Stargardt's disease/fundus flavimaculatus: psychophysical and electrophysiologic results

Retinal functions were analyzed psychophysically and electrophysiologically in 73 patients (146 eyes) with Stargardt's disease/fundus flavimaculatus. Patients were classified into types 1, 2, 3, and 4; patients with type 3 were subdivided into 3E and 3L (early and late onset of initial symptoms). Most had visual acuity (VA) of 20/200 or greater at initial testing. VA declined 0.25 octave/year during follow-up (mean, 6.1 years). Four of 16 patients (25%) older than 40 years had VA of 20/200 or less in the stronger eye. Psychophysical tests (flicker profile, central scotoma, dark adaptation) showed variable degrees of abnormalities. Electrophysiological tests showed significant photopic b-wave amplitude decreases, particularly in type 3E (42.2% of normal). The electro-oculogram light peak/dark trough ratio was abnormal in 60 of 132 eyes (45.5%), especially in type 3E (25 of 34 eyes, 73.5%). Visual prognosis and overall visual function varied depending upon disease type, location of retinal lesions, and age of onset. In Type 3E, overall retinal function was poorest and accompanied by the most severe decline of central vision and function in the surrounding macula.