Demographic and clinical characteristics at each site of onset
| Upper limbs | Lower limbs | Bulbar | Combined | Total | |
| 50 | 52 | 32 | 16 | 150 | |
| Age (years) | 61.4±9.1 | 60.5±11.1 | 63.1±6.53 | 62.1±7.83 | 61.6±9.24 |
| Sex (M/F) | 32/18 | 30/22 | 15/17 | 8/8 | 85/65 |
| FS-FE (months) | 15.5±11.8 | 15.9±9.42 | 12.2±6.47 | 9.36±5.58* | 14.3±9.71 |
| FRS-D | 39.7±4.48 | 38.8±3.74 | 39.4±5.55 | 38.2±7.17 | 39.1±4.48 |
| FS-SS (months) | 15.2±13.4 | 13.6±12.0 | 10.8±8.32 | 0.35±0.50 | 12.8±12.1 |
| MST (months) | 33 | 32 | 26 | 18** | 32 |
| 5 year (%) | 18 | 21 | 16 | 0 | 17 |
| PS (%) | 26 (52) | 24 (46) | 4 (13) | 5 (31) | 59 (36) |
| U | 0 | 3 | 1 | 0 | 4 |
| L | 9 | 0 | 3 | 3 | 15 |
| B | 17 | 21 | 0 | 2 | 40 |
U, L, B indicate numbers of patients in whom each function was normally preserved at the onset of respiratory symptoms.
Combined (C) type patients were diagnosed significantly earlier compared with those with limb onset (*C vs L, p<0.001; C vs U, p<0.001) and MST was shortest for C-type as compared to limb onset (**p=001). Bulbar onset showed less frequent preservation (13%) of lower motor neuron function at the appearance of respiratory symptom as compared to those with U (52%) and L (46%). Age, age at onset; B, bulbar symptoms; FRS-D, Amyotrophic Lateral Sclerosis Functional Rating Scale revised score at diagnosis; FS-FE, interval between first symptoms and first examination; FS-SS, interval between first symptoms and second symptoms; L, lower limb symptoms; MST, median survival time; 5 year (%), percentage of patients surviving >5 years for each type of onset; PS (%), number (percentage) of normally preserved sites (not number of patients) at each site of onset; U, upper limb symptoms.