Dulz et al reported the Hamburg CLN3 Ophthalmic Rating Scale for evaluating ocular involvement in juvenile CLN3 disease. The authors are from University Medical Center Hamburg-Eppendorf.
Parameters:
(1) visual acuity
(2) optic pallor
(3) macular striae
(4) macular orange pigment
(5) peripheral bony spicules
(6) vessel rarefication
(7) ellipsoid zone
Parameter
Finding
Points
visual acuity
>= 20/25
7
>= 20/63
6
>= 20/160
5
>= 20/400
4
>= 20/1000
3
>= counting fingers
2
>= hand motion
1
light perception
0
optic pallor
none
1
severe (? present)
0
macular striae
none
1
present
0
macular orange pigment
none
1
present
0
peripheral bony spicules
none
1
present
0
vessel rarefication
none
1
present
0
ellipsoid zone
intact
2
disruption < 2,000 µm in diameter
1
complete loss
0
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 0
• maximum score: 14
• The lower the score the worse the condition.
Total Score
Grade
14
0 (normal)
10 to 13
1 (affected)
5 to 9
2 (severe)
0 to 4
3 (end stage)
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