Table 1 of Kjellström, Mol Vis 2014; 20:89-104.

Table 1. Patient demographics

Subject Age (years) Sex Age at onset (years) VA OD VA OS ff-ERG cone ff-ERG rod mERG Visual fields
1 Ia 69 F early childhood CF CF ↓↓ ↓↓ ↓↓ Minimal temporal crescent-shaped remnants
1 Ib 61 M NVP 1.0 1.0 N N - Normal
1 Ic 60 F NVP 0.8* 0.4** N N - Normal
1 IIa 23 F 9 0.04 0.05 ↓↓ N ↓↓ Large nasal paracentral/central scotomas
2 Ia 42 F NVP 0.7 1.0 N N - Normal
2 Ib 48 M 40 0.5 0.3 N N ↓ Small nasal central scotomas for 04:e
2 IIa 25 F 7 0.03 0.04 ↓↓ ↓↓ ↓↓ Large central/para-central scotomas and peripheral constriction
2 IIb 23 F 20 0.16 0.13 N N ↓ Small central scotomas for 03:e OD and 04:e OS within the central 5°
2 IIc 12 F NVP 1.0 1,0 N N ↓ Normal
2 IId 9 M NVP 1.0 1.0 N N - Normal
3 Ia 41 F 15 0.2 0.1 N N ↓ Small central scotomas within 5–10°, concentric reduction of II4:e
3 Ib 43 M NVP - - - - - -
3 IIa 12 M 6–7 0.09 0.16 ↓ N ↓ Central scotomas within 20–30°

Abbreviations: VA visual acuity, OD right eye, OS left eye, F female, M male, CF count fingers, NVP no visual problems, *clinical presentation at last visit reduced vision due to capsular opacities after cataract surgery, **reduced vision due to cataract Ff-ERG recordings: ↓reduced, ↓↓severely reduced, N normal

Kjellström, Mol Vis 2014; 20:89-104. http://www.molvis.org/molvis/v20/89

©2014 Molecular Vision http://www.molvis.org/molvis/

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