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. 2020 Jun 9;94(23):e2436–e2440. doi: 10.1212/WNL.0000000000009454

Figure 2. Relationships among UBDRS subscale severity scores, CLN3 Staging System stages, and participant age.

Figure 2

Distributions of Unified Batten Disease Rating Scale (UBDRS) (A) Physical and (B) capability subscale scores for each of the stages. Mean severity scores and 95% confidence intervals are represented by diamonds; median, quartiles, and range are represented by box-and-whisker plots. Individual points represent outliers, which are >1.5 times the interquartile range above the third quartile or below the first. (A) t Tests showed significant differences between stages (stage 1 vs 2: t = 7.1, p < 0.0001; stage 2 vs 3: t = 24.4, p < 0.0001; stage 1 vs 3: t = 22.7, p < 0.0001). (B) t Tests showed significant differences between stages (stage 1 vs 2: t = 9.4, p < 0.0001; stage 2 vs 3: t = 20.2, p < 0.0001; stage 1 vs 3: t = 14.6, p < 0.0001). Data density contour plots, by stage, of relationship between age and severity for UBDRS (C) Physical and (D) Capability subscale scores. Stage 1 data are represented in green; stage 2, in blue; and stage 3, in red. Data density is represented by color density.