Table 1.
Electrophysiological studies
| |
Index |
Sister |
Mother |
|||
|---|---|---|---|---|---|---|
| Nerve | CMAP/mV | mNCV/ ms -1 | CMAP/mV | mNCV/ ms -1 | CMAP/mV | mNCV/ ms -1 |
| N. ulnaris [4.0 mV / 50 ms-1] |
23 |
68 |
19 |
61 |
15 |
57 |
| N. tibialis [5.0 mV / 40 ms-1] |
11 |
51 |
24 |
51 |
6.4 |
40 |
| N. peronaeus [4.0 mV / 41 ms-1] |
9.9 |
47 |
n.d. |
n.d. |
n.d. |
n.d. |
| |
Index |
Sister |
Mother |
|||
|
Nerve |
SNAP/
μ
V |
sNCV/ ms
-1
|
SNAP/
μ
V |
sNCV/ ms
-1
|
SNAP/
μ
V |
sNCV/ ms
-1
|
| N. radialis [16 μV / 55 ms-1] |
8.8 |
67 |
15 |
55 |
6.1 |
58 |
| N. suralis [3.8 μV / 39 ms-1] |
1.6 |
50 |
2.7 |
43 |
Ø |
n.a. |
| |
Index |
Sister |
Mother |
|||
|
Muscle |
ASA |
MUAP/mV |
ASA |
MUAP/mV |
ASA |
MUAP/mV |
| M. deltoideus [1.5 mV] |
Ø |
2.0 |
Ø |
2.7 |
Ø |
6.4 |
| M. IOD1 [2.3 mV] |
Ø |
7.5 |
n.d. |
n.d. |
Ø |
12 |
| M. vastus medialis [2.0 mV] |
Ø |
16 |
Ø |
28 |
Ø |
28 |
| M. tibialis anterior [1.6 mV] | Ø | 4.3 | n.d. | n.d. | n.d. | n.d. |
Motor nerve conduction studies, sensory nerve conduction studies and needle EMG recorded from the index patient (index), his sister and his mother. CMAP - compound motor action potential; mNCV - motor nerve conduction velocity; SNAP - sensory nerve action potential; sNCV - sensory nerve conduction velocity; M. IOD1 - musculus interosseus dorsalis 1; ASA - abnormal spontaneous activity (fibrillation potentials or positive sharp waves); Ø - not present; n.d. - not determined; n.a. - not applicable. Normal ranges for CMAP, SNAP and MUAP amplitudes as well as NCVs are indicated in brackets in column 1. Pathological results are highlighted bold.