| Electrophysiological characteristics |
Patients with the 1,5MB deletion of PMP22 gene |
Patients with another genetic anomaly of the PMP22 gene |
Normal limits of our laboratory |
Frequency of abnormal results (%) |
| |
Mean ± SD |
Mean ± SD |
|
|
| Median nerve (n=35) |
|
|
|
|
| Motor CV (m/s) |
53.0 ± 5.1 |
48.7 ± 8.5 |
≥ 53 |
49 |
| DML (ms) |
5.2 ± 1.6 |
4.7 ± 0.5 |
≤ 3.7 |
100 |
| TLI |
0.19 ± 0.03 |
0.22 ± 0.03 |
≥ 0.25 |
100 |
| Motor amplitude A1 (mV) |
7.3 ± 3.4 |
7.2 ± 1.6 |
≥ 4.8 |
17 |
| Motor amplitude A2 (mV) |
7.1 ± 3.4 |
6.5 ± 1.4 |
≥ 4.8 |
17 |
| Conduction block |
|
|
|
3 |
| F-wave latency (ms) |
30.7 ± 4.4 |
32.0 ± 4.9 |
≤ 30.6 |
37 |
| F-wave velocity (m/s) |
56.4 ± 5.5 |
51.7 ± 8.5 |
≥ 54 |
23 |
| Ulnar nerve (n=35) |
|
|
|
|
| Distal motor CV (m/s) |
52.8 ± 5.2 |
50.7 ± 7.8 |
≥ 53 |
54 |
| Motor CV at the elbow (m/s) |
36.6 ± 8.7 |
34.0 ± 9.8 |
≥ 44 |
86 |
| DML (ms) |
3.1 ± 0.6 |
3.0 ± 0.5 |
≤ 3.0 |
46 |
| TLI |
0.01 ± 0.01 |
0.01 ± 0.00 |
≥ 0.25 |
100 |
| Motor amplitude A1 (mV) |
8.7 ± 2.1 |
7.7 ± 0.2 |
≥ 5.6 |
6 |
| Motor amplitude A2 (mV) |
8.2 ± 2.1 |
6.9 ± 0.8 |
≥ 4.5 |
3 |
| Motor amplitude A3 (mV) |
6.9 ± 2.6 |
6.4 ± 0.4 |
≥ 4.5 |
17 |
| Conduction block |
|
|
|
20 |
| F-wave latency (ms) |
30.8 ± 7.3 |
34.0 ± 6.6 |
≤ 32.2 |
37 |
| F-wave velocity (m/s) |
48.3 ± 10.5 |
46.0 ± 8.7 |
≥ 50 |
43 |
| |
Patients with the 1,5MB deletion of PMP22 gene |
Patients with another genetic anomaly of the PMP22 gene |
Normal limits of our laboratory |
Frequency of abnormal results (%) |
| |
Mean ± SD |
Mean ± SD |
|
|
| Peroneal nerve (n=32) |
|
|
|
|
| Distal motor CV (m/s) |
39.4 ± 4.6 |
37.0 ± 9.6 |
≥ 41 |
59 |
| Motor CV at the fibular neck (m/s) |
38.7 ± 8.8 |
29.3 ± 7.5 |
≥ 39 |
59 |
| DML (ms) |
7.3 ± 2.7 |
5.3 ± 1.0 |
≤ 5.5 |
78 |
| TLI |
0.01 ± 0.00 |
0.02 ± 0.00 |
≥ 0.25 |
100 |
| Motor amplitude A1 (mV) |
4.0 ± 1.7 |
1.7 ± 2.2 |
≥ 3.3 |
28 |
| Motor amplitude A2 (mV) |
3.7 ± 1.7 |
1.6 ± 2.1 |
≥ 3.0 |
28 |
| Motor amplitude A3 (mV) |
3.3 ± 1.7 |
1.6 ± 2.3 |
≥ 2.9 |
38 |
| Conduction block |
|
|
|
13 |
| F-wave latency (ms) |
57.4 ± 10.5 |
62.1 ± 18.1 |
≤ 57.9 |
56 |
| F-wave velocity (m/s) |
42.2 ± 5.2 |
39.5 ± 12.0 |
≥ 43 |
66 |
| Tibial nerve (=31) |
|
|
|
|
| Motor CV (m/s) |
42.1 ± 4.0 |
40.7 ± 5.9 |
≥ 41 |
39 |
| DML (ms) |
5.4 ± 1.1 |
4.5 ± 0.9 |
≤ 6.0 |
23 |
| TLI |
0.32 ± 0.05 |
0.39 ± 0.03 |
≥ 0.25 |
6 |
| Motor amplitude A1 (mV) |
8.1 ± 3.0 |
4.2 ± 0.4 |
≥ 6.6 |
32 |
| Motor amplitude A2 (mV) |
6.8 ± 2.2 |
3.8 ± 0.9 |
≥ 5.4 |
32 |
| Conduction block |
|
|
|
32 |
| F-wave latency (ms) |
56.3 ± 7.3 |
60.7 ± 19.6 |
≤ 59.2 |
35 |
| F-wave velocity (m/s) |
41.1 ± 3.7 |
40.7 ± 11.0 |
≥ 42 |
58 |
| Abbreviations: HNPP: Hereditary Neuropathy with liability to Pressure Palsies; SD: Standard Deviation; CV: Conduction Velocity; DML: Distal Motor Latency; TLI: Terminal Latency Index; m/s: meter/seconde; ms: milliseconde; mV: millivolt; µV: microvolt. |
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