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Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model
Westeneng HJ, Debray TPA, Visser AE, van Eijk RPA, Rooney JPK, Calvo A, Martin S, McDermott CJ et al.. The Lancet. Neurology, 2018-03-26
Topic: Amyloid, tau and blood-test diagnosis
Eight candidate predictors entered the prediction model: bulbar versus non-bulbar onset (univariable hazard ratio [HR] 1·71, 95% CI 1·63-1·79), age at onset (1·03, 1·03-1·03), definite versus probable or possible ALS (1·47, 1·39-1·55), diagnostic delay (0·52, 0·51-0·53), forced vital capacity (HR 0·99, 0·99-0·99), progression rate (6·33, 5·92-6·76), frontotemporal dementia (1·34, 1·20-1·50), and presence of a C9orf72 repeat expansion (1·45, 1·31-1·61), all p Interpretation We have developed an externally validated model to predict survival without tracheostomy and non-invasive ventilation for more than 23 h per day in European patients with ALS.
Key findings
- Eight candidate predictors entered the prediction model: bulbar versus non-bulbar onset (univariable hazard ratio [HR] 1·71, 95% CI 1·63-1·79), age at onset (1·03, 1·03-1·03), definite versus probable or possible ALS (1·47, 1·39-1·55), diagnostic delay (0·52, 0·51-0·53), forced vital capacity (HR 0·99, 0·99-0·99), progression rate (6·33, 5·92-6·76), frontotemporal dementia (1·34, 1·20-1·50), and presence of a C9orf72 repeat expansion (1·45, 1·31-1·61), all p<0·0001.
Why it matters
This study developed and validated a model that predicts survival in European patients with ALS using eight factors such as age at onset and progression rate. It is about prognosis in ALS, not about treating, curing or preventing Alzheimer's disease.
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Study details
- Participants1,936
- Evidence strengthModerate
How this study scored 63/100
Published in a leading specialty journal. Scored zero for study design, effect reported and randomised / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 9 / 10 | Tier 2 — leading specialty journal (The Lancet. Neurology) |
| Journal's citation record | 3 / 4 | The Lancet Neurology: journal h-index 457 (OpenAlex, 2026-10-29) |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 3 / 5 | Rated “moderate” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 2 / 5 | 2018 — within 10 year(s) of the newest study here |
| Sample size | 3 / 4 | 1,936 participants |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 3 / 3 | Studied in people (participant count stated) |
| Randomised / blinded | 0 / 2 | No randomisation or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 6 / 7 | Cited 527 times (OpenAlex, 2026-10-10) |
| Citations per year | 5 / 5 | 52.7 citations a year over 10 year(s) since 2018 |
| Impact for its field and year | 5 / 6 | Cited 28.8× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 1 / 6 | Cited by 1 other studies in this library |
| Still being cited | 3 / 4 | Cited 183 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 3 / 4 | Last-listed author Leonard H. van den Berg: h-index 94 (OpenAlex, whole career) |
| Institution's track record | 2 / 3 | University of Oxford: institution h-index 2,205 (OpenAlex) |
| Multi-centre collaboration | 3 / 3 | OpenAlex lists 20 institution(s) in 9 countries |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 29598923) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 63 / 100 | — |
What each of these 20 components measures, and what this score is not →
Read the original
- DOI: 10.1016/s1474-4422(18)30089-9
- PubMed: 29598923
- Original source →
- Free full text: Institutional Research Information System University of Turin (University of Turin) →
Full text: free to read — OpenAlex lists this copy as free to read: the link above (checked 6 Feb 1020).
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
Built from the record alzhub holds for this paper, not looked up. Volume, issue and page numbers are not held in the record. The record's author list ends in “et al.”, so not every author is named. Check the citation against the original before you use it.
APA 7th
Westeneng, H. J., Debray, T. P. A., Visser, A. E., van Eijk, R. P. A., Rooney, J. P. K., Calvo, A., et al. (2018). Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model. The Lancet. Neurology. https://doi.org/10.1016/s1474-4422(18)30089-9
Vancouver
Westeneng HJ, Debray TPA, Visser AE, van Eijk RPA, Rooney JPK, Calvo A, et al. Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model. The Lancet. Neurology. 2018. doi:10.1016/s1474-4422(18)30089-9. PMID: 29598923.
BibTeX
@article{westeneng2018-29598923,
author = {Westeneng, H. J. and Debray, T. P. A. and Visser, A. E. and van Eijk, R. P. A. and Rooney, J. P. K. and Calvo, A. and Martin, S. and McDermott, C. J. and others},
title = {{Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model}},
journal = {The Lancet. Neurology},
year = {2018},
doi = {10.1016/s1474-4422(18)30089-9},
pmid = {29598923},
url = {https://doi.org/10.1016/s1474-4422(18)30089-9}
}
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