Early weight loss in amyotrophic lateral sclerosis: Outcome relevance and clinical correlates in a population-based cohort

Piemonte and Valle d’Aosta Register for ALS (PARALS)

Research output: Contribution to journalArticle

Abstract

Objectives To assess the role of body mass index (BMI) and of the rate of weight loss as prognostic factors in amyotrophic lateral sclerosis (ALS) and to explore the clinical correlates of weight loss in the early phases of the disease. Methods The study cohort included all ALS patients in Piemonte/Valle d'Aosta in the 2007-2011 period. Overall survival and the probability of death/tracheostomy at 18 months (logistic regression model) were calculated. Results Of the 712 patients, 620 (87.1%) were included in the study. Patients ' survival was related to the mean monthly percentage of weight loss at diagnosis (p<0.0001), but not to pre-morbid BMI or BMI at diagnosis. Spinal onset patients with dysphagia at diagnosis had a median survival similar to bulbar onset patients. About 20% of spinal onset patients without dysphagia at diagnosis had severe weight loss and initial respiratory impairment, and had a median survival time similar to bulbar onset patients. Conclusions The rate of weight loss from onset to diagnosis was found to be a strong and independent prognostic factor in ALS. Weight loss was mainly due to the reduction of nutritional intake related to dysphagia, but a subgroup of spinal onset patients without dysphagia at diagnosis had a severe weight loss and an outcome similar to bulbar patients. According to our findings, we recommend that in clinical trials patients should be stratified according to the presence of dysphagia at the time of enrolment and not by site of onset of symptoms.

Original languageEnglish
Pages (from-to)666-673
Number of pages8
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume90
Issue number6
DOIs
Publication statusPublished - Jun 1 2019

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Amyotrophic Lateral Sclerosis
Weight Loss
Deglutition Disorders
Population
Body Mass Index
Survival
Logistic Models
Tracheostomy
Cohort Studies
Clinical Trials

Keywords

  • amyotrophic lateral sclerosis
  • body mass index
  • dysphagia
  • respiratory function
  • weight loss

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology
  • Psychiatry and Mental health

Cite this

Early weight loss in amyotrophic lateral sclerosis : Outcome relevance and clinical correlates in a population-based cohort. / Piemonte and Valle d’Aosta Register for ALS (PARALS).

In: Journal of Neurology, Neurosurgery and Psychiatry, Vol. 90, No. 6, 01.06.2019, p. 666-673.

Research output: Contribution to journalArticle

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abstract = "Objectives To assess the role of body mass index (BMI) and of the rate of weight loss as prognostic factors in amyotrophic lateral sclerosis (ALS) and to explore the clinical correlates of weight loss in the early phases of the disease. Methods The study cohort included all ALS patients in Piemonte/Valle d'Aosta in the 2007-2011 period. Overall survival and the probability of death/tracheostomy at 18 months (logistic regression model) were calculated. Results Of the 712 patients, 620 (87.1{\%}) were included in the study. Patients ' survival was related to the mean monthly percentage of weight loss at diagnosis (p<0.0001), but not to pre-morbid BMI or BMI at diagnosis. Spinal onset patients with dysphagia at diagnosis had a median survival similar to bulbar onset patients. About 20{\%} of spinal onset patients without dysphagia at diagnosis had severe weight loss and initial respiratory impairment, and had a median survival time similar to bulbar onset patients. Conclusions The rate of weight loss from onset to diagnosis was found to be a strong and independent prognostic factor in ALS. Weight loss was mainly due to the reduction of nutritional intake related to dysphagia, but a subgroup of spinal onset patients without dysphagia at diagnosis had a severe weight loss and an outcome similar to bulbar patients. According to our findings, we recommend that in clinical trials patients should be stratified according to the presence of dysphagia at the time of enrolment and not by site of onset of symptoms.",
keywords = "amyotrophic lateral sclerosis, body mass index, dysphagia, respiratory function, weight loss",
author = "{Piemonte and Valle d’Aosta Register for ALS (PARALS)} and Cristina Moglia and Andrea Calvo and Maurizio Grassano and Antonio Canosa and Umberto Manera and Fabrizio D'ovidio and Alessandro Bombaci and Enrica Bersano and Letizia Mazzini and Gabriele Mora and Adriano Chi{\`o} and Stefania Cammarosano and Rosario Vasta and Torrieri, {Maria Claudia} and Luca Solero and Marinella Clerico and {De Mercanti}, Stefania and Alessandro Mauro and Luca Pradotto and {De Marchi}, Fabiola and Luisa Sosso and Daniela Leotta and Lucia Appendino and Daniele Imperiale and Roberto Cavallo and Claudio Geda and Fabio Poglio and Paola Santimaria and Umberto Massazza and Antonio Villani and Roberto Conti and Ruiz, {Luigi C.} and Mario Palermo and Franco Vergnano and Eugenia Rota and Penza, {Maria Teresa} and Marco Aguggia and Piero Meineri and Paolo Ghiglione and Nicola Launaro and Giuseppe Astegiano and Giovanni Corso",
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T1 - Early weight loss in amyotrophic lateral sclerosis

T2 - Outcome relevance and clinical correlates in a population-based cohort

AU - Piemonte and Valle d’Aosta Register for ALS (PARALS)

AU - Moglia, Cristina

AU - Calvo, Andrea

AU - Grassano, Maurizio

AU - Canosa, Antonio

AU - Manera, Umberto

AU - D'ovidio, Fabrizio

AU - Bombaci, Alessandro

AU - Bersano, Enrica

AU - Mazzini, Letizia

AU - Mora, Gabriele

AU - Chiò, Adriano

AU - Cammarosano, Stefania

AU - Vasta, Rosario

AU - Torrieri, Maria Claudia

AU - Solero, Luca

AU - Clerico, Marinella

AU - De Mercanti, Stefania

AU - Mauro, Alessandro

AU - Pradotto, Luca

AU - De Marchi, Fabiola

AU - Sosso, Luisa

AU - Leotta, Daniela

AU - Appendino, Lucia

AU - Imperiale, Daniele

AU - Cavallo, Roberto

AU - Geda, Claudio

AU - Poglio, Fabio

AU - Santimaria, Paola

AU - Massazza, Umberto

AU - Villani, Antonio

AU - Conti, Roberto

AU - Ruiz, Luigi C.

AU - Palermo, Mario

AU - Vergnano, Franco

AU - Rota, Eugenia

AU - Penza, Maria Teresa

AU - Aguggia, Marco

AU - Meineri, Piero

AU - Ghiglione, Paolo

AU - Launaro, Nicola

AU - Astegiano, Giuseppe

AU - Corso, Giovanni

PY - 2019/6/1

Y1 - 2019/6/1

N2 - Objectives To assess the role of body mass index (BMI) and of the rate of weight loss as prognostic factors in amyotrophic lateral sclerosis (ALS) and to explore the clinical correlates of weight loss in the early phases of the disease. Methods The study cohort included all ALS patients in Piemonte/Valle d'Aosta in the 2007-2011 period. Overall survival and the probability of death/tracheostomy at 18 months (logistic regression model) were calculated. Results Of the 712 patients, 620 (87.1%) were included in the study. Patients ' survival was related to the mean monthly percentage of weight loss at diagnosis (p<0.0001), but not to pre-morbid BMI or BMI at diagnosis. Spinal onset patients with dysphagia at diagnosis had a median survival similar to bulbar onset patients. About 20% of spinal onset patients without dysphagia at diagnosis had severe weight loss and initial respiratory impairment, and had a median survival time similar to bulbar onset patients. Conclusions The rate of weight loss from onset to diagnosis was found to be a strong and independent prognostic factor in ALS. Weight loss was mainly due to the reduction of nutritional intake related to dysphagia, but a subgroup of spinal onset patients without dysphagia at diagnosis had a severe weight loss and an outcome similar to bulbar patients. According to our findings, we recommend that in clinical trials patients should be stratified according to the presence of dysphagia at the time of enrolment and not by site of onset of symptoms.

AB - Objectives To assess the role of body mass index (BMI) and of the rate of weight loss as prognostic factors in amyotrophic lateral sclerosis (ALS) and to explore the clinical correlates of weight loss in the early phases of the disease. Methods The study cohort included all ALS patients in Piemonte/Valle d'Aosta in the 2007-2011 period. Overall survival and the probability of death/tracheostomy at 18 months (logistic regression model) were calculated. Results Of the 712 patients, 620 (87.1%) were included in the study. Patients ' survival was related to the mean monthly percentage of weight loss at diagnosis (p<0.0001), but not to pre-morbid BMI or BMI at diagnosis. Spinal onset patients with dysphagia at diagnosis had a median survival similar to bulbar onset patients. About 20% of spinal onset patients without dysphagia at diagnosis had severe weight loss and initial respiratory impairment, and had a median survival time similar to bulbar onset patients. Conclusions The rate of weight loss from onset to diagnosis was found to be a strong and independent prognostic factor in ALS. Weight loss was mainly due to the reduction of nutritional intake related to dysphagia, but a subgroup of spinal onset patients without dysphagia at diagnosis had a severe weight loss and an outcome similar to bulbar patients. According to our findings, we recommend that in clinical trials patients should be stratified according to the presence of dysphagia at the time of enrolment and not by site of onset of symptoms.

KW - amyotrophic lateral sclerosis

KW - body mass index

KW - dysphagia

KW - respiratory function

KW - weight loss

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U2 - 10.1136/jnnp-2018-319611

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