Table 1.
Visual Acuity and Multifocal ERG Findings in Right Eyes of 4 Reexamined Family Members With Autosomal Dominant Retinitis Pigmentosa Caused by KLHL7 Mutation
| Patient/Age, ya | Best-Corrected VA | Multifocal ERG Result | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| P1 Amplitude, nV/degree2 | P1 Implicit Time, ms | ||||||||||
| Ring (1 + 2) | Ring 3 | Ring 4 | Ring 5 | Ring 6 | Ring (1 + 2) | Ring 3 | Ring 4 | Ring 5 | Ring 6 | ||
| II-1/69 | 20/200 | ND | ND | ND | ND | ND | ND | ND | ND | ND | ND |
| III-1/38 | 20/20 | 19.3 | 6.4 | 5.1 | 4.7 | 3.5 | 33.3 | 34.2 | 36.7 | 37.5 | 39.2 |
| III-2/36 | 20/30 | 10.3 | 5.3 | 4.5 | 3.5 | 1.7 | 33.3 | 40.0 | 43.3 | 43.3 | 41.7 |
| III-3/34 | 20/20 | 51.1 | 25.5 | 13.4 | 8.3 | 6.4 | 30.0 | 30.8 | 31.7 | 35.0 | 35.8 |
| Normal, mean (2 SDs)b | … | 32.0 (23.8) |
22.4 (14.5) |
18.2 (10.8) |
16.1 (9.1) |
15.7 (9.2) |
27.0 (3.0) |
26.2 (2.9) |
25.7 (2.9) |
25.9 (2.9) |
26.3 (3.0) |
Abbreviations: ellipses, not applicable; ERG, electroretinography; ND, nondetectable; VA, visual acuity.
a
At the time of the presented data.
b
Values at the Department of Clinical Sciences, Ophthalmology, Lund University, Lund, Sweden, among 30 eyes of 21 individuals (age range, 8–55 years [mean age, 38 years]).