Retinal Physician

JAN-FEB 2017

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R E T I N A L P H Y S I C I A N | J A N U A R Y / F E B R U A R Y 2 0 1 7 29 components. 23 Immunohistochemistry can identify antiretinal antibodies, most common of which are antirecoverin and anti- alpha-enolase antibodies. 20 Myotonic Dystrophy e classic ocular finding in myotonic dystrophy is the "Christmas tree cataract," a posterior subcapsular, red and green, iridescent opacification of the lens. Patients can pres- ent with other ophthalmic findings including ocular hypotony and pigmentary retinopathy (Figure 7) that can be confused for AMD. 24,25 e pathognomonic systemic feature is muscle weakness and myotonia of the distal legs, hands, neck and face. 26 Fundus examination can reveal butterfly-shaped macu- lar pigmentary changes, peripheral reticular pigmentation and peripheral polygonal-shaped atrophy. 27 FAF of the macular pigmentary changes demonstrates a branching linear pattern of mixed hyper- and hypoautofluorescence. 28 Macular OCT of these changes identifies patches of inderdigitation zone hyper- reflectivity and ellipsoid zone hyporeflectivity. 28 CONCLUSION Despite the prevalence of dry AMD, it is important for ophthalmologists to consider less common diseases and multi-modal imaging to arrive at the correct diagnosis. As demonstrated in this series, numerous pathologies have over- lapping features with dry AMD, including pattern dystrophies, cone-rod dystrophies, medication toxicities, and systemic disor- ders associated with maculopathies. Other common disorders with overlapping features of dry AMD include inactive central serous retinopathy and other "pachychoroid"-related maculop- athies, particularly in older patients, as these entities can cause macular pigmentary changes. Historical and exam features that raise suspicion of "Dry AMD-masquerade syndromes" include younger age, suspect medications and systemic disorders, family history, highly symmetric macular pathology and lack of typi- cal drusen. Cuticular drusen and flecks, as demonstrated above, as well as dominant drusen (Malattia Leventinese), and even crystalline retinopathies such as tamoxifen maculopathy, could mimic drusen. Ancillary testing with red-free photography, FA, FAF, macular OCT and ERG can be helpful in further charac- terizing the retinal pathology. Newer techniques such as OCT with enhanced depth imaging can be used to demonstrate thickened choroid, which is characteristic of central serous reti- nopathy and other "pachychoroid"-related maculopathies. In the future, genetic testing will become commonplace, and will further delineate many of these maculopathies. RP REFERENCES 1. Wong WL, Su X, Li X, et al. Global prevalence of age-related macular degeneration and disease burden projection for 2020 and 2040: a systematic review and meta- analysis. The Lancet Global health. 2014;2(2):e106-116. 2. Spaide RF, Curcio CA. Drusen characterization with multimodal imaging. Retina (Philadelphia, Pa). 2010;30(9):1441-1454. 3. Boon CJ, van de Ven JP, Hoyng CB, den Hollander AI, Klevering BJ. Cuticular drusen: stars in the sky. Progress in retinal and eye research. 2013;37:90-113. 4. 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