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Table 5 Progression rates

From: Disease progression in women with X-linked adrenoleukodystrophy is slow

 

N

Clinical progression

Stable score

Improvement score

Baseline (range)

Follow-up (range)

Progression rates per year

All women

Symptomatic at baseline

(N = 19)

Asymptomatic at baseline

(N = 15)

EDSS

32

21

11

0

2.75

(0–6)

3.5

(1.5–6)

0.08

0.06

0.17

ALDS

34

10

17

7

89.47

(71.92–89.47)

89.47

(71.92–89.47)

0.00

0.00

0.00

SF-36: PF

34

19

1

14

0.22

(−2.66–1.16)

− 0.07

(−2.86–1.26)

0.00

−0.03

0.02

SF-36: PCS

34

21

0

13

50.11

(17.26–62.36)

49.16

(16.67–64.72)

− 0.21

− 0.17

− 0.37

  1. Progression rates were calculated with values unadjusted for covariates. The EDSS ranges from 0 (normal) to 10 (death). The ALDS scores are regression coefficients which were linearly transformed for interpretation, ranging from 10 (highest level of disability) to 89.47 (lowest level of disability). The SF-36 subdomain physical functioning is expressed in Z scores, ranging from −4 (lowest quality of life) to + 4 (highest quality of life). The SF-36 physical component summary score was linearly transformed to a range from 0 (lowest quality of life) to 100 (highest quality of life), with a mean of 50 and standard deviation of 10. ALDS = AMC Linear Disability Scale; EDSS = Expanded Disability Status Scale; N = number of women; PCS = physical component summary; PF = physical functioning