Table 2.
Motor nerve conduction findings in patients with CMT1C Gly112Ser LITAF/SIMPLE mutation and in those with CMT1A
| Normal | CMT1C (n = 7) | CMT1A[17] (n = 119) | |
|---|---|---|---|
| 1. Data expressed as mean ± standard deviation (range). CMAP, compound muscle action potential (motor, millivolts); DML, distal motor latency; MNCV, motor nerve conduction velocity; amplitudes represent baseline to peak values; TA, tibialis anterior. | |||
| 2. a | |||
| Fibular motor recording from the tibialis anterior (TA) was not performed. | |||
| Median nerve | No response = 0 | No response = 4 | |
| DML (ms) | <4.5 | 6.1 ± 1.3 (4.6–7.9) | 9.8 ± 2.6 (4.6–22.3) |
| CMAP (mV) | |||
| Distal | 4.9 ± 1.4 (3.6–7.5) | 2.4 ± 1.9 (0.1–9.5) | |
| Proximal | >4 | 4.2 (2.7–5.8) | |
| MNCV (m/s) | >48 | 27.9 ± 6.4 (20–38) | 20.2 ± 4.9 (7–33) |
| Ulnar nerve | No response = 0 | No response = 0 | |
| DML (ms) | <3.5 | 4.3 ± 0.81 (2.9–5.4) | 8 ± 3.1 (4.5–16.4) |
| CMAP (mV) | |||
| Distal | >6 | 5.5 ± 1.93 (2.9–5.4) | 2.4 ± 1.6 (0.1–9.5) |
| Proximal | >6 | 4.0 (0.99–7.5) | |
| MNCV (m/s) | >49 | 28 ± 7.2 (20.3–38.5) | 17.3 ± 5.1 (6–30) |
| Fibular nerve/EDB | No response = 1 | No response = 24 | |
| DML (ms) | <5.5 | 9.5 ± 3.1 (5.8–13.3) | 11.9 ± 3.4 (6–19.4) |
| CMAP (mV) | |||
| Distal | >3 | 1.9 ± 5.7 (0.27–4) | 0.9 ± 1.2 (0.07–4) |
| Proximal | >3 | 1.4 (0.2–2.97) | |
| MNCV (m/s) | >41 | 20 ± 0.058 (19.7–20) | 17 ± 4.6 (5–24) |
| Fibular nerve, TA | No response = 0 | Not availablea | |
| DML (ms) | <6.7 | 4.6 ± 0.86 (3.8–6) | Not availablea |
| CMAP (mV) | Not availablea | ||
| Distal | >5 | 3.7 ± 0.11 (3.2–4.5) | |
| Proximal | >5 | 3.4 (2.7–4.4) | |
| MNCV (m/s) | >44 | 26 ± 2.83 (19.7–33) | |