Table 1.

Summary of disease stages

Disease stage	CLN2 clinical rating scale score (motor score + language score)	Additional characteristics
6	6 (3 + 3)	None
5	5 (2 + 3)	None
4	4 (2 + 2)	None
3	3 (1 + 2)	None
2	2 (1 + 1)	None
1	1 (1 + 0)	None
0	0	None
0 + VL	0	With vision loss
0 + VL + PC	0	With vision loss and requiring palliative care

Disease progression increases with decreasing disease stage

CLN2, neuronal ceroid lipofuscinosis type 2; PC, palliative care; VL, vision loss