Table 1.
Clinical characteristics of subjects
| Subject | WeeFIM score a |
Summary diagnosisb |
Visual acuity (OD, OS)c |
Shunt | Seizure | Optic nerve appearance d |
Nystagmus | |
|---|---|---|---|---|---|---|---|---|
| Latent | Manifest | |||||||
| Group 1: patients with definite CVI | ||||||||
| 1 | 29 | 1,1 | ND | Yes | No | Normal | No | Yes |
| 2 | 37 | 1,1 | ND | No | No | Normal | No | No |
| 3 | 53 | 1,1 | 20/252, 20/320 | Yes | No | ONA, C/D >0.5 | Yes | No |
| 4 | 23 | 1,1 | ND | Yes | No | ONA | Yes | No |
| 5 | 48 | 1,1 | ND | No | No | Normal | No | No |
| 6 | 99 | 1,1 | 20/200, 20/1280 |
No | No | ONA | Yes | Yes |
| 7 | 58 | 1,1 | ND | No | No | ONA, C/D >0.5 | Yes | Yes |
| 8 | 44 | 1,1 | 20/252, 20/400 | No | No | ONA | Yes | Yes |
| 9 | 20 | 1,1 | ND | No | Yes | ONA | No | No |
| 10 | 25 | 1,1 | LP | Yes | Yes | Normal | Yes | Yes |
| 11 | 64 | 1,1 | 20/640, 20/252 | No | Yes | ONA, C/D >0.5 | Yes | Yes |
| 12 | 88 | 1,1 | 20/400, 20/200 | No | No | Normal | Yes | No |
| 13 | 50 | 1,1 | ND | No | No | Normal | Yes | Yes |
| 14 | 103 | 1,1 | 20/200, 20/252 | No | No | Normal | Yes | Yes |
| 15 | 101 | 1,1 | 20/252, 20/252 | Yes | No | C/D >0.5 | Yes | Yes |
| 16 | 27 | 1,1 | ND | No | No | Normal | Yes | Yes |
| 17 | 29 | 1,1 | ND | No | No | ONA | Yes | Yes |
| 18 | 27 | 1,1 | ND | No | Yes | ONA, C/D >0.5 | No | No |
| 19 | 18 | 1,1 | LV | No | No | ONA, C/D >0.5 | Yes | Yes |
| 20 | 47 | 1,1 | 20/500, 20/640 | No | No | Normal | Yes | No |
| 21 | 50 | 1,1 | ND | Yes | No | ONA, C/D >0.5 | Yes | Yes |
| 22 | 26 | 1,1 | ND | No | No | ONA, C/D >0.5 | Yes | Yes |
| 23 | 90 | 1,1 | ND | No | No | C/D >0.5 | No | No |
| 24 | 18 | 1,1 | ND | No | No | ONA | Yes | No |
| 25 | 93 | 1,1 | ND | No | No | Normal | Yes | Yes |
| Group 2: patients with probable CVI vs combined anterior and posterior pathway disease | ||||||||
| 26 | 100 | 3,1 | 20/800, 20/400 | No | No | Normal | Yes | Yes |
| 27 | 32 | 2,2 | ND | No | No | Normal | Yes | No |
| 28 | 24 | 2,2 | ND | No | Yes | Normal | Yes | Yes |
| 29 | 55 | 2,2 | ND | No | No | Normal | No | Yes |
| 30 | 51 | 1,3 | LV | No | Yes | Normal | No | No |
| 31 | 38 | 2,2 | LV | No | Yes | ONA, C/D >0.5 | Yes | Yes |
| 32 | 91 | 2,2 | 20/200, 20/800 | No | No | ONA, C/D >0.5 | Yes | Yes |
| 33 | 19 | 2,2 | LV | No | No | ONA | No | No |
| 34 | 34 | 1,2 | ND | Yes | No | Normal | No | No |
| 35 | 64 | 1,2 | ND | No | No | C/D >0.5 | No | Yes |
| 36 | 103 | 2,2 | ND | Yes | Yes | ONA | No | No |
| 37 | 71 | 4B,2 | LP, 20/252 | No | No | ONA | Yes | Yes |
| Group 3: patients with indeterminate cause of visual loss | ||||||||
| 38 | 123 | 1/5B | 20/200, LP | No | No | Normal | Yes | Yes |
| 39 | 111 | 1,1 | ND | No | No | Normal | No | Yes |
C/D, cup/disc ratio; CVI, cortical visual impairment; LP, light perception; LV, low vision Teller card; ND, neuro-developmental delay; OD: right eye; ONA, optic nerve atrophy; OS: left eye; WeeFIM, pediatric functional independence measure.
WeeFIM scores (out of maximum of 126): >95, normal; 77-95, below normal; <77, severe disability.
Summary diagnosis (retinal appearance for right eye, left eye):1, normal-appearing fundus; 2, straightening of temporal vessels; 3, macular ectopia, 4B, incomplete retinal detachment involving the macula 5B, total retinal detachment.
C/D >0.5 refers to nonglaucomatous cupping of optic nerve.
Neuro-developmental delay prevented visual acuity assessment.