Exploring the relationships between composite scores of disease severity, seizure-freedom and quality of life in Dravet syndrome

Table 1 Composite symptom scores and their components

Variable	Definition	Range of scores
Composite symptom score
CS1	CS2 = MM + MH + MS + BA + LS + CD	0–18
CS2	CS2 = CS1 + D + CG	0–24
CS3	CS3 = CS1 + 1.5 x (D + CG)	0–24
Domain
Physical	MM + MH + MS	0–9
Psychosocial	BA + LS + CD	0–9
Care requirement	D + CG	0–6
Sub-domains
MM	0 = no problem, 1 = minor problem, 2 = moderate problem, 3 = severe problem	0–3
MH	0 = no problem, 1 = minor problem, 2 = moderate problem, 3 = severe problem	0–3
MS	0 = no problem, 1 = minor problem, 2 = moderate problem, 3 = severe problem	0–3
BA	0 = no problem, 1 = minor problem, 2 = moderate problem, 3 = severe problem	0–3
LS	0 = no problem, 1 = minor problem, 2 = moderate problem, 3 = severe problem	0–3
CD	0 = no problem, 1 = minor problem, 2 = moderate problem, 3 = severe problem	0–3
D	0 = no disability ID, 1 = disability score 10–30%, 2 = disability score < 50% and eligible for financial compensation, 3 = disability score ≥ 50%¹	0–3
CG	0 = not in need of care (level 1)²; reimbursement received €689³, 1 = not in need of care (level 1)²; reimbursement received €1,298³, 2 = no care level but in need of care (level 2)²; reimbursement received €1,612³, 3 = receiving care (level 3)²; reimbursement received €1,995³	0–3

MM motor movement problems score; MH muscular hypotonia score; MS muscular spasticity score; BA behaviour and attention problems score; LS language and speech problems score; CD cognitive disorders score; D disability score; CS care grade score
¹Described legally as a severe disability [21]
²based on the pre-2017 care level rating expressed on the 1–3 (Pflegebedürftigkeit—Need of care) scale [13]
³based on Die Pflegestärkungsgesetze I-III—Nursing improvement laws I-III, 2017 [7]

ISSN: 2524-3489