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Table 7 Frequency of ranking symptom in top 5 by disease severity of patient

From: Validation of the 5-domain Niemann-Pick type C Clinical Severity Scale

n (%) Disease severity based on responses to patient/caregiver reported 5-domain NPCCSS in the survey Overall
(n = 49)
 ≤ 4 (n = 7) 5–19 (n = 36)  ≥ 20 (n = 6)
Ambulation 6 (85.7) 31 (86.1) 4 (66.7) 41 (83.7)
Fine motor skills 2 (28.6) 17 (47.2) 3 (50.0) 22 (44.9)
Speech 4 (57.1) 26 (72.2) 2 (33.3) 32 (65.3)
Swallow 3 (42.9) 26 (72.2) 6 (100.0) 35 (71.4)
Cognition 5 (71.4) 16 (44.4) 2 (33.3) 23 (46.9)