Walsh & Hoyt: Paraneoplastic Retinopathies

Visual loss from retinal degeneration as a remote effect of systemic cancer was first described in 1976 by Sawyer et al. who reported three patients who initially complained of episodic blurring and dimming of vision, photopsias, and nyctalopia, and who subsequently experienced slowly progressive, bilateral visual loss initially characterized by constriction of the visual fields, paracentral scotomas, and eventual loss of central vision. The appearance of the fundi in these patients was initially unremarkable; however, with time, the retinal arteries became markedly narrowed. In one of the patients, an electroretinogram (ERG) was flat. All three patients eventually died from small-cell lung cancer. At autopsy, the eyes of all three patients showed total degeneration of the retinal photoreceptors with preservation of the inner layers of the retina. Sawyer et al. postulated that the degeneration of photoreceptors that occurred in these patients was cause by the distant effects of their neoplasms and emphasized that in two of the three patients, the neoplasm was not known to be present at the time visual symptoms began.