Early Detection of Stargardt Disease with OCT and AF – Page 21 of 30

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 degenerate, they are less densely packed and occupy a larger space. But as this occurs, the entire ONL becomes less hypo-reflective and more hyper-reflective. This newly hyper-reflective zone appears as an extensive of the hyper-reflective ELM. This hypothesis appears to be supported by some rare histo-pathological specimens.⁵