Table 1.
Distribution of clinical categories among subjects carrying a bDRA.
| CCEF category (n) | Mean age at evaluation | Mean age at onset | Mean FSHD score |
|---|---|---|---|
| a. Index cases 134 (84 M, 50 F) | |||
| A 73 (46 M, 27 F) | 57.5 ± 15.8 | 32.9 ± 16.0 | 6.8 ± 3.0 |
| B 27 (20 M, 7 F) | 51.6 ± 15.1 | 35.3 ± 18.2 | 3.0 ± 1.8 |
| C 9 (4 M, 5 F) | 33.7 ± 20.9 | – | – |
| D 25 (14 M, 11 F) | 56.1 ± 13.0 | 38.2 ± 18.5 | 6.0 ± 3.4 |
| b. Relatives 110 (56 M, 54 F) | |||
| A 11 (3 M, 8 F) | 55.2 ± 19.3 | 32.2 ± 17.6 | 5.5 ± 2.8 |
| B 15 (12 M, 3 F) | 51.7 ± 19.7 | 35.3 ± 17.0 | 2.5 ± 2.1 |
| C 78 (39 M, 39 F) | 42.0 ± 16.0 | – | – |
| D 6 (2 M, 4 F) | 57.0 ± 27.8 | 45.0 ± 36.1 | 5.3 ± 4.5 |