Progressive Cone Dystrophies

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Abstract
Patients with progressive generalized cone dystrophy often presented nystagmus (or strabismus) and complained of photophobia, decrease in visual acuity or disturbances in color perception. The most classic fundus abnormality was the bull''s eye maculopathy or a pallor of the optic disk. Minimal macular changes were sometimes seen, which may progress to a bull''s eye type of macular degeneration. The photopic ERG [electroretinogram] was always affected, whereas at first the scotopic ERG seemed normal. Progressive deterioration of the visual functions was accompanied by increasing fundus lesions and rod involvement, as suggested by the modifications of the dark adaptation curve and the scotopic ERG. The progression of typical generalized cone dysfunction was very slow. In some cases of so-called Stargardt''s disease with peripheral participation, a very rapid progression was observed. In such cases a normal ERG did not necessarily mean that the disease would remain localized to the macular area. No definite prognosis can be made on 1 single ERG. In 3 cases with sector pigmentary retinopathy the photopic ERG was more affected than the scotopic ERG. These cases are probably primary cone-rod dystrophies. Although there is no electrophysiological control, the clinical impression is that the evolution, if possible, is very slow.

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