Summary of electrophysiological findings at presentation
| Case | Age (years) | Pattern ERG | Scotopic ERGs | Photopic ERGs | ||||
|---|---|---|---|---|---|---|---|---|
| Rod specific | Maximal | 30 Hz flicker | Single flash | ON b-wave | OFF d-wave | |||
| Right eye findings appear above left eye findings. N, normal; A, abnormal; A-ve, electronegative waveform; A (low b:a), waveform with a low b-wave to a-wave amplitude ratio. ON-OFF ERGs in patients 4, 10, and 17 (*) were first performed respectively 3.5 years, 1 year, and 4.5 years after presentation and after full field ERGs had deteriorated. | ||||||||
| 1 | 55 | A | A | A -ve | A | N | A-ve | N |
| A | A | A –ve | A | A | A-ve | N | ||
| 2 | 56 | A | A | A | A | N | A | N |
| N | A | A | N | N | N | N | ||
| 3 | 38 | A | A | N | A | N | – | – |
| A | A | N | A | N | – | – | ||
| 4 | 55 | A | A | A -ve | A | A | A-ve* | A (mild)* |
| A | A | A –ve | A | A | A-ve* | A (mild)* | ||
| 5 | 62 | A | A | A -ve | A | A | A-ve | N |
| A | A | A -ve | A | A | A-ve | N | ||
| 6 | 65 | A | N | N | A | A | A | N |
| A | N | N | A | A | A | N | ||
| 7 | 60 | A | A | A-ve (low a) | A | A | A-ve | A (mild) |
| A | A | A-ve (low a) | A | A | A-ve | A (mild) | ||
| 8 | 46 | A | A | A -ve | A | A | A-ve | N |
| A | N | N | N | N | – | – | ||
| 9 | 49 | A | N | N | A | A | – | – |
| A | N | N | A | A | – | – | ||
| 10 | 45 | A | A | A -ve | A | N | A-ve* | N* |
| A | A | A –ve | A | N | – | – | ||
| 11 | 30 | A | A | A-ve | A | A | A | N |
| A | A | A | A | N | N | N | ||
| 12 | 26 | N | N | N | N | N | – | – |
| A | N | N | A | N | – | – | ||
| 13 | 51 | A | A | A-ve | A | A | A | N |
| A | A | A-ve | A | A | A | N | ||
| 14 | 52 | A | A | A (low b:a) | A | A | A -ve | N |
| A | N | N | A | N | – | – | ||
| 15 | 64 | A | N | A-ve | A | A | A | A |
| A | A | A | A | A | – | – | ||
| 16 | 49 | A | N | A | A | N | – | – |
| N | N | A | A | N | – | – | ||
| 17 | 44 | N | N | N | A | A | A (low b;a)* | N* |
| A | N | A | A | A | A (low b:a)* | N* | ||
| 18 | 62 | A | N | N | A | N | N | N |
| A | N | N | A | N | N | N | ||