Number of patients | 14 | 6 | 3 |
Men/women | 11/3 | 3/3 | 0/3 |
Mean age at onset, years (range) | 38.2 (21–62) | 38.8 (24–53) | 47.3 (39–57) |
Mean age at first visit, years (range) | 44.1 (26–73) | 47.8 (36–60) | 67.7 (64–73) |
Mean disease duration at first visit, years (range) | 5.9* (0.3–19)† | 9* (1–25)† | 20.3 (16–25) |
Type of progression (chronic progressive/stepwise) | 3/11 | 2/4 | 1/2 |
Limb weakness distribution | | | |
UL>/=/<LL | 9/3/2 | 3/2/1 | 3/0/0 |
Asymmetric/symmetric | 13/1 | 6/0 | 3/0 |
Mean scores | | | |
Rankin score | 2.2 | 2.2 | 2.7 |
UL/LL impairment score | 2.4/1.3 | 2.3/1.8 | 2.7/0 |
UL/LL MRC sumscore | 75.8/88.3 | 79.5/83.3 | 57.3/96.3 |
UL motor nerves clinically affected | | | |
Total | 56 | 17 | 18 |
Ulnar | 25 | 8 | 6 |
Median | 16 | 4 | 5 |
Radial | 14 | 5 | 5 |
Musculocutaneous | 1 | – | 2 |
LL motor nerves clinically affected | | | |
Total | 17 | 11 | – |
Peroneal | 10 | 7 | – |
Tibial | 3 | 4 | – |
Femoral | 4 | – | – |
Mean number of affected nerves/patient (range) | 5.2 (2–8) | 4.7 (3–6) | 6 (4–7) |
Number of patients with mild sensory impairment | 1 (7%) | 1 (17%) | 0 (0%) |