Table 1.
Comparison of phenotypic characteristics between long survivors and incident cohort of people with motor neuron disease
| Phenotypic characteristics | Incident cohort (n = 437) | Long survivors (n = 54) | Statistical test p-value |
|---|---|---|---|
| Sex: Male (%) | 275 (62.9) | 36 (66.6) | Fisher's p = 0.66 |
| Ethnicity: White Scottish/British/Other (%) | 415 (98.6) | 51 (100) | Fisher's p = 0.76 |
| Mean age of onset, years (SD) | 63.9 (10.9) | 47.4 (13.6) | t-test p < 0.0001 |
| Mean age of diagnosis, years (SD) | 65.5 (10.7) | 51.5 (13.5) | t-test p < 0.0001 |
| Median time to diagnosis, months (IQR) | 12.0 (8.0–23.0) | 26.0 (13.0–45.0) | Wilcoxon p < 0.0001 |
| Site of Onset | Fisher's p = 0.038 | ||
| Bulbar (%) | 123 (28.5) | 14 (26.9) | |
| Upper limb (%) | 103 (23.8) | 8 (15.4) | |
| Lower limb (%) | 144 (33.3) | 29 (55.8) | |
| Mixed (%) | 41 (9.5) | 1 (1.9) | |
| Cognition (%) | 12 (2.8) | 0 (0) | |
| Respiratory (%) | 5 (1.2) | 0 (0) | |
| Other (%) | 4 (0.9) | 0 (0) | |
| Classification | Fisher's p < 0.0001 | ||
| ALS (%) | 338 (77.4) | 29 (54.7) | |
| PBP (%) | 25 (5.7) | 1 (1.9) | |
| MND-FTD (%) | 25 (5.7) | 0 (0) | |
| PLS (%) | 19 (4.3) | 22 (41.5) | |
| PMA (%) | 19 (4.3) | 0 (0) | |
| Other (%) | 11 (2.5) | 1 (1.9) | |
| Family history of MND: Yes (%) | 39 (9.2) | 6 (11.5) | Fisher's p = 0.66 |
| Taking Riluzole: Yes (%) | 173 (40.0) | 32 (62.7) | Fisher's p = 0.0005 |
| Feeding Tube Inserted: Yes (%) | 136 (31.3) | 6 (11.8) | Fisher's p = 0.0003 |
| Non-invasive/Invasive ventilation commenced: Yes (%) | 118 (27.2) | 13 (25.0) | Fisher's p = 0.15 |
ALS amyotrophic lateral sclerosis, PBP progressive bulbar palsy, MND-FTD motor neuron disease with frontotemporal dementia, PLS primary lateral sclerosis, PMA progressive muscular atrophy
Significant p-values in bold