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Table 2 Mean RSBQ subscale and total scores by covariates among individuals with Rett syndrome

From: Associations between genotype, phenotype and behaviours measured by the Rett syndrome behaviour questionnaire in Rett syndrome

 

Subscale

Total*

(0–90)

Factor (range)

Factor 1

General Mood

(0–16)

Factor 2 Breathing Problems

(0–10)

Factor 3

Hand Behaviours

(0–12)

Factor 4

Face Movements

(0–8)

Factor 5

Body Rocking

(0–12)

Factor 6

Night-time Behaviours

(0–6)

Factor 7

Fear/Anxiety

(0–8)

Factor 8*

Walking/

Standing

(0–4)

 

mean (SD)

Overall

5.4 (3.9)

3.8 (3.0)

7.2 (3.0)

2.4 (1.9)

4.4 (2.1)

1.2 (1.4)

3.3 (2.0)

1.8 (1.5)

35.0 (14.0)

Age, in years

         

 2–12

6.4 (3.7)

4.3 (2.8)

6.8 (2.9)

3.1 (2.1)

4.5 (2.3)

1.6 (1.5)

3.7 (2.1)

1.7 (1.6)

38.5 (14.7)

 13–17

4.9 (3.8)

3.9 (3.3)

7.3 (3.0)

2.4 (2.0)

4.4 (2.0)

0.9 (1.2)

3.4 (1.9)

1.8 (1.6)

34.7 (15.0)

 18–24

5.4 (3.9)

3.6 (2.8)

7.5 (2.8)

2.2 (1.7)

4.5 (2.2)

1.4 (1.6)

3.3 (2.1)

1.8 (1.5)

34.5 (13.1)

 25+

5.1 (4.0)

3.4 (2.9)

7.2 (3.1)

2.0 (1.7)

4.2 (2.0)

1.0 (1.4)

2.8 (1.8)

1.9 (1.5)

32.4 (12.5)

Variant

         

 C-terminal deletion

4.6 (3.3)

3.8 (3.1)

6.7 (3.3)

2.2 (1.6)

4.2 (2.1)

1.0 (1.3)

3.2 (2.3)

1.5 (1.4)

32.0 (13.1)

 Early truncation

4.9 (3.8)

4.2 (3.3)

7.7 (3.1)

2.4 (2.0)

4.1 (2.0)

1.1 (1.6)

2.6 (2.1)

1.9 (1.6)

34.1 (14.9)

 Large deletion

5.4 (3.6)

3.6 (3.3)

6.6 (3.2)

2.6 (1.8)

4.6 (1.7)

1.2 (1.4)

2.9 (1.8)

2.0 (1.5)

34.8 (13.6)

 p.Arg106Trp

5.7 (4.4)

3.6 (3.0)

7.7 (3.1)

1.9 (1.1)

5.2 (2.7)

1.2 (1.3)

2.9 (1.7)

2.0 (1.3)

35.3 (14.3)

 p.Arg133Cys

5.6 (4.2)

3.1 (2.8)

6.1 (3.3)

2.4 (2.3)

4.4 (2.2)

1.0 (1.3)

3.2 (1.9)

1.9 (1.4)

33.2 (15.5)

 p.Thr158Met

5.6 (3.9)

4.0 (3.1)

7.7 (2.6)

2.4 (1.9)

4.3 (1.9)

1.2 (1.4)

3.4 (2.2)

2.1 (1.6)

35.9 (13.7)

 p.Arg168*

4.8 (3.2)

3.7 (3.2)

7.9 (2.8)

2.9 (2.1)

4.0 (2.2)

1.5 (1.5)

3.5 (2.2)

1.3 (1.6)

35.7 (14.6)

 p.Arg255*

5.1 (4.5)

4.5 (3.3)

7.2 (3.2)

2.7 (2.3)

4.7 (2.2)

1.3 (1.5)

3.4 (2.2)

1.8 (1.7)

36.4 (16.4)

 p.Arg270*

5.4 (3.9)

3.9 (2.8)

8.2 (2.4)

2.7 (2.3)

4.3 (2.2)

1.5 (1.6)

3.1 (1.8)

1.7 (1.8)

36.9 (15.6)

 p.Arg294*

7.3 (3.7)

3.2 (2.9)

7.0 (2.9)

1.8 (1.8)

4.3 (2.2)

1.6 (1.9)

3.7 (1.7)

1.6 (1.2)

36.1 (11.8)

 Arg306Cys

6.4 (3.4)

3.0 (2.3)

7.0 (2.9)

2.4 (1.7)

4.6 (2.0)

1.7 (1.6)

4.0 (2.2)

1.8 (1.4)

34.7 (13.8)

 Other

5.9 (4.3)

4.3 (2.3)

7.5 (2.6)

2.3 (1.8)

4.1 (2.4)

1.0 (1.3)

3.1 (1.9)

2.0 (1.7)

35.8 (11.4)

 Unknown

5.6 (4.3)

4.0 (3.1)

6.0 (2.8)

2.8 (1.7)

4.9 (1.8)

0.8 (1.4)

3.4 (1.8)

2.0 (1.6)

35.5 (14.3)

Walking ability

         

 Unable

4.8 (3.5)

3.4 (2.8)

6.9 (3.0)

2.5 (1.8)

3.9 (2.0)

1.1 (1.3)

3.1 (2.1)

1.0 (1.3)

32.0 (13.0)

 Assisted

5.8 (4.1)

4.3 (3.0)

8.0 (2.8)

2.5 (2.1)

4.7 (2.2)

1.5 (1.6)

3.8 (2.0)

2.4 (1.4)

38.7 (14.4)

 Independent

5.7 (3.9)

3.5 (3.0)

6.8 (3.0)

2.3 (1.9)

4.5 (2.1)

1.1 (1.3)

3.0 (2.0)

2.1 (1.5)

34.3 (13.7)

Hand function

         

 Unable

4.8 (3.6)

4.0 (2.9)

8.0 (2.6)

2.6 (1.9)

4.3 (2.0)

1.1 (1.4)

3.1 (2.0)

1.7 (1.7)

34.7 (12.3)

 Large objects

5.8 (4.1)

4.7 (3.1)

8.5 (2.4)

2.8 (2.0)

4.7 (2.4)

1.3 (1.4)

3.8 (2.0)

1.8 (1.5)

39.5 (15.3)

 Small objects

5.9 (3.9)

3.1 (2.9)

5.8 (3.0)

2.1 (1.9)

4.3 (2.1)

1.3 (1.5)

3.3 (2.1)

1.9 (1.4)

32.9 (14.3)

Seizure frequency

         

 Never or controlled

5.7 (4.0)

3.1 (2.9)

6.7 (3.1)

2.3 (1.9)

4.4 (2.2)

1.2 (1.4)

3.2 (2.1)

1.7 (1.5)

33.5 (14.1)

 Monthly or less

5.0 (3.7)

4.1 (3.0)

7.6 (2.9)

2.4 (1.9)

4.3 (2.0)

1.0 (1.2)

3.3 (1.9)

1.8 (1.6)

34.7 (13.0)

 Weekly

5.5 (3.5)

4.8 (2.7)

7.7 (2.8)

2.8 (2.1)

4.5 (2.5)

1.5 (1.7)

3.5 (2.0)

1.9 (1.7)

38.4 (15.3)

 Daily

5.6 (4.1)

4.2 (3.1)

7.5 (2.6)

2.5 (1.7)

4.4 (1.8)

1.6 (1.7)

3.5 (2.1)

2.0 (1.5)

36.8 (13.8)

Constipation

         

 No

5.3 (3.8)

3.9 (3.1)

7.1 (3.0)

2.4 (2.0)

4.5 (2.1)

1.2 (1.4)

3.3 (2.0)

1.7 (1.5)

34.8 (14.0)

 Yes

5.7 (3.9)

3.6 (2.7)

7.4 (3.0)

2.5 (1.8)

4.2 (2.3)

1.4 (1.5)

3.4 (2.2)

2.0 (1.6)

35.6 (14.0)

Reflux

         

 No

5.7 (3.7)

4.2 (3.0)

7.3 (3.0)

2.6 (1.9)

4.4 (2.2)

1.4 (1.4)

3.4 (2.0)

2.0 (1.6)

36.7 (14.1)

 Yes

5.1 (4.0)

3.2 (2.9)

7.1 (2.9)

2.3 (2.0)

4.3 (2.0)

1.0 (1.4)

3.1 (2.0)

1.6 (1.5)

32.5 (13.2)

Insomnia

         

 Normal

4.8 (3.7)

3.7 (2.9)

7.1 (3.0)

2.3 (1.9)

4.2 (2.0)

0.9 (1.2)

3.0 (2.0)

1.8 (1.5)

32.7 (13.2)

 Abnormal

7.0 (3.8)

4.1 (3.1)

7.5 (3.0)

2.8 (2.0)

5.0 (2.3)

2.0 (1.6)

4.0 (1.9)

1.8 (1.6)

40.7 (14.4)

Excessive daytime sleepiness

         

 Normal

4.9 (3.7)

3.6 (2.9)

6.8 (3.0)

2.2 (1.9)

4.2 (2.1)

1.1 (1.3)

3.0 (1.9)

1.7 (1.5)

32.5 (13.4)

 Abnormal

7.1 (4.0)

4.6 (2.9)

8.5 (2.4)

3.1 (1.8)

5.2 (2.1)

1.8 (1.7)

4.4 (2.0)

2.1 (1.6)

43.3 (12.7)

Data source

         

 AussieRett

6.1 (3.7)

3.8 (2.9)

7.3 (3.0)

2.7 (1.9)

4.6 (2.2)

1.6 (1.4)

3.6 (2.1)

2.3 (1.5)

37.5 (13.8)

 InterRett

5.0 (3.9)

3.8 (3.0)

7.2 (3.0)

2.2 (1.9)

4.2 (2.1)

1.0 (1.4)

3.1 (2.0)

1.5 (1.4)

33.3 (13.9)

  1. * n = 362
  2. SD, standard deviation
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Journal of Neurodevelopmental Disorders

ISSN: 1866-1955

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