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) |