Table 2 Electrophysiological recordings
From: Peripheral myelin protein 2 – a novel cluster of mutations causing Charcot-Marie-Tooth neuropathy
FAMILY A | FAMILY B | |||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
A.II-2 | A.III-1 (proband) | B.II-1 | B.II-2 (proband) | |||||||||||||||||||||
Age at evaluation | 34 years of age | 20 years of age | 30 years of age | 35 years of age | 37 years of age | 46 years of age | 37 years of age | |||||||||||||||||
Sensory | CV (m/s) | A (μV) | DL (ms) | CV (m/s) | A (μV) | DL (ms) | CV (m/s) | A (μV) | DL (ms) | CV (m/s) | A (μV) | DL (ms) | CV (m/s) | A (μV) | DL (ms) | CV (m/s) | A (μV) | DL (ms) | CV (m/s) | A (μV) | DL (ms) | |||
Median | NR | NR | NR | NE | NE | NE | NR | NR | NR | NR | NR | NR | NR | NR | NR | 54.9 | 5.9 | NR | 53.1 | 6.2 | NR | |||
Ulnar | NR | NR | NR | NE | NE | NE | NR | NR | NR | NR | NR | NR | NR | NR | NR | 53.1 | 2.4 | NR | 56.3 | 2.4 | NR | |||
Sural | NR | NR | NR | NE | NE | NE | NR | NR | NR | NR | NR | NR | NR | NR | NR | 43.3 | 6 | NR | 39.9 | 6.4 | NR | |||
Motor | CV (m/s) | A (mV) | DL (ms) | FW-L (ms) | CV (m/s) | A (mV) | DL (ms) | CV (m/s) | A (mV) | DL (ms) | CV (m/s) | A (mV) | DL (ms) | CV (m/s) | A (mV) | DL (ms) | CV (m/s) | A (mV) | DL (ms) | FW-L (ms) | CV (m/s) | A (mV) | DL (ms) | FW-L (ms) |
Median | 12 | 3.1 | 5.13 | NE | NE | NE | NE | 8.9 | 8.3 | 6.4 | 8 | 4.1 | 6.13 | 6.5 | 4.3 | 5.6 | 49.3 | 16.5 | 3.7 | 33.8 | 39.6 | 14 | 3.5 | 47.7 |
Ulnar | 14.7 | 2.9 | 5.8 | NE | 21.9 | 12.5 | 8.8 | 11.1 | 6.2 | 7.9 | 11.7 | 2.7 | 5.9 | 12.5 | 2.7 | 6.4 | 56.3 | 10.1 | 2.6 | 35.4 | 45.3 | 10.3 | 3.3 | 43 |
Peroneal | NR | NR | NR | NE | NR | 4.3 | 14 | NR | NR | NR | NR | NR | NR | NR | NR | NR | 43.4 | 6.5 | 4.5 | NE | 30.6 | 0.7 | 4.1 | NE |
Tibial | NR | NR | NR | NE | NE | NE | NE | NR | NR | NR | NR | NR | NR | NR | NR | NR | 47.5 | 9.5 | 4.5 | 60.3 | 35.9 | 4 | 4.2 | 76 |