ReferencesK. Stargardt (1909) Uber familiare, progressive Degeneration in der Makulagegend des Auges. Graefe’s Arch Clin Exp Ophthalmol. 7t 534-550N. Lois, A. Halfyard, AC Bird, G. Holder, FW Fitzke 2004 Fundus Autofluorescence in Stargardt Macular Dystrophy - Fundus Flavimaculatus. American Journal of Ophthalmology. 138(1): 55-63S. Özdek, Z. Onaran, G. Gürelik, K. Bilgihan, C. Acar, B. Hasanreisoglu.
Special AcknowledgmentDan Epshtein AcknowledgmentsArnold ShermanIrit ZakaimAyah AhamedMichelle YudinaThomas BurkeSanjeev Nath
Jerome Sherman, OD, FAAO Jerome Sherman, O.D., is perhaps optometry's most prolific writer, publishing over 650 clinical articles, research manuscripts, book chapters and two CDs. He is senior author of three books that were published in 2007, and has delivered over three thousand lectures both nationally and internationally. He has served as a
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Comments and Conclusions• The initial clinical finding of early Stargardts Disease (SD) may be the abnormal ELM as exhibited in both eyes of each daughter.5• The hyper-reflective ELM with OCT may represent degeneration of the cone and rod nuclei within the outer nuclear layer as hypothesized on pages 21-23.• Oral treatment to reduce the accumulation
Case 5: Topcon 3D OCT OD Dad also has a normal fundus and a normal OCT. SD typically has an autosomal recessive inheritance pattern. Hence, mom and dad most likely each have one abnormal gene and unfortunately, all 3 kids inherited the abnormal recessive gene from both parents.
Case 4: Topcon 3D OCT OD Mom has normal VA, a normal fundus and a normal OCT.
Topcon 3D OCT OS Early Dx of SD may help development of early interventions and benefit patients the most.Genetic confirmation, registration of patient into central registry, and publication of natural history data may help locate patients for trials and improve patient counseling.Oral treatment such as ALK-001 from Alkeus Pharmaceuticals may prevent formation of
Topcon 3D OCT OS The abnormal ELM (for the reasons explained on the previous page) is probably the earliest clinical indication of Stargardts Disease. We have not seen a similar finding in RP or other retinal degenerations to date. This apparent thickened, irregular and hyper-reflective ELM may be a bio-marker for very early SD.
Topcon 3D OCT OD In the two sisters, the apparent thickening of the ELM is likely due to cellular debris in close proximity to the ELM. The outer nuclear layer (ONL-above the ELM) is normally composed of dense and closely packed nuclei of the cones and rods and is hence hypo-reflective. As the nuclei