 Okay, so our second presenter today is Adam to have none and he's one of our Rotating neurology residents, and he's going to present a neuro ophthalmology case presentation today's case will be about a 34 year old female Who was seen obviously in neuro ophthalmology clinic? our History was pretty unremarkable She did present to an outside ophthalmologist basically with blurry vision in the right eye Which she had had a sty on that eyelid for several weeks, which she was rubbing in the ed. She was evaluated and There was some concern That she had had papillodema By the outside ophthalmologist. We didn't really concur with that We did a B scan looking for Druze and that was negative when she came to neuro ophthalmology her vision was quite intact She didn't have anything suggesting optic nerve dysfunction But on dilated exam there was Indistinct margin of the right inferior disc And we did do autofluorescence, which was negative So this is sort of a classic example Unfortunately, this patient came late in the day and we weren't able to photograph her but her Exam looked almost identical to this. It was slightly more inferior But I think some of you probably know what we're dealing with here, but this was a myelinated retinal nerve fiber and It has been described in literature for quite some time since the 19th century but Typically, you do see them unilaterally a very small percentage will be in both eyes these are found most often in children during routine exams and it doesn't really appear that there's a disposition to women or men and The studies on autopsy show that it's probably present in about 1% In a large series in Japan that looked at people just presenting to an ophthalmology clinic and It was less prevalent The presentation when it is symptomatic is Typically Visual field deficits it does spare the macula there are reported cases of involvement of the macula which Would produce more dramatic field deficits, but Overall the prognosis is quite good in children You will see axiomyopia and amblyopia and this can be resistant to therapy In cases where it is very very extensive. It could be Consistent with lucacoria which in that age population you would think of retinoblastoma and if Intervention is needed. It's typically because of Neovascularization or vitreous hemorrhage that appears to be quite rare though Characteristics typically it's not progressive. There are cases of progressive Myelinated retinal nerve fibers and one was described here at the Moran of an adolescent with a seven-year interim between appointments had Quite a dramatic progression Some of this may have to do with disruption of the lamina crebrosa, which I'll go into in a second There has been regression documented this usually follows an insult Such as optic neuritis or glaucoma here to be the most common It is associated with some conditions such as neurofibromatosis Gorlin syndrome and There are reports of familial cases that do not have a genetic basis but it is a hallmark of the autosomal recessive spastic ataxi of Charlevo and Saginae and and When you see it the key to the diagnosis is the feathery or frayed edge and I think you'll notice that on a few examples that I'll show in a second They of course are quite white the myelin Producing that and they they track along the nerve fibers, which makes sense But they're not always contiguous and they're not always contiguous with the disc itself So you can see them separated by you know, it's a significant amount from the disc When they are around the disc, it's very easy to mistake them for nerve edema Also, you know cotton wool spots Other things that you guys see that I don't like retinal infiltrate and epiretinal membranes They can obscure vessels either partially or entirely and there can be neovascularization that's associated with them so just a quick note on optic nerve myelination it Proceeds from posterior to anterior and it begins probably about five months Some authors have said eight months But by the time it reaches the lamina crib rosa it should stop there And by full term that process should be complete one model that Illustrates how important the lamina crib rosa is is Rabbits who do not have a fully developed lamina and it's a normal variant for them to have myelination past the lamina So the etiology is subject to debate, but people of course do think that the lamina is probably involved One theory is that the oligodendrocytes arrive embryologically before the lamina is fully formed Another is that the astrocytes who secrete what is thought to be a inhibitory protein may be dysfunctional in these cases other cases of progressive Myelinated retinal neurofibers show disrupted lamina crib rosa from tumors trauma surgery So while the etiology is not clear certainly it has something to do with that on Studies of em you see axons of varying Size as well as Varying degrees of myelination and we'll look at a couple pictures of that. I just wanted to show some retro bulb our optic nerve axons Very nicely myelinated And pretty uniformly myelinated This is a nice picture showing the transition zone from myelinated neurofiber to Unmyelinated and you can see the lamina there quite nicely Here's a study of Neurofiber That is myelinated and you can see these fiber bundles coming through and actually there's an area here that is not Myelinated and then the myelination begins subsequently These are these are from humans here's a abnormal myelination and One thing to point out is that you can see that you know There are degrees of myelination that vary quite dramatically here in these nerve fibers The axons are very disparate in their size and then they're nerve fibers that aren't myelinated So there's a mix and generally I'd characterize it as disorganized So I'd like to thank Julie Shelton who was the fellow last year who did some work is actually Going to be published Alternative imaging methods Here we see a classic case. This is very nice feathery frayed edge Coming off the disc here on OCT We see a thickened nerve fiber layer And presumably that's the myelin or the increased size in the axons probably the myelin This very dark The image here was auto fluorescence Which didn't show anything suggestive of drusen and then we have red free and Infrared which actually demonstrate the myelinated fibers quite well Here's a second case and what I liked about this was it's not contiguous with the nerve obviously We again see on the OCT that there's thickened nerve fiber layer on Autofluorescence we this is actually auto fluorescence here. We see blocking So it's a negative auto fluorescence and then red free and infrared. We see that again. It's highlighted quite well so this Would be the first published case of OCT being used In this manner and it you know appears to correlate quite well So hopefully that will be something that will be Used in the future