Table 3:
Disease progression over 2-years according to the CMTPedS Total score by CMT genetic subtype.
| CMT type | N | Baseline Score | Follow-up Score | Difference | Change (% from baseline) |
|---|---|---|---|---|---|
| All cases | 187 | 17.3±9.1 (1 – 40) |
19.6±9.4 (0 – 42) |
2.4±4.9* (95%CI, 1.7 – 3.1) |
14 |
| CMT1A | 111 | 14.6±7.1 (1 – 39) |
16.4±6.9 (0 – 36) |
1.8±4.2* (95%CI, 1.0 – 2.6) |
12 |
| CMT1B | 9 | 19.4±5.2 (14 – 30) |
21.7±6.6 (13 – 35) |
2.2±5.1 (95%CI, −1.7 – 6.1) |
11 |
| CMT1E | 5 | 27.6±9.0 (16 – 37) |
31.6±6.7 (23 – 38) |
4.0±4.6 (95%CI, −1.8 – 9.8) |
15 |
| CMT2A | 6 | 26.7±9.6#
(14 – 36) |
32.9±9.5 (17 – 42) |
6.2±7.9# (95%CI, −2.2 – 14.5) |
23 |
| CMT2D | 3 | 22.3±7.2 (14 – 27) |
28.7±12.7 (14 – 36) |
6.3±5.5 (95%CI, −7.4 – 20.0) |
28 |
| CMT2, unknown | 6 | 21.7±8.0 (14 – 36) |
26.0±5.5 (21 – 34) |
4.3±6.7 (95%CI, −2.7 – 11.3) |
20 |
| CMT4C | 7 | 26.1±11.5#
(8 – 38) |
29.1±11.0 (13 – 39) |
3.0±4.5 (95%CI, −1.2 – 7.2) |
12 |
| CMTX1 | 4 | 9.8±8.2 (1 – 19) |
12.3±10.0 (1 – 25) |
2.5±3.0 (95%CI, −2.3 – 7.3) |
26 |
| Unknown | 22 | 20.8±11.1 (2 – 40) |
22.7±10.2 (5 – 38) |
1.9±6.9 (95%CI, −1.1 – 5.0) |
9 |
Data are mean±SD (range) for baseline and follow-up scores and mean±SD (95% Confidence Interval) for Difference scores.
*
Significant change from baseline (p<0.001),
#
Significant difference to CMT1A (p=0.02).