Table 6.
Univariate Cox regression analysis of survival in oldest-old ALS patients.
| Variable | HR (95% CI) | p Value |
|---|---|---|
| Gender | ||
| Female sex | 1 | |
| Male sex | 0.99 (0.75–1.31) | 0.956 |
| Site of onset | 1.05 (0.95–1.15) | 0.330 |
| Bulbar | ||
| Spinal UL | ||
| Spinal LL | ||
| Respiratory | ||
| Phenotype | 1.04 (0.98–1.10) | 0.231 |
| Bulbar | ||
| Classic | ||
| Flail Arm | ||
| Flail Leg | ||
| UMN-p | ||
| Respiratory | ||
| FTD | 1.44 (0.89–2.34) | 0.136 |
| Genetic analysis (C9ORF72 expansion vs. other mutations) | 0.73 (0.40–1.32) | 0.296 |
| Weight loss at diagnosis, kg | 1.54 (0.98–2.43) | 0.060 |
| BMI at diagnosis, kg/m2 | 0.92 (0.87–0.97) | 0.005 |
| ALSFRS-R score at diagnosis, points | 0.98 (0.96–0.99) | 0.010 |
| Progression rate at diagnosis, points/months | 1.53 (1.38–1.70) | <0.001 |
| Age at onset, years | 1.04 (1.01–1.07) | 0.007 |
| Diagnostic delay, months | 0.95 (0.94–0.97) | <0.001 |
| FVC value at diagnosis, % | 0.99 (0.98–1.00) | 0.258 |
| Use of Riluzole | 0.81 (0.58–1.14) | 0.239 |
| Family history of ALS/FTD | 0.95 (0.58–1.56) | 0.849 |
| Hypertension | 0.87 (0.63–1.21) | 0.425 |
| COPD | 1.20 (0.75–1.92) | 0.451 |
| Other respiratory diseases | 0.70 (0.31–1.60) | 0.402 |
| Heart diseases | 1.44 (1.02–2.03) | 0.036 |
| Autoimmune disorders | 0.98 (0.57–1.70) | 0.950 |
| Diabetes | 0.76 (0.46–1.27) | 0.293 |
| Thyroid diseases | 0.89 (0.51–1.54) | 0.678 |
| Psychiatric disorders | 1.84 (0.90–3.79) | 0.093 |
| Hematological disorders | 0.53 (0.26–1.09) | 0.083 |
| Neoplastic disease | 1.11 (0.76–1.64) | 0.588 |
| Urologic diseases | 0.83 (0.52–1.32) | 0.430 |
| Gastrointestinal diseases | 0.94 (0.61–1.46) | 0.793 |
| Metabolic disorders | 0.97 (0.59–1.58) | 0.891 |
Spinal UL: spinal upper limb; spinal LL: spinal lower limb; UMN-p: upper motor neuron predominant; FTD: frontotemporal dementia. BMI: body mass index; ALSFRS-R: ALS Functional Rating Scale-Revised; progression rate at diagnosis was calculated as the monthly decline in ALSFRS-R scores, assuming a total score of 48 at onset; FVC: forced vital capacity; family history of ALS/FTD: family history of amyotrophic lateral sclerosis/frontotemporal dementia; COPD: chronic obstructive pulmonary disease; HR: hazard ratio; CI: confidence interval.