Figure 1.

Optical coherence tomography, monochromatic funduscopy images and stereophotographs in left eye from a patient with Spastic Ataxia of Charlevoix-Saguenay (ARSACS), in left eye with hypermyelinated retinal fibres, and in left eye from a healthy subject. The optical coherence tomography of an eye from an ARSACS patient shows a regular increase of retinal nerve fibre layer thickness in all quadrants (yellow arrows). Notice the values of retinal nerve fibre layer thickness (below), uniformly above the green (95% normal range) zone around the nerve optic and the high average thickness (193 microns). Colour funduscopy image of the same patient shows the characteristic telltale yellow discoloration of this disease while the monochromatic photograph (red free procedure) illustrates an increased visibility of the retinal nerve fibre layer. The optical coherence tomography of an eye with Persistent Myelinated Retinal Nerve Fibres shows a localised thickening of the retinal nerve-fibre layer in the temporal superior area (red arrow) while the rest of the layer remains between the normal limits (green area below). The colour and monochromatic funduscopy images of the abovementioned subject with Persistent Myelinated Retinal Nerve Fibres showed that the myelinated nerve fibres are localised in the superior temporal area showing a white-yellowish tone, whereas the rest of the area surrounding the optic disc is normal. The eye of a healthy subject shows no alterations, localised or diffuse thickening, in all tests: optical coherence tomography, colour funduscopy image and monochromatic photograph.