Table 1.
Family. | Patient | Age at baseline | Disease duration at baseline | Duration of follow-up | MIRS |
|||||
---|---|---|---|---|---|---|---|---|---|---|
no. | no. | Sex | Relationship | (years) | (years) | Inheritance | CTG repeats | (years) | Basal | Follow-up |
1 | 1 | M | Proband | 60 | 17 | Paternal | 100 | 7 | 4 | 5 |
1 | 2 | F | Parent | 44 | 24 | Paternal | 500 | 20 | 4 | 5 |
1 | 3 | F | Sibling | 22 | 8 | Paternal | 300 | 19 | 2 | 4 |
2 | 4 | F | Sibling | 34 | 4 | Paternal | 625 | 15 | 3 | 3 |
2 | 5 | M | Sibling | 27 | 8 | Paternal | 625 | 16 | 2 | 3 |
3 | 6 | M | Proband | 24 | 12 | Paternal | 467 | 12 | 2 | 3 |
3 | 7 | F | Sibling | 24 | 10 | Paternal | 467 | 13 | 3 | 4 |
3 | 8 | M | Parent | 54 | 0 | Unknown | 66 | 12 | 1 | 2 |
4 | 9 | M | Proband | 43 | 10 | Maternal | 210 | 12 | 3 | 4 |
4 | 10 | M | Sibling | 16 | 2 | Paternal | 650 | 15 | 2 | 3 |
5 | 11 | M | Proband | 39 | 26 | Maternal | 405 | 9 | 4 | 5 |
5 | 12 | F | Sibling | 29 | 0 | Maternal | 255 | 16 | 2 | 4 |
13 | M | 39 | 10 | Maternal | 467 | 8 | 3 | 4 |
MIRS: Muscular impairment rating scale.40