Nerve fibre layer loss in diseases of the outer retinal layer.

We present examples of nerve fibre layer changes in diseases thought to affect primarily the outer retinal layers. These disease processes include cone-rod dystrophies, rod-cone dystrophies, juvenile macular degeneration (Stargardt's disease) and fundus flavimaculatis, vitelliform macular dystrophy, and Leber's congenital amaurosis. All were associated with alterations in the retinal nerve fibre layer, either diffuse or focal. The presence of these nerve fibre layer changes raises the question of transsynaptic degeneration and of possible primary or associated disease of other retinal cells than the receptors-namely, bipolar, amacrine, Mueller, or ganglion cells--in these disease of the outer retinal layer. Involvement of the nerve fibre layer also indicates the need to examine patients with defects in the nerve fibre layer by electroretinograms and other tests for outer retinal layer disease when no obvious optic nerve disease is present.