Sex | Age (years) at | R/L | Motor nerves | Sensory nerves | EMG chronic denervation | ||||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Onset | Exami-nation | Median | Ulnar | Peroneal | Tibial | Median | Ulnar | Sural | |||||||||||
CMAP | CV | CMAP | CV | CMAP | CV | CMAP | CV | SNAP | CV | SNAP | CV | SNAP | CV | ||||||
Normal values → | 4.0 | 49.0 | 4.0 | 49.0 | 3.0 | 41.0 | 3.0 | 41.0 | 12.0 | 46.0 | 17.0 | 47.0 | 17.0 | 44.0 | |||||
Family 1 | |||||||||||||||||||
II-2 | M | 18 | 57 | R | - | - | - | - | A | A | 0.4 | A | - | - | - | - | A | A | Present |
L | 1.6 | 37.5 | 4.9 | 50.2 | A | A | 0.9 | A | 1.9 | 50.0 | 1.2 | 45.8 | 1.8 | 38.1 | Present | ||||
59 | L | 0.6 | 40.2 | 5.6 | 47.4 | A | A | A | A | - | - | - | - | - | - | Present | |||
61 | L | 1.0 | 38.1 | 5.8 | 51.1 | A | A | A | A | - | - | - | - | - | - | - | |||
63 | L | 1.0 | 35.8 | 6.8 | 51.1 | A | A | A | A | - | - | - | - | - | - | Present | |||
64 | L | 0.4 | 50.0 | 5.9 | 44.1 | A | A | A | A | - | - | - | - | - | - | Present | |||
65 | L | 0.6 | 43.8 | 3.7 | 45.3 | - | - | - | - | - | - | - | - | - | - | Present | |||
66 | R | 0.2 | 46.2 | 6.3 | 41.7 | - | - | - | - | - | - | - | - | - | - | - | |||
L | 0.3 | 40.7 | 6.7 | 46.1 | - | - | - | - | - | - | - | - | - | - | - | ||||
II-3 | F | 7 | 58 | R | - | - | - | - | 0.3 | 24.1 | 0.2 | A | - | - | - | - | 3 | 36.1 | Present |
L | - | - | - | - | 1.5 | 28.3 | 0.2 | A | - | - | - | - | 6 | 38.1 | - | ||||
63 | R | - | - | - | - | A | A | 0.2 | 29.6 | - | - | - | - | 3.3 | 46.2 | Present | |||
L | - | - | - | - | 0.1 | 37.1 | 0.3 | 39.8 | - | - | - | - | 2.9 | 50.0 | Present | ||||
67 | R | 5.0 | 41.7 | 6.5 | 52.3 | A | A | 0.2 | A | 2.7 | - | 1.0 | - | 2.6 | 43.1 | ||||
L | 0.8 | 39.0 | 4.8 | 45.8 | A | A | 0.6 | A | 2.5 | - | 1.7 | - | 2.1 | 52.0 | Present | ||||
II-7 | M | 45 | 60 | R | 7.8 | 47.2 | - | - | A | A | 5.4 | A | 17 | 44.2 | - | - | - | - | Present |
L | - | - | - | - | A | A | A | A | - | - | - | - | A | A | Present | ||||
III-1 | M | 35 | 40 | R | N | ↓ | N | ↓ | A | A | A | A | N | N | - | - | ↓ | ↓ | Present |
L | - | - | - | - | A | A | A | A | - | - | - | - | ↓ | ↓ | - | ||||
III-2 | M | 25 | 37 | R | N | 40.0 | N | N | ↓ | 25.0 | ↓ | 31.0 | N | N | - | - | 3.8 | 37.0 | - |
L | - | - | - | - | ↓ | 29.0 | ↓ | 31.0 | - | - | - | - | - | - | Present | ||||
III-5 | F | 32 | 32 | R | 3.8 | 45.5 | - | - | 1 | 34.6 | 2.5 | 34.2 | 8.5 | 50 | 5.6 | 51.4 | 3.8 | 45.5 | Present |
L | - | - | - | - | 0.4 | 33.7 | 4.9 | 32.6 | 2.4 | 49.1 | - | ||||||||
42 | R | 3.6 | 41.1 | 4.8 | 46.1 | 1.5 | 34.5 | 3.7 | 36.4 | 2.6 | 46.8 | 2.4 | 58.1 | 7.3 | 51.7 | Present | |||
Family 2 | |||||||||||||||||||
II-1 | M | 22 | 38 | R | - | - | - | - | ↓ | - | - | - | - | - | - | - | ↓ | - | - |
L | - | - | - | - | - | - | - | - | - | - | - | - | ↓ | - | - | ||||
46 | R | ↓ | ↓ | ↓ | ↓ | - | - | - | - | A | A | ↓ | ↓ | - | - | - | |||
L | ↓ | N | ↓ | ↓ | - | - | - | - | ↓ | - | ↓ | ↓ | - | - | - | ||||
II-2 | M | - | 67 | R | 2.2 | 34.3 | 9.2 | 49.5 | A | A | A | A | 5.3 | 36.4 | 6.6 | 40.8 | A | A | Present |
L | - | - | - | - | A | A | A | A | A | A | Present | ||||||||
69 | R | 0.4 | 33.0 | - | - | - | - | - | - | A | A | – | - | - | - | ||||
L | 1.3 | 31.0 | 3.4 | - | - | - | - | - | A | A | 1.9 | 41.6 | - | - | - | ||||
III-2 | F | 42 | 44 | R | 8.0 | 52.2 | 7.9 | - | 2.5 | 33.3 | 5.2 | 35.5 | 70.0 | - | 60.0 | - | 6.2 | 40.4 | Normala |
L | 7.5 | 50.0 | 10.6 | - | 0.9 | 31.6 | 3.0 | - | 57.0 | - | 64.0 | - | 6.1 | 50.0 | Normala | ||||
IV-1 | M | 11 | 12 | R | 6.4 | 44.2 | 8.2 | - | 1.7 | 35.4 | 3.9 | 39.2 | 2.4 | - | 3.9 | - | 2.8 | 45.4 | Normal |
L | - | - | - | - | 1.3 | 32.9 | - | - | - | - | - | - | 3.0 | 45.9 | Normal |