Table 3.
Demographical, clinical and analytical characteristics of ALS patients classified according to the predefined threshold of SNh area (0.22 cm2).
| SN− (n = 42) | SN+(n = 55) | |
|---|---|---|
| Age (years) | ||
| Mean (SD) | 57.35 (12.84) | 62.68 (11.48) |
| Median (IQR) | 57.45 (47.65, 68.2) | 62.97 (56.54, 71.55) |
| Gender (male) | ||
| n (%) | 20 (62.5%) | 41 (74.5%) |
| Body mass index, n = 102 | ||
| Mean (SD) | 27.22 (4.49) | 27.18 (4.3) |
| Median (IQR) | 26.53 (24.54, 29.76) | 26.53 (23.98, 29.76) |
| Family history | 7 (16.7%) | 18 (32.7%) |
| Dementia n (%) | 1 (3.1%) | 8 (14.5%) |
| PD n (%) | 1 (3.1%) | 2 (3.6%) |
| Dementia and PD n (%) | 2 (6.2%) | 0 (0%) |
| ALS or FTD n (%) | 3 (9.4%) | 8 (14.5%) |
| Region of onset | ||
| Bulbar n (%) | 6 (18.8%) | 15 (27.3%) |
| Spinal n (%) | 26 (81.2%) | 40 (72.7%) |
| Parkinsonism | ||
| n (%) | 1 (2.4%) | 3 (5.5%) |
| Time from onset (months) | ||
| Mean (SD) | 36.45 (46.83) | 32.58 (32.2) |
| Median (IQR) | 15.58 (8.43, 55.51) | 21.6 (13.62, 42.77) |
| ALSFRS-R | ||
| Mean (SD) | 34.06 (8.55) | 35.41 (7.53) |
| Median (IQR) | 34.5 (31, 40.5) | 36.5 (31.25, 41.75) |
| UMN score | ||
| Mean (SD) | 5.78 (5.94) | 4.75 (5.11) |
| Median (IQR) | 3.5 (0, 12.25) | 2.5 (1, 9) |
| EB impairment (n = 80) | ||
| No | 21 (72.4%) | 30 (58.8%) |
| Mild | 8 (27.6%) | 19 (37.3%) |
| FTD | 0 (0%) | 2 (3.9%) |
| Familial or genetic ALS, n (%) | 3 (9.37%) | 9 (16.36%) |
| C9ORF72, n (%) | 1 (3.12%) | 6 (10.91%) |
| SOD1, n (%) | 1 (3.12%) | 3 (5.45%) |
| Unknown, n (%) | 1 (3.12%) | 0 (0%) |
| Ferritin ug/L | ||
| Mean (SD) | 195.31 (155.14) | 192.31 (128.31) |
| Median (IQR) | 154 (68, 311) | 155 (105.25, 240.25) |
ALS: amyotrophic lateral sclerosis; BMI: body mass index; EB: executive or behavioural; FTD: frontotemporal dementia; PD: Parkinson’s disease; SNh: hyperechogenicity of the substantia nigra; SN+: area of hyperechogenicity of one substantia nigra > 0.22 cm2; SN−: area of hyperechogenicity of both substantia nigra ≤0.22 cm2; UMN: upper motor neuron.