Table 1.
Clinical characterization of the French CMT4G cohort.
| Id. # Patient |
Age at Onset of LL Weakness (Years) |
Age at Onset of UL Weakness (Years) |
Foot Deformities | CMTNS | MNCV Median (m/s) | CMAP Median, (mV) | SNAP |
|---|---|---|---|---|---|---|---|
| 1 | 6 | 16 | +Arthrodesis | 24 | 16.7 (38) | 0.2 | NR |
| 2 | 6 | 15 | +Arthrodesis | 23 | NR (29) | NR | NR |
| 3 | 9 | 14 | + | 28 | NR (65) | NR | NR |
| 4 | 10 | 11 | + Arthrodesis | 12 | 11.2 (10) | 0.4 | NR |
| 5 | 7 | ND | - | ND | ND | ND | ND |
| 6 | 9 | ND | + | ND | ND | ND | ND |
| 7 | 7 | 16 | +Arthrodesis | ND | ND | ND | ND |
| 8 | 5 | 16 | - | 23 | 29 (9) | 1.5 | NR |
| 9 | 9 | 16 | - | 19 | 16.2 (21) | 1.4 | NR |
| 10 | 8 | ND | +Arthrodesis | 6 | 24.1 (10) | 5.6 | NR |
| 11 | 3 | ND | - | 1 | 28.9 (3) | 4.3 | NR |
| 12 | 4 | ND | + | 8 | 32 (9) | 1.93 | NR |
| 13 | 12 | 35 | - | 25 | ND | ND | ND |
| 14 | 10 | - | ND | - | - | - | - |
| 15 | 3 | - | ND | - | - | - | - |
| 16 | 3 | 16 | +Arthrodesis | 16 | 21 (36) | 0.59 | NR |
| 17 | 3 | - | ND | - | - | - | - |
| 18 | 10 | 10 | + Arthrodesis | 17 | 23.8 (12) | - | NR |
| 19 | 11 | 45 | ND | ND | ND | ND | ND |
| 20 | 13 | 20 | +Arthrodesis | - | - | - | - |
| 21 | 6 | ND | - | 6 | 30.6 (7) | 5.3 | NR |
| 22 | 10 | 14 | +Arthrodesis | ND | 22.2 (8) | 1.19 | NR |
| 23 | 5 | ND | - | ND | 24 (6) | 6.4 | NR |
| 24 | 4 | 7 | - | 20 | 16 (27) | 0.45 | NR |
| 25 | 12 | 8 | - | ND | ND | ND | ND |
CMAP: compound motor action potential; CMTNS: Charcot–Marie–Tooth Neuropathy Score; MNCV: motor nerve conduction velocity; NR: no response; ND: not done; SNAP: sensory nerve action potential.