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Table 1 5-domain NPCCSS

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

Domain Scoring Minimum–Maximum Score
Ambulation 0 = Normal
1 = Clumsy
2 = Ataxic unassisted gait or not walking by 18 months
4 = Assisted ambulation or not walking by 24 months
5 = Wheelchair dependent
0–5
Fine Motor Skills 0 = Normal
1 = Slight dysmetria/dystonia (independent manipulation)
2 = Mild dysmetria/Dystonia (requires little to no assistance, able to feed self without difficulty)
4 = Moderate dysmetria/dystonia (limited fine motor skills, difficulty feeding self)
5 = Severe dysmetria/Dystonia (gross motor limitation, requires assistance for selfcare activities)
0–5
Swallow 0 = Normal, no dysphagia
1 = Cough while eating
Intermittent dysphagia*
 + 1 = w/Liquids
 + 1 = w/Solids
Dysphagia*
 + 2 = w/Liquids
 + 2 = w/Solids
4 = Nasogastric tube or gastric tube for supplemental feeding
5 = Nasogastric tube or gastric tube feeding only
0–5
Cognition 0 = Normal
1 = Mild learning delay, grade appropriate for age
3 = Moderate learning delay, individualized curriculum or modified work setting
4 = Severe delay/plateau, no longer in school or no longer able to work, some loss of cognitive function^
5 = Minimal cognitive function
0–5
Speech 0 = Normal
1 = Mild dysarthria (easily Understood
2 = Severe dysarthria (difficult to understand)
3 = Non-verbal/functional communication skills for needs
5 = Minimal communication
0–5
5-domain NPCCSS score Sum of all scores from the 5 domains above 0–25
(higher score = more severe clinical impairment)
  1. *Score is additive (to the “cough while eating”-score of 1) within the two subsections of intermittent dysphagia and dysphagia (example: for intermittent dysphagia with solids and dysphagia with liquids a score of 4 applies (1 + 1 + 2))