Table III. Studies of prognosis in ALS: studies based on prospective epidemiological series (register methodology).
| Reference | Cases# | Population- based | P/R | Mean age at onset (years) | Median survival from onset (months) | % 5-year survival from onset | Significant prognostic factors | Comments | Drop-out rate |
|---|---|---|---|---|---|---|---|---|---|
| [23] | 229 | Y (Scotland) | P | 64.3 (sALS) 55.6 (fALS) | 30 | 28 | PMA (better); PBP (worse); Age (<65 vs. > 65); Gender (women worse) | 0 | |
| [24] | 388 | Y (Ireland) | P | 63.3(M) § 64.4(W) § | NA | 17§ | No effect of El Escorial criteria on ALS survival | 0 | |
| [25] | 193 | Y (Piemonte, Italy) | P | 62.8 | 30.5 | 24.7 | COX: FVC (progression rate), lower limbs (progression rate), PEG, age, bulbar (progression rate), definite ALS (EEC), riluzole | 0 | |
| [26] | 180 | Y (Washington State, U.S.) | P | 61.3§ | 32 | 7§ | Gender (women worse), age, bulbar, ALS-SS, residence, no partner, BMI change, scarce physical activity (worse) COX: age, bulbar, SF-36 (physical function), time from onset to diagnosis | No information on PEG, NIPPV | 0 |
| [30] | 1226 | Y (Scotland) | P | 65.2 (M) 67.6 (W) | 25 | 11 | COX: Date of diagnosis (<1993 better than ≥1994); bulbar (worse); UMN + LMN worse than pure LMN Positive factors: riluzole, longer time onset-diagnosis, to be followed by a neurologist | n.s. gender, fALS not included PEG, NIV Cox final model on 1048 patients | 0 |
| [31] | 1226 | Y (Scotland) | P | NS | 20.3 (≥80 yrs) 25.1 (<80 yrs) | NA | Age strong predictor, independent from type of onset | Comparison of patients with age ≥80 vs. <80. | 0 |
| [27] | 79 | Y (Lombardia, Italy) | P | 64.4 | 39.2 | 43 (4 year survival) | Age, bulbar, duration of symptoms at diagnosis COX: age, definite ALS (EEC) | Large proportion of suspected ALS (mostly PMA) | 6% |
Note: Only studies using Kaplan-Meier survival curves are included.
§
from diagnosis.
*
incomplete ascertainment of cases.
Abbrevations: ALS: amyotrophic lateral sclerosis; COX: Cox multivariate model; EEC: El Escorial diagnostic criteria; FALS: familial amyotrophic lateral sclerosis; FTLD: fronto-temporal lobe dementia; LMN: lower motor neuron; M: men; N: no; NA: not available; NIPPV: non-invasive positive pressure ventilation; NS: not stated; P: prospective; PBP: progressive bulbar palsy; PEG: percutaneous endoscopic gastrostomy; PMA: progressive muscular atrophy; R: retrospective; SALS: sporadic ALS; UMN: upper motor neuron; W: women; Y: yes.