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Table 2 Comparison of clinical profile and features of unrelated Thai patients with SCA1, SCA2, MJD and SCA6

From: Clinical analysis of adult-onset spinocerebellar ataxias in Thailand

 

SCA1

SCA2

MJD

SCA6

p-value*

Total numbers

21

15

39

8

 

Sex (M/F)

11/10

8/7

14/25

4/4

0.500

Age at onset (years)

     

 Mean age at onset

38.43

41.47

39.97

43.5

0.715

 SD

10.14

13.01

12.17

6.76

 

 Range

15 – 55

19 - 60

16 - 64

34 - 58

 

Duration (years)

     

 Mean duration

5.43

3.93

6.62

6

0.328

 SD

5.13

2.99

5.01

5.90

 

 Range

1 - 20

1 - 10

0.25 - 22

1 - 18

 

Positive family history (%)

16 (76.2)

13 (86.7)

32 (82.1)

7 (87.5)

0.900

CAG repeat size (repeats)

     

 Mean

50.14

37.40

69.97

21.88

 

 SD

6.27

4.47

4.04

0.83

 

 Range

41 – 65

32 - 52

62 - 78

21 - 23

 

Clinical features (%)

     

 Slow saccade

12 (57.1)

7 (46.7)

21 (53.9)

2 (25)

0.454

 Horizontal nystagmus

5 (23.8)

4 (26.7)

34 (87.2)

6 (75)

0.0000002**

 Vertical nystagmus

0 (0)

1 (6.7)

6 (15.4)

3 (37.5)

0.026**

 Opthalmoparesis

6 (28.6)

3 (20.0)

26 (66.7)

2 (25)

0.002**

 Pale optic disc

1 (4.8)

0 (0)

2 (5.1)

1 (12.5)

0.610

 Hyperreflexia

19 (90.5)

5 (33.3)

26 (66.7)

7 (87.5)

0.002**

 Babinski’s sign

11 (52.4)

5 (33.3)

17 (43.6)

5 (62.5)

0.510

 Areflexia

0 (0)

5 (35.7)

9 (23.1)

0 (0)

0.011**

 Sensory impairment

4 (19.0)

3 (20.0)

9 (23.1)

0 (0)

0.630

 Parkinsonism

0 (0)

0 (0)

0 (0)

0 (0)

1.000

 Dystonia

1 (4.8)

0 (0)

1 (2.6)

0 (0)

0.475

 Chorea

0 (0)

0 (0)

1 (2.6)

0 (0)

1.000

 Dementia

0 (0)

1 (6.7)

0 (0)

1 (12.5)

0.074

 Facial fasciculation

2 (9.5)

0 (0)

5 (12.8)

0 (0)

0.579

SARA

     

 Mean scale

16.97

13.18

16.76

15.71

0.767

 SD

7.45

3.66

7.27

7.24

 

 Range

4 - 30

9 - 20

8 - 35

3.5 - 27

 
  1. *Continuous variables were analyzed by using ANOVA. Comparisons of frequencies of clinical profiles and features were analyzed by using Fisher’s exact test.
  2. **P < 0.05 is considered as significant difference.
  3. Abbreviations as follows: SCA spinocerebellar ataxia, MJD Machado-Joseph disease, SD standard deviation, SARA scale for the assessment and rating of ataxia.