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Table 7 Mean 5-domain NPCCSS score by participant videos

From: Clinical disease progression and biomarkers in Niemann–Pick disease type C: a prospective cohort study

Participants* MEAN 5-domain NPCCSS score (± SD)
Initial rating Repeat rating
Participant A 5.85 (2.115) 7.00 (1.633)
Participant B 11.08 (2.326) 10.46 (1.713)
Participant C 18.15 (1.772) 18.85 (1.068)
Participant D 4.46 (1.391) 4.77 (2.204)
p value  < 0.0001  < 0.0001
  1. * Facets measures were as follows: participant C = 3.30 (most severe); participant B = 0.22; participant A = –1.32; participant D = –2.20 (least severe)
  2. NPCCSS: Niemann–Pick type C disease Clinical Severity Scale