Table 1.
Clinical and demographic characteristics of the recruited sample.
| Group | ALS | TDP43+ | CTRL | AD | FTLD | MN-m |
|---|---|---|---|---|---|---|
| N | 66 | 4 | 40 | 20 | 15 | 20 |
| M (%) | 45 (68.1%) | 1 (25%) | 25 (62.5%) | 9 (45%) | 10 (66.7%) | 9 (45%) |
| Age, years |
63.6 ± 10.1 (37–79) |
57.0 ± 8.8 (50–70) |
64.5 ± 11.2 (39–82) |
74.7 ± 3.3 (67–80) |
65.6 ± 7.3 (56–77) |
72.3 ± 13.8 (51–88) |
| Onset, Spinal/Bulbar | 53/13 | 4/0 | – | – | 15 bvFTD | – |
| Disease duration, months since estimated onset |
24.5 ± 15.1 (5–62) |
42.3 ± 39.9 (9–97) |
– |
34.8 ± 22.3 (12–84) |
31.9 ± 18.1 (6–60) |
33.9 ± 15.9 (10–60) |
| Functional scale |
ALSFRS-R 32.3 ± 8.8 (8–45) |
ALSFRS-R 33.2 ± 1.8 (32–36) |
– |
MMSE 19.2 ± 5.9 (6–25) |
FTLD_modified CDR SoB 13.0 ± 4.7 (3.5–16.0) |
na |
| DPIa |
0.82 ± 0.65 (0.10–2.69) |
0.69 ± 0.54 (0.16–1.32) |
– |
0.36 ± 0.20 (0.13–0.80) |
na | na |
| BMI |
23.1 ± 3.1 (17.2–31.5) |
26.3 ± 4.8 (20.2–31.9) |
na | na | na | na |
|
Riluzole, yes (%) 50 mg b.i.d |
54 (81%) | 3 (75%) | – | 0 | 0 | 0 |
| EN, yes (%) | 5 (7.5%) | 0 | – | 0 | 0 | 0 |
| NIV, yes (%) | 9 (13.6%) | 1 (25%) | – | 0 | 0 | 0 |
| Dementia, yes (%) | 2 (3%) | 1 (25%) | 0 | 20 (100%) | 15 (100%) | 0 |
AD Alzheimer’s disease, ALSFRS-R ALS Functional Rating Scale-revised version, BMI body mass index, CTRL controls, DPI disease progression index, EN enteral nutrition, MMSE Mini-Mental State Examination, na not available, NIV non-invasive ventilation, TDP43 + ALS patients carrying a pathogenic TARDBP mutation. Data are shown as mean ± SD.
aThe DPI was calculated as [48-ALSFRS-R score]/disease duration (months) for ALS patients and [30-MMSE score]/disease duration (months) for AD patients.