions, angiomatosis, and serous retinal detachments. As some of you may know, treatment for Bartonella neuroventinitis is somewhat controversial. Some studies have shown a potential benefit and some studies have shown no benefit and all of these studies are essentially retrospective. There's been no, to my knowledge, randomized clinical trials to study this. In 1998, Reed et al retrospectively found that antibiotic therapy potentially shortened the duration for visual recovery. And then in 2004, Rowlain et al recommended dual coverage for serious complications of Bartonella including neuroventinitis. And then in 2012, Chee et al retrospectively found no improvement with antibiotics, steroids or the combination of both. More recently, in a 2014 point counterpoint article in the Journal of Neural Ophthalmology, Michael Lee proposed treatment for patients with severe vision loss, bilateral involvement, systemic symptoms, immunocompromised state or protracted clinical course, while the opposing author, Tariq Bhatti, argued for no treatment as long as the patient is immunocompetent. Importantly, Bartonella can cause serious neurologic sacroiliac, which were particularly interesting to me. There is a review that went through all of the cases in which neurologic complications of a disseminated cat scratch disease have been reported. And these cases included patients with meningitis, encephalitis, seizures, demyelination in the form of acute disseminated encephalomyelitis or transverse myelitis, encephalitis lethargica, which is essentially a post-infectious Parkinsonism, and then also Guillain-Barre syndrome. In summary, neuroventinitis ideologies include infectious, inflammatory, and in some cases idiopathic. And the most commonly identified cause for neuroventinitis is Bartonella. The treatment for Bartonella neuroventinitis is controversial and clinicians should be aware of kind of red flag symptoms that make an alternative diagnosis more likely. And then ocular Bartonella is not the only complication of disseminated cat scratch disease. There are other serious neurologic and medical complications that can occur. So now we've resolved that, Judith. What do you recommend? Stigens-Gallup, ever since this article came out and very recently suggested there was no treatment that really makes a difference in this. Right. Well, in this particular instance she had already been started on treatment and was fairly adamant that she was going to take prednisone, whether I gave her the prescription or otherwise. And I think that at least she had been doing quite a bit of review of the literature and was fairly sure of the treatment course that she wanted to undergo. That's not always the situation and there may be circumstances under which one might have a little more influence over what's going to be happening with the patient. But in this instance she certainly had made a careful review of the literature and made her own decisions. What would you recommend? In the first case you make the diagnosis. Do you generally recommend both antibiotic treatment as well as oral steroids? So I like the antibiotic treatment. I'm a lot more leery of the steroid treatment. And then the other one is, and I'm not a pharmacologist, my understanding is that we've called it infectious, but no one has shown that the neuro-recognizance itself is actually an infection. I mean nobody has ever pulled out an infection. I mean it would be very hard to do, but no one has shown that this is not necessarily an immunological reaction to infection. We really don't know if it's a true infectious etiology. Correct. Correct. That is still the case. In fact I think the majority of people feel the neuro-recognizance is probably not actual infection. That it's the most likely immune reaction. Correct. That's my understanding. Yes. How often was bilateral? Did you say bilateral? I think bilateral is pretty rare. I've never seen it. Which is surprising. But if it is an immune reaction, it's very interesting. Very confusing. It'll be interesting to see what happens. She did have a slightly, just clinically it didn't look abnormal, but on OCT her right optic nerve was a little on the full side. It'll be interesting to see what happens to that over time. I think that Sarah's point that there may be subclinical involvement in the other eye is very apt. And of course we're used to dealing with that sort of thing with optic neuritis as well. We certainly see parainfectious optic neuritis, especially in children it is more likely to be bilateral than under normal circumstances, but certainly not always. Your references are very thorough, but you did not include the reference to the well-known neuro-ophthalmologist in Rockhead Banger or Ted Nugent who assumes a Bobcat scratch fever. We will definitely, next week, put a video in. Okay. Better. Yeah. Yes. Is Dr. Cycling the antibiotic choice for Bartonello? It is, yes. Did the initial ophthalmologist know it was Bartonello? Yes. Yeah, it was an optometrist and an ophthalmologist and an emergency room and a neuro-ophthalmologist. So you didn't go on all those details. Yes. But yeah, she had a pretty bang-up evaluation in Seattle. Nice. When she came for follow-up, they were just managing to get the flea situation under control. We're very lucky here in Utah that fleas do not prosper. So that's nice. Any other questions? No? If not, I think we will move on. Thank you very much.