Table 3 Nerve conduction studies in genetically defined subtypes of HSP
Genotype | Tibial nerve | Ulnar nerve | Sural nerve | Radial nerve | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
DML (in ms) | CMAP (in mV) | MNCV (in m/s) | FWL (in ms) | DML (in ms) | CMAP (in mV) | MNCV (in m/s) | FWL (in ms) | SNAP (in μV) | SNCV (in m/s) | SNAP (in μV) | SNCV (in m/s) | |
SPG3 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 |
3.6 | 14.6 | 52 | 48.8 | 2.2 | 10.1 | 61 | 28 | 24.0 | 57 | 20.3 | 57 | |
SPG4 | 6/35 | 0/35 | 2/35 | 1/35 | 1/34 | 0/34 | 1/34 | 1/34 | 4/35 | 6/35 | 5/29 | 2/29 |
4.3±0.8 | 18.7±6.8 | 47.2±5.7 | 51.7±4.9 | 2.4±0.4 | 15.9±3.2 | 60.8±4.5 | 27.6±2.3 | 11.1±7.8 | 47.7±7.0 | 22.2±7.0 | 62.0±5.0 | |
3.0-6.5 | 7.3-35.0 | 36-69 | 43.0-66.3 | 1.7-3.2 | 6.8-22.7 | 50-69 | 23.2-33.7 | 0–37.7 | 34-64 | 10.9-35.2 | 50-73 | |
SPG5 | 0/3 | 0/3 | 0/3 | 0/3 | 1/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 |
3.8±0.9 | 26.6±11.0 | 46.0±5.0 | 48.7±6.5 | 2.8±0.6 | 15.5±4.4 | 66.7±3.2 | 24.3±2.7 | 28.7±13.1 | 46.7±8.1 | 32.9±2.6 | 64.3±4.2 | |
2.9-4.7 | 17.3-38.7 | 41-51 | 41.2-53.0 | 2.1-3.3 | 12.8-20.5 | 63-69 | 21.6-27.0 | 14.6-40.5 | 41-56 | 30.6-35.7 | 61-69 | |
SPG7 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 | 0/3 |
4.0±0.4 | 22.1±9.1 | 46.7±3.5 | 47.5±5.7 | 2.2±0.3 | 15.1±4.0 | 65.7±1.5 | 28.8±0.3 | 17.5±4.9 | 48.7±2.3 | 24.0±2.4 | 63.7±2.5 | |
3.7-4.5 | 11.7-28.3 | 43-50 | 41.2-52.4 | 1.8-2.4 | 12.3-19.7 | 64-67 | 28.4-29.0 | 12.9-22.6 | 46-50 | 21.9-26.6 | 61-66 | |
SPG8 | 0/1 | 0/1 | 1/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 |
3.2 | 10.2 | 37 | 54.3 | 2.4 | 16.1 | 59 | 28.3 | 26.0 | 43 | 19.7 | 59 | |
SPG10 | 0/1 | 0/1 | 1/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 | 0/1 |
4.2 | 22.1 | 40 | 51.4 | 2.5 | 11.4 | 51 | 27.2 | 5.9 | 52 | 23.1 | 61 | |
SPG11 | 1/6 | 1/6 | 3/6 | 1/5 | 1/6 | 2/6 | 0/6 | 1/5 | 1/6 | 1/6 | 2/5 | 3/5 |
4.7±2.1 | 18.6±12.2 | 41.7±3.6 | 51.9±7.6 | 2.9±0.7 | 14.0±7.1 | 61.5±4.3 | 27.6±2.0 | 12.1±10.9 | 47.0±5.8 | 19.2±5.6 | 55.0±4.4 | |
3.6-8.8 | 0.9-36.1 | 37-46 | 45.1-64.8 | 2.1-4.3 | 1.1-21.1 | 58-70 | 24.6-29.0 | 1.6-31.6 | 38-56 | 12.5-24.5 | 48-60 | |
SPG15 | 0/4 | 0/4 | 3/4 | 0/4 | 0/4 | 0/4 | 0/4 | 2/4 | 1/4 | 1/4 | 1/3 | 1/3 |
4.3±1.0 | 8.8±3.8 | 38.3±5.7 | 56.9±5.4 | 2.7±0.2 | 14.2±2.3 | 55.3±4.6 | 30.3±2.8 | 7.1±3.7 | 47.5±13.9 | 18.6±6.0 | 56.3±2.9 | |
2.8-5.0 | 5.8-14.3 | 30-43 | 49.0-61.0 | 2.4-2.8 | 11.6-16.4 | 51-61 | 26.1-32.2 | 2.9-11.9 | 31-65 | 11.7-22.4 | 53-58 | |
Threshold value | < 5.1 | > 5.0 | > 40 | < 63.6 | < 3.2 | > 4.0 | > 50 | < 31.0 | > 3.8 | > 39 | > 16.0 | > 55 |