Table 1.
Subject characteristics
| Diagnostic group | All ALS (n=47) | ThS+ ALS (n=13) | ThS− ALS (n=34) | FTLD-TDP (n=22) |
|---|---|---|---|---|
| Gender, M:F (%:%) | 33:15 (70:30) | 9:4 (69:31) | 24:11 (71:29) | 12:9 (55:45) |
| Motor symptoms, n (%) | 47 (100) | 13 (100) | 34 (100) | 3 (14) |
| Cognitive deficits, n (%) | 4 (9) | 2 (15) | 2 (6) | 22 (100) |
| Age at onset, y (stdev) | 57 (12) | 56 (10) | 58 (12) | 60 (9) |
| Disease duration, y (stdev) | 3.1 (4.1) | 2.7 (1.8) | 3.3 (2.6) | 6.6 (4.4) |
| Family History1, n (%) | 13 (28) | 5 (38) | 8 (24) | 13 (59) |
| C9orf72 expansion, n (%) | 7 (15) | 3 (23) | 4 (12) | 4 (18) |
| Brain weight, g (stdev) | 1338 (138) | 1367 (129) | 1327 (151) | 1096 (194) |
| PMI, h (stdev) | 13.4 (7.4) | 12.5 (6.5) | 13.2 (6.7) | 12.0 (6.2) |
| TDP-43 pathology2 | mean (stdev) | |||
| Spinal cord | 2.3 (0.7) | 2.6 (0.5) | 2.1 (0.8) | 1.4 (1.1) |
| Motor Cortex | 1.6 (1.0) | 1.7 (1.0) | 1.6 (1.0) | 0.8 (0.9) |
| Hippocampus | 0.8 (0.8) | 0.7 (0.5) | 0.8 (0.9) | 2.3 (0.9) |
1
Confirmed family history with the same or another neurodegenerative disease (unknown for 9 subjects)
2
TDP-43 pathology is a mean of the semi-quantitative scores (0–3)