Skip to main content

Table 2 Clinical and phenotypic characteristics of SCA 3 patients in Malaysia using INAS

From: Frequency of Spinocerebellar Ataxia type 1, 2, 3,6 and 7 and clinical profile of Spinocerebellar Ataxia type 3 in Malaysia

Baseline characteristics (n = 15)

 

Gender:

 Male (n, %)

9.0(60.0%)

 Female (n, %)

6.0(40.0%)

 Mean age of presentation (years,(SD))

35.6(±9.2)

 Mean age of onset (years, (SD)

31.2(±8.1)

 Mean duration of illness (years, (SD))

4.5(±2.9)

 Median time to wheel chair (months, (IQR))

12.0(12.0–24.0)

 Median time to ophthalmoplegia (months, IQR))

12.0(6.0–21.0)

 Mean SARA at baseline score (score (SD))

12.3(±8.9)

Phenotypic Characteristics (n, %)

 Broken smooth pursuit

7(46.7)

 Square jerk fixation

7(46.7)

 Downbeat nystagmus

4(26.7)

 Gaze evoked horizontal nystagmus

8(53.3.)

 Gaze evoked vertical nystagmus

3(20.0)

 Ophthalmoparesis on horizontal nystagmus

7(46.7)

 Opthalmoparesis vertical gaze

7(46.7)

 Slowing saccades

7(46.7)

 Hypermetric saccades

3(20.0)

 Hypometric saccades

7(46.7)

 Diplopia

7(46.7)

 Ophthalmoplegia

7(46.7)

 Hyperreflexia

9(60.0)

 Extensor plantar reflex

3(20.0)

 Clonus

2(13.3)

 Spasticity

6(40.0)

 Rigidity

2(13.3)

 Resting tremor

0 (0)

 Dystonia

2(13.3)

 Paresis

0 (0)

 Muscle atrophy

2(13.3)

 Fasciculation

2(13.3)

 Chorea

0 (0)

 Impaired vibration sense

1(6.7)

 Dysarthria

14(93.3)

 Dysphagia

3(20.0)

 Cognitive impairment

1(6.7)