Table 1.
Clinical features of patients in the American family.
| year | 1980 | 2003 | |||||
|---|---|---|---|---|---|---|---|
|
| |||||||
| dark-adapted thresholds | acuity | visual field | |||||
| subject | age | Central Ft.L |
Peripheral Ft.L |
Central Ft.L |
OD | OS | OU |
| II-1 | 39/62 | 4×10−3 | 4×10−3 | 3×10−3 | 20/40 | 20/50 | full |
| II-3 | 33/56 | 7×10−3 | 7×10−2 | difficult | 20/50 | 20/50 | constricted |
| III-2 | 18/37 | 7×10−3 | 8×10−2 | difficult | 20/20 | 20/20 | constricted |
| IV-1 | 13 | - | - | 4×10−4 | 20/20 | 20/20 | ring scotoma |
| IV-2 | 10 | - | - | 3×10−4 | 20/20 | 20/20 | ring scotoma |
| normal | - | 2-8×10−6 | 1-5×10−6 | 2-8×10−6 | 20/20 | 20/20 | full |
Note: Dark-adapted thresholds (Foot Lamberts =Ft.L) were determined with a Goldmann-Weekers adaptometer (see Methods). All patients had normal color vision. The proband II-1 and her sister II-3 presented macular scotomas. The probands grandchildren IV-1 and IV-2 presented ring scotomas.