Patient | Age at onset (year) | Age at biopsy (year) | CAGs (nr) | ADL scale grade at biopsy | Muscle pathology |
---|---|---|---|---|---|
1 | 45 | 61 | 47 | 2 | II |
2 | 46 | 43 | 42 | 1 | I |
3 | 50 | 57 | 45 | 2 | II |
4 | 64 | 71 | 44 | 1 | III |
5 | 41 | 45 | 47 | 0 | II |
6 | 37 | 44 | 50 | 1 | III |
7 | 46 | 55 | 48 | 1 | III |
8 | 24 | 28 | 44 | 1 | I |
9 | 29 | 39 | 47 | 1 | III |
10 | 49 | 55 | 45 | 2 | III |
11 | 48 | 51 | 44 | 1 | II |
12 | 35 | 38 | 50 | 0 | III |
13 | 50 | 52 | 44 | 1 | III |
14 | 54 | 54 | 44 | 0 | II |
15 | 52 | 56 | 41 | 2 | III |
16 | 58 | 70 | 45 | 2 | II |
17 | 70 | 80 | 42 | 1 | III |
18 | 68 | 68 | 45 | 1 | II |
19 | 45 | 57 | 46 | 2 | III |
20 | 50 | 59 | 47 | 2 | I |
ADL scale, activity of daily living scale (0: normal; 1: mild weakness of limb muscles, climbs stairs easily but aware of weakness; 2: mild-to-moderate weakness, climbs stairs with difficulty and generally uses a cane; 3: moderate-to-severe weakness, uses a wheelchair most of the time or mostly recumbent); muscle pathology (type I, only signs of neurogenic atrophy; type II, signs of neurogenic atrophy plus 1 or 2 myopathic changes; type III, signs of neurogenic atrophy plus 3 or more myopathic changes); nr, number of triplets.