Table 5 Adjusted* associations of covariates with selected RSBQ scores in adults with Rett syndrome

n

179

179

179

179

178

Adjusted coefficient (95% CI), P-value

Age, in years

 18–24

-0.21 (-1.36,0.94),0.72

0.34 (-0.50,1.18),0.42

0.31 (-0.12,0.74),0.15

0.37 (-0.24,0.98),0.23

1.48 (-1.98,4.95),0.40

 25+

Ref

Ref

Ref

Ref

Ref

Variant

 C-terminal deletion

Ref

Ref

Ref

Ref

Ref

 Early truncation

-0.72 (-3.70,2.27),0.64

0.44 (-1.75,2.62),0.69

0.56 (-0.56,1.67),0.32

-1.35 (-2.93,0.23),0.09

-1.18 (-10.16,7.80),0.80

 Large deletion

1.49 (-1.24,4.22),0.28

0.46 (-1.54,2.46),0.65

0.62 (-0.40,1.64),0.23

-1.32 (-2.77,0.12),0.07

3.43 (-4.77,11.64),0.41

 p.Arg106Trp

-1.47 (-4.30,1.36),0.31

-0.55 (-2.62,1.52),0.60

-0.27 (-1.32,0.79),0.62

-0.59 (-2.09,0.91),0.44

-3.23 (-11.74,5.27),0.45

 p.Arg133Cys

-0.49 (-2.99,2.01),0.70

-0.74 (-2.57,1.09),0.43

-0.04 (-0.97,0.90),0.94

0.02 (-1.31,1.34),0.98

-2.43 (-9.95,5.09),0.52

 p.Thr158Met

0.06 (-2.42,2.54),0.96

0.00 (-1.81,1.82),1.00

0.03 (-0.90,0.95),0.95

0.23 (-1.09,1.54),0.74

-0.57 (-8.02,6.89),0.88

 p.Arg168*

0.81 (-1.51,3.14),0.49

0.17 (-1.53,1.87),0.84

0.79 (-0.08,1.66),0.08

0.24 (-0.99,1.47),0.70

4.61 (-2.39,11.62),0.20

 p.Arg255*

1.69 (-0.73,4.11),0.17

0.57 (-1.20,2.34),0.53

1.31 (0.41,2.21),<0.01

0.11 (-1.17,1.39),0.86

7.05 (-0.22,14.32),0.06

 p.Arg270*

1.06 (-2.09,4.21),0.51

-1.36 (-3.67,0.95),0.25

0.29 (-0.89,1.47),0.63

-0.29 (-1.96,1.37),0.73

-2.13 (-11.61,7.35),0.66

 p.Arg294*

1.56 (-1.08,4.20),0.25

-0.22 (-2.15,1.71),0.82

0.51 (-0.48,1.49),0.31

0.78 (-0.61,2.18),0.27

3.39 (-4.54,11.32),0.40

 Arg306Cys

1.21 (-1.44,3.86),0.37

-0.40 (-2.34,1.54),0.68

0.62 (-0.37,1.61),0.22

0.29 (-1.11,1.69),0.68

2.21 (-5.96,10.38),0.59

 Other

1.80 (-0.55,4.14),0.13

1.01 (-0.71,2.73),0.25

0.48 (-0.39,1.36),0.28

-0.18 (-1.42,1.06),0.78

4.86 (-2.20,11.92),0.18

 Unknown

0.11 (-3.62,3.85),0.95

0.34 (-2.39,3.07),0.81

-0.28 (-1.68,1.11),0.69

0.17 (-1.81,2.14),0.87

-1.63 (-12.86,9.59),0.77

Walking ability

 Unable

-0.94 (-2.39,0.52),0.20

-0.29 (-1.36,0.77),0.59

0.05 (-0.49,0.59),0.86

-0.13 (-0.90,0.63),0.73

-4.28 (-8.64,0.09),0.05

 Assisted

1.10 (-0.29,2.49),0.12

0.85 (-0.16,1.87),0.10

0.63 (0.11,1.15),0.02

0.73 (-0.00,1.47),0.05

7.09 (2.92,11.27),<0.01

 Independent

Ref

Ref

Ref

Ref

Ref

Hand function

 Unable

-0.92 (-2.33,0.48),0.20

1.10 (0.07,2.13),0.04

-0.14 (-0.66,0.39),0.60

0.10 (-0.64,0.85),0.79

3.55 (-0.70,7.80),0.10

 Large objects

-0.91 (-2.80,0.98),0.34

0.97 (-0.41,2.35),0.17

-0.63 (-1.33,0.08),0.08

0.40 (-0.60,1.40),0.43

4.92 (-0.79,10.62),0.09

 Small objects

Ref

Ref

Ref

Ref

Ref

Seizure frequency

 Never or controlled

Ref

Ref

Ref

Ref

Ref

 Monthly or less

-1.47 (-2.84,-0.10),0.04

1.26 (0.26,2.26),0.01

-0.47 (-0.99,0.04),0.07

-0.27 (-1.00,0.46),0.46

-1.10 (-5.22,3.03),0.60

 Weekly

-0.49 (-2.33,1.34),0.60

1.72 (0.38,3.06),0.01

0.12 (-0.57,0.80),0.73

-0.52 (-1.49,0.45),0.29

1.39 (-4.13,6.90),0.62

 Daily

-1.37 (-3.34,0.61),0.17

0.54 (-0.90,1.98),0.46

-0.15 (-0.88,0.59),0.69

-0.76 (-1.80,0.28),0.15

-5.35 (-11.48,0.79),0.09

Constipation

 No

Ref

Ref

Ref

Ref

Ref

 Yes

-0.11 (-1.53,1.31),0.88

0.68 (-0.36,1.71),0.20

0.14 (-0.38,0.67),0.59

0.20 (-0.55,0.95),0.59

2.32 (-1.94,6.59),0.28

Reflux

 No

Ref

Ref

Ref

Ref

Ref

 Yes

0.28 (-0.98,1.54),0.66

-1.40 (-2.32,-0.48),<0.01

-0.20 (-0.67,0.27),0.40

0.11 (-0.55,0.78),0.74

-1.79 (-5.61,2.03),0.35

Insomnia

 Normal

Ref

Ref

Ref

Ref

Ref

 Abnormal

2.46 (1.08,3.85),<0.01

0.24 (-0.77,1.25),0.64

1.17 (0.65,1.69),<0.01

0.53 (-0.21,1.26),0.16

6.99 (2.82,11.16),<0.01

Excessive daytime sleepiness

 Normal

Ref

Ref

Ref

Ref

Ref

 Abnormal

1.35 (-0.22,2.91),0.09

-0.38 (-1.53,0.76),0.51

0.16 (-0.43,0.74),0.60

0.71 (-0.11,1.54),0.09

2.78 (-1.96,7.52),0.25

Data source

 AussieRett

Ref

Ref

Ref

Ref

Ref

 InterRett

-1.34 (-2.74,0.07),0.06

-0.27 (-1.30,0.76),0.61

-0.35 (-0.87,0.18),0.19

-0.97 (-1.71,-0.22),0.01

-5.29 (-9.52,-1.06),0.01