Table 2.
Skin innervation, neurophysiological, and psychophysiological data of patients with hereditary transthyretin amyloidosis (ATTRv).
| Large fiber nerves | n = 38 Value (Abnormal rate) |
|---|---|
| Nerve conduction study | |
| Median nerve | |
| Distal motor latency (ms) | 6.4 ± 1.9 (91.9%) |
| Distal CMAP (mV) | 2.2 ± 2.0 (89.2%) |
| Motor NCV (m/s) | 44.7 ± 6.9 (81.1%) |
| Distal SNAP (μV) | 2.9 ± 7.5 (91.9%) |
| Sensory NCV (m/s) | 44.0 ± 10.3 (94.7%) |
| Ulnar nerve | |
| Distal motor latency (ms) | 3.8 ± 0.9 (55.3%) |
| Distal CMAP (mV) | 4.5 ± 3.0 (65.8%) |
| Motor NCV (m/s) | 49.4 ± 6.4 (47.4%) |
| Distal SNAP (μV) | 5.6 ± 7.3 (86.8%) |
| Sensory NCV (m/s) | 50.7 ± 6.8 (57.9%) |
| Peroneal nerve | |
| Distal motor latency (ms) | 5.0 ± 1.4 (52.6%) |
| Distal CMAP (mV) | 1.1 ± 1.8 (79.0%) |
| Motor NCV (m/s) | 39.2 ± 5.7 (76.3%) |
| Tibial nerve | |
| Distal motor latency (ms) | 4.6 ± 1.5 (37.8%) |
| Distal CMAP (mV) | 2.1 ± 4.2 (89.2%) |
| Motor NCV (m/s) | 37.6 ± 6.7 (75.7%) |
| Sural nerve | |
| Distal SNAP (μV) | 1.4 ± 3.2 (84.2%) |
| Sensory NCV (m/s) | 44.9 ± 8.2 (84.2%) |
| Quantitative sensory testing | |
| Vibratory at index finger | 20.8 ± 38.8 (57.9%) |
| Vibratory at ankle | 62.8 ± 51.6 (86.8%) |
| Small fiber nerves | n = 38 Value (Abnormal rate) |
|---|---|
| Skin biopsy | |
| Intraepidermal nerve fiber density (fibers/mm) | 1.75 ± 1.98 (81.6%) |
| Quantitative sensory testing | |
| Warm at thenar (°C) | 38.3 ± 6.2 (68.4%) |
| Cold at thenar (°C) | 24.2 ± 11.0 (65.8%) |
| Warm at foot dorsum (°C) | 44.3 ± 4.8 (71.1%) |
| Cold at foot dorsum (°C) | 19.9 ± 11.9 (47.4%) |
CMAP, compound muscle action potential; NCV, nerve conduction velocity; n, number of patients; SNAP, sensory nerve action potential.