Demographic and clinical features of the ALS patients
| ALS patients | n | Gender F/M | Age (years) | Diagnostic delay* | Survival (months)† | % Patients alive at censoring‡ | ALSFRS-r§ slope | ALSFRS-r¶ | % Caucasian vs non-Caucasian |
|---|---|---|---|---|---|---|---|---|---|
| Total | 73 | 24/49 | 65.6±13.2 | 15.8±16.9 | 10.3±7.4 | 27.3 | 0.82±0.7 | 34.3±9.1 | 91.5/8.5 |
| Disease phase | |||||||||
| Early | 31 | 4/27 | 62.5±12.4 | 13.8±10.6 | 13.5±7.5 | 45.1 | 0.46±0.5 | 42.5±2.9 | 96.1/6.9 |
| Intermediate | 20 | 9/11 | 71.8±12.0 | 11.7±5.9 | 12.7±7.0 | 25 | 0.9±0.3 | 33.0±3.3 | 100/0 |
| Advanced | 22 | 11/11 | 66.4±14.1 | 19.9±25.2 | 5.5±3.8 | 13.6 | 1.2±0.9 | 23.0±4.0 | 86.3/13.6 |
| Site of disease onset | |||||||||
| Bulbar | 12 | 6/6 | 62.0±11.7 | 19.7±13.9 | 8.2±5.5 | 16.6 | 0.81±0.5 | 30.1±10.2 | 90.9/9.1 |
| Limb | 61 | 18/43 | 66.1±13.5 | 15.5±17.6 | 10.8±7.6 | 31.2 | 0.82±0.7 | 35.1±8.8 | 93.9/6.1 |
| Riluzole treatment | 53 | 22/31 | 66.1±12.5 | 15.0±18.2 | 8.3±6.4 | 23.2 | 0.9±0.7 | 32.7±9.3 | 86.2/13.8 |
| Healthy controls | 52 | 30/22 | 58.0±12.2 | NA | NA | 100 | NA | NA | 100/0 |
The characterisation of the clinical phases of ALS patients was obtained following the proposed staging criteria by Roche et al.2 early phase (symptoms onset, involvement of the first region and diagnosis—stage 1 and 2A); intermediate phase (involvement of a second and third region—stage 2B and 3); advanced phase (need for gastrostomy and for non-invasive ventilation—stage 4A and 4B). We also report the ALSFRS-r range of values for each subgroup. Data are presented as mean±SD.
*Time of ALS onset to diagnosis, expressed in months.
†Survival time from sampling to death (in months).
‡Percentage of patients alive at the time of censoring.
§Median ALSFRS-r monthly decline (slope) from sampling throughout the follow-up period.
¶Amyotrophic lateral sclerosis Functional Rating Scale Revised (ALSFRS-r) score at the time of sample collection.
NA, not applicable.