Disease progression and survival in ALS: First multi-state model approach

Although several prognostic factors have been identified in ALS, there remains some discordance concerning the prognostic significance of the age and clinical form at onset. In order to clarify these findings, we have analysed already known prognostic factors using a multi-state model. Two hundred a...

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Published inAmyotrophic lateral sclerosis Vol. 8; no. 4; pp. 224 - 229
Main Authors Gil, Juan, Preux, Pierre-Marie, Alioum, Ahmadou, Ketzoian, Carlos, Desport, Jean-Claude, Druet-Cabanac, Michel, Couratier, Philippe
Format Journal Article
LanguageEnglish
Published England Informa UK Ltd 01.01.2007
Taylor & Francis
Elsevier
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Abstract Although several prognostic factors have been identified in ALS, there remains some discordance concerning the prognostic significance of the age and clinical form at onset. In order to clarify these findings, we have analysed already known prognostic factors using a multi-state model. Two hundred and twenty-two sporadic ALS patients were followed. A simple unidirectional three-states model was used to summarize clinical course of ALS. States 1 and 2 reflected the progression of neurological impairment and state 3 represented the end of follow-up (tracheotomy or death). Gender, diagnostic delay, body mass index (BMI) and slow vital capacity (SVC) were also recorded. A time-inhomogeneous Markov model with piecewise constant transition intensities was used to estimate the effect of the covariates in each transition. The bulbar form at onset was only correlated with a more rapid clinical progression between state 1 and state 2. In contrast, an advanced age at diagnosis affected only survival from state 2. This methodological approach suggests that these two factors have a different prognostic significance: age at onset is related to patient's survival and the clinical form at onset predicts the progression of motoneuronal impairment in different regions.
AbstractList Although several prognostic factors have been identified in ALS, there remains some discordance concerning the prognostic significance of the age and clinical form at onset. In order to clarify these findings, we have analysed already known prognostic factors using a multi-state model. Two hundred and twenty-two sporadic ALS patients were followed. A simple unidirectional three-states model was used to summarize clinical course of ALS. States 1 and 2 reflected the progression of neurological impairment and state 3 represented the end of follow-up (tracheotomy or death). Gender, diagnostic delay, body mass index (BMI) and slow vital capacity (SVC) were also recorded. A time-inhomogeneous Markov model with piecewise constant transition intensities was used to estimate the effect of the covariates in each transition. The bulbar form at onset was only correlated with a more rapid clinical progression between state 1 and state 2. In contrast, an advanced age at diagnosis affected only survival from state 2. This methodological approach suggests that these two factors have a different prognostic significance: age at onset is related to patient's survival and the clinical form at onset predicts the progression of motoneuronal impairment in different regions.
Author Preux, Pierre-Marie
Desport, Jean-Claude
Gil, Juan
Couratier, Philippe
Druet-Cabanac, Michel
Alioum, Ahmadou
Ketzoian, Carlos
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Snippet Although several prognostic factors have been identified in ALS, there remains some discordance concerning the prognostic significance of the age and clinical...
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SubjectTerms Age Factors
Age of Onset
Aged
Amyotrophic Lateral Sclerosis
Amyotrophic Lateral Sclerosis - diagnosis
Amyotrophic Lateral Sclerosis - epidemiology
Amyotrophic Lateral Sclerosis - mortality
Amyotrophic Lateral Sclerosis - physiopathology
Analysis of Variance
Antimetabolites, Antineoplastic
Antineoplastic Combined Chemotherapy Protocols
Body Mass Index
Carcinoma, Non-Small-Cell Lung
Cohort Studies
Comorbidity
Deoxycytidine
Disease Progression
epidemiology
Female
Humans
Life Sciences
Male
Markov Chains
Middle Aged
multi-states model
Neoplasm Staging
Outcome Assessment (Health Care)
Outcome Assessment (Health Care) - methods
Patient Selection
Pleural Neoplasms
prognostic factors
Respiration
Retrospective Studies
Santé publique et épidémiologie
Severity of Illness Index
Taxoids
Treatment Outcome
Title Disease progression and survival in ALS: First multi-state model approach
URI https://www.tandfonline.com/doi/abs/10.1080/17482960701278562
https://www.ncbi.nlm.nih.gov/pubmed/17653920
https://search.proquest.com/docview/70747826
https://hal.science/hal-00293964
Volume 8
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