Age (years) |
60.8+/−16.4 (mean+/−SD) |
18–90 |
Gender (female) |
52% (n=33) |
|
El Escorial diagnostic classification |
|
|
Possible ALS |
11% (n=7) |
|
Probable ALS |
32% (n=20) |
|
Definite ALS |
57% (n=36) |
|
Duration of symptoms (years) |
3.6+/−3.1 (mean+/−SD) |
0.6–17 |
Bulbar onset |
28.6% (n=18) |
|
Family history of ALS |
15.9% (n=10) |
|
Forced vital capacity (% predicted) |
58+/−27 (mean+/−SD) |
8–111 |
ALS revised functional rating score, current |
29 (median) |
6–46 |
Worst bulbar function (FRS-R bulbar) |
9 (median) |
|
Rate of functional decline (points/yr) |
6.5 (median) |
0.3–43.5 |
Phonemic fluency (F-words), lowest ever (n=54) |
10 (median) |
4–23 |
Affective lability (CNS-LS), highest ever (n=60) |
10 (median) |
7–28 |