Autoimmune Retinopathy

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Identifier Autoimmune_Retinopathy
Title Autoimmune Retinopathy
Creator Andrew G. Lee, MD; Qiancheng Wang
Affiliation (AGL) Chairman, Department of Ophthalmology, The Methodist Hospital, Houston, Texas; Professor of Ophthalmology, Weill Cornell Medicine, New York City, New York; (QW) Class of 2023, Baylor College of Medicine, Houston, Texas
Subject Autoimmune Retinopathy; Photopsia; Ring Scotoma
Description Dr. Lee lectures medical students on auto immune retinopathy.
Transcript So today we're gonna be talking about auto immune retinopathy and you might be wondering why this would come to neuro-ophthalmology. And the reason it comes to neuro-ophthalmology is because the retina often looks normal in the beginning.And there's two types of this autoimmune retinopathy. One is associated with cancer which we call cancer associated retinopathy. Sometimes the malignancy is melanoma, which we call melanoma associated retinopathy. And if you don't have any cancer it's an autoimmune mediated retinopathy. Or a autoimmune retinopathy and related optic neuropathy. So the main thing is identifying the patient clinically. Usually it's bilateral and symmetric loss of vision. It can be central or peripheral. It tends to be relatively subacute and occur bilaterally and simultaneously. Initially the fundus looks normal. Over time there will be arterial narrowing, RPE change, and the nerve will turn pale but in the beginning the fundus looks normal. the key and differentiating features are the presence of the photopsia, which is the patient says they see a flashing light or sparkle or something. So that photopsia is an uncommon presentation, presenting finding in patients who have optic neuropathy. And in patients with retinopathy they might have night blindness which is nyctalopia, or they might have day blindness which is hemeralopia. And so either day or night blindness are retinal symptoms because the optic nerve really doesn't care that much whether it's day or night, but the rods and the cones care whether it's day or night. And the typical scotoma is a ring scotoma in retinal disease so it tends to be a pair of oval ring or a bull's-eye type of appearance or target type of appearance in the visual field. But it can be a central scotoma or just diffuse generalized depression. So for these patients we really have to establish the localization and that usually means we have to use electrophysiology so we typically are going to do some combination of VEP, ERG and multifocal ERG. If it's just the central vision, a central scotoma, then we're gonna do the mERG. If it's a full field effect we're gonna do ERG so for CAR and MAR they often have decreased full field ERG's and decrease VEP's. For cone specific dystrophies and cone specific autoimmune retinopathies, mER-multifocal ERG is necessary. In CAR, the patients don't know they have cancer, so you have to go look for it. The most common cancer is small cell carcinoma of the lung but it can be any, any cancer really. In MAR, the patients have melanoma and they know they have melanoma. And so we still have to search for recurrence or a metastasis, but MAR patients often know that they have melanoma. CAR patients don't know they have cancer. So we're going to do full-body scans on both of these patients to look for it. And the antibody can be detected in both cancer associated paraneoplastic retinopathies and in the non paraneoplastic autoimmune retinopathies. And we normally send those antibodies out. The most common antibodies are anti retinal antibodies that can be at various different levels in kilodaltons, and so on the Western blot we can say how much the thing weighs but often we don't know what the actual target protein is. So it'll be like 46 kilodalton's or 23 kilodaltons, but some of the antibodies we know what the target is like recoverin, or enolase, or some other protein that has been identified. That's not as important as trying to recognize CAR versus non-CAR autoimmune retinopathy. So in summary you should be thinking about autoimmune retinopathies in patients who have bilateral, symmetric, subacute central or diffuse vision loss with a normal fundus. Over time the nerve will turn pale, the arteries will get narrow, RPE change will occur, but in the beginning it looks normal. W e're gonna send the paraneoplastic antibodies, that's the send out tests. And you're going to do the electrical test to confirm the localization usually a combination of the VEP, ERG, and/or mERG and the key in differentiating symptoms: photopsia, nyctalopia, hemeralopia, which is day blindness, and the ring scotoma.
Language eng
Format video/mp4
Type Image/MovingImage
Collection Neuro-Ophthalmology Virtual Education Library: Andrew G. Lee Collection: https://novel.utah.edu/Lee/
Publisher North American Neuro-Ophthalmology Society
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-5890
Rights Management Copyright 2019. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6ht87mf
Setname ehsl_novel_lee
ID 1578872
Reference URL https://collections.lib.utah.edu/ark:/87278/s6ht87mf
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