Table e-1Baseline characteristics of patients with and without at least one follow-up
Patients with follow-up / Patients without follow-up / p-valueSCA1 Patients (n) / 113 / 4
Gender (M) / 70 (62%) / 1 (25%)
Repeat length expanded allele / 48 ± 6
(39 – 67) 47 / 47 ± 1
(46 – 47) 46,5
Repeat length normal allele / 30 ± 2
(23 – 36) 29 / 29 ± 0
(29 – 29) 29
Age [years] / 46 ± 12
(18 – 76) 46 / 51 ± 10
(43 – 66) 48
Age at onset [years] / 37 ± 11
(15 – 65) 37,5 / 34 ± 4
(29 – 38) 34,5
Disease duration [years] / 9 ± 5
(1 – 23) 8 / 17 ± 8
(10-28) 15,5
SARA score / 15 ± 9
(2 – 40) 13 / 25 ± 7
(18,5 – 34) 23
INAS count / 5 ± 2
(1– 11) 5 / 4 ± 1
(4 – 5) 4
SCA2 Patients (n) / 158 / 5
Gender (M) / 73 (46%) / 2 (40%)
Repeat length expanded allele / 39 ± 3
(33-52) 39 / 38 ± 1
(37-39) 37
Repeat length normal allele / 22 ± 1
(14-33) 22 / 22 ± 0
(22-22) 22
Age [years] / 46 ± 13
(18-84) 47 / 52 ± 12
(35-66) 50 / 0.27
Age at onset [years] / 35 ± 13
(7-66) 35 / 34 ± 12
(16-46) 40 / 0.99
Disease duration [years] / 11 ± 6
(0-40) 10 / 18 ± 11
(7-33) 14 / 0.15
SARA score / 16 ± 8
(2-39) 14 / 15 ± 8
(5-26) 15 / 0.91
INAS count / 5 ± 2
(1-13) 4 / 2 ± 2
(0-4) 2,5
SCA3 Patients (n) / 128 / 11
Gender (M) / 66 (52%) / 7 (64%) / 0.53
Repeat length expanded allele / 69 ± 4
(56-79) 69 / 68 ± 6
(59-73) 71 / 0.91
Repeat length normal allele / 21 ± 5
(14-35) 22 / 22 ± 5
(14-28) 22 / 0.59
Age [years] / 49 ± 12
(24-81) 48 / 42 ± 11
(14-55) 43 / 0.08
Age at onset [years] / 38 ± 11
(16-76) 37 / 28 ± 10
(5-42) 27 / 0.0106
Disease duration [years] / 11 ± 6
(1-28) 10 / 14 ± 7
(6-25) 14 / 0.25
SARA score / 14 ± 8
(1-39) 13 / 24 ± 10
(11-40) 23,5 / 0.0029
INAS count / 5 ± 2
(0-12) 5 / 7 ± 4
(3-12) 6 / 0.40
SCA6 Patients (n) / 91 / 16
Gender (M) / 51 (56%) / 7 (44%) / 0.42
Repeat length expanded allele / 22 ± 1
(21-28) 22 / 22 ± 0
(22-23) 22 / 0.19
Repeat length normal allele / 13 ± 1
(8-16) 13 / 13 ± 1
(11-14) 13 / 0.38
Age [years] / 64 ± 11
(37-85) 66,5 / 67 ± 12
(49-84) 67 / 0.54
Age at onset [years] / 54 ± 10
(31-77) 56 / 54 ± 9
(41-74) 54 / 0.66
Disease duration [years] / 10 ± 6
(1-33) 9 / 13 ± 9
(3-30) 10 / 0.35
SARA score / 15 ± 6
(1-33) 13,5 / 16 ± 8
(3-33) 16 / 0.51
INAS count / 2 ± 2
(0-9) 2 / 2 ± 2
(0-4) 2 / 0.45
If applicable, values are given as mean ± SD with the ranges in brackets.
P-values are computed only when at least 5 patients were available.