Table 1.

5-domain NPCCSS

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)

^*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))