King's college progression rate at first clinical evaluation: A new measure of disease progression in amyotrophic lateral sclerosis.


Journal

Journal of the neurological sciences
ISSN: 1878-5883
Titre abrégé: J Neurol Sci
Pays: Netherlands
ID NLM: 0375403

Informations de publication

Date de publication:
15 Dec 2021
Historique:
received: 26 05 2021
revised: 22 10 2021
accepted: 25 10 2021
pubmed: 5 11 2021
medline: 29 1 2022
entrez: 4 11 2021
Statut: ppublish

Résumé

To estimate King's college clinical stage progression rate (ΔKC) at first clinical evaluation in order to define its predictive and prognostic role on survival in a large cohort of Amyotrophic Lateral Sclerosis (ALS) patients. The ΔKC was calculated with the following formula: 0 - KC clinical stage at first clinical evaluation/disease duration from onset to first evaluation, and each result was reported as absolute value. All the evaluations were performed in two cohorts: one from our tertiary centre for motor neuron disease and the other one from a pooled resource open-access ALS clinical trials (PRO-ACT) database. C-statistic was used to evaluate the model discrimination of survival at different time points (1-3 years). Cox proportional hazard model was used to identify factors associated with survival. ΔKC predicted survival at three years in our centre and in the PRO-ACT cohort (C-statistic 0.83, 95% CI 0.8-0.86, p < 0.0001; 0.7, 95% CI 0.68-0.73, p < 0.0001, respectively). At multivariate analysis, ΔKC was independently associated with survival both in our cohort (HR 3.62 95% CI 2.71-4.83 p = 0.001) and in the PRO-ACT cohort (HR 2.75 95% CI 2.1-3.6 p = 0.001). Based on our results, ΔKC could be used as a novel measure of disease progression, hence as an accurate predictor of survival in ALS patients. Indeed, greater values of ΔKC were associated with a 3.5-fold higher risk to experience the event, confirming its robust prognostic value.

Sections du résumé

BACKGROUND BACKGROUND
To estimate King's college clinical stage progression rate (ΔKC) at first clinical evaluation in order to define its predictive and prognostic role on survival in a large cohort of Amyotrophic Lateral Sclerosis (ALS) patients.
METHODS METHODS
The ΔKC was calculated with the following formula: 0 - KC clinical stage at first clinical evaluation/disease duration from onset to first evaluation, and each result was reported as absolute value. All the evaluations were performed in two cohorts: one from our tertiary centre for motor neuron disease and the other one from a pooled resource open-access ALS clinical trials (PRO-ACT) database. C-statistic was used to evaluate the model discrimination of survival at different time points (1-3 years). Cox proportional hazard model was used to identify factors associated with survival.
RESULTS RESULTS
ΔKC predicted survival at three years in our centre and in the PRO-ACT cohort (C-statistic 0.83, 95% CI 0.8-0.86, p < 0.0001; 0.7, 95% CI 0.68-0.73, p < 0.0001, respectively). At multivariate analysis, ΔKC was independently associated with survival both in our cohort (HR 3.62 95% CI 2.71-4.83 p = 0.001) and in the PRO-ACT cohort (HR 2.75 95% CI 2.1-3.6 p = 0.001).
CONCLUSIONS CONCLUSIONS
Based on our results, ΔKC could be used as a novel measure of disease progression, hence as an accurate predictor of survival in ALS patients. Indeed, greater values of ΔKC were associated with a 3.5-fold higher risk to experience the event, confirming its robust prognostic value.

Identifiants

pubmed: 34736124
pii: S0022-510X(21)02743-X
doi: 10.1016/j.jns.2021.120041
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

120041

Informations de copyright

Copyright © 2021 Elsevier B.V. All rights reserved.

Auteurs

Alessandro Introna (A)

Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", piazza Giulio Cesare 11, 70100 Bari, Italy.

Giammarco Milella (G)

Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", piazza Giulio Cesare 11, 70100 Bari, Italy.

Antonella Morea (A)

Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", piazza Giulio Cesare 11, 70100 Bari, Italy.

Maria Ucci (M)

Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", piazza Giulio Cesare 11, 70100 Bari, Italy.

Angela Fraddosio (A)

Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", piazza Giulio Cesare 11, 70100 Bari, Italy.

Stefano Zoccolella (S)

ASL Bari, San Paolo Hospital, Bari, Italy.

Eustachio D'Errico (E)

Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", piazza Giulio Cesare 11, 70100 Bari, Italy.

Isabella Laura Simone (IL)

Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", piazza Giulio Cesare 11, 70100 Bari, Italy. Electronic address: isabellalaura.simone@uniba.it.

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Classifications MeSH