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Table 1 Summary of disease stages

From: Investigating health-related quality of life in rare diseases: a case study in utility value determination for patients with CLN2 disease (neuronal ceroid lipofuscinosis type 2)

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
  1. Disease progression increases with decreasing disease stage
  2. CLN2, neuronal ceroid lipofuscinosis type 2; PC, palliative care; VL, vision loss