Malnutrition at the time of diagnosis is associated with a shorter disease duration in ALS

doi:10.1016/j.jns.2010.06.028

Journal of the Neurological Sciences

Volume 297, Issues 1–2, 15 October 2010, Pages 36-39

https://doi.org/10.1016/j.jns.2010.06.028 Get rights and content

Abstract

Background

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. During the course of the illness, malnutrition can occur and may shorten survival. The aim of our study was to determine whether clinical nutritional parameters that are used in daily practice are associated with prognosis and whether they can help guide therapeutic decisions.

Methods

We retrospectively reviewed a cohort of ALS patients in our institution between January 2002 and January 2006. Clinical and demographic outcomes were compiled. To evaluate predictors of survival, we analyzed several clinical nutritional parameters available in daily practice (body mass index, weight loss exceeding 10% of premorbid weight at the time of diagnosis and during the course of the disease and the use of technical supports such as percutaneous endoscopic gastrostomy (PEG) and non-invasive ventilation).

Results

Sixty-three patients were retrospectively studied. Thirteen patients had weight loss exceeding 10% of premorbid weight at the time of diagnosis and thirty patients had weight loss meeting this criterion at final examination. Weight loss exceeding 10% at the time of diagnosis was associated with a shorter duration of disease (17 ± 6 months versus 35 ± 26 months; p = 0.002). A linear correlation was found between mean disease duration and time between onset and diagnosis (p < 0.0001). The subgroup of patients with a PEG had a longer survival time than the other subgroup of patients (p = 0.02).

Conclusions

In ALS patients, early and marked weight loss significantly predicts a worse prognosis. The percentage of premorbid weight loss is a suitable and useful measure that can be used in daily practice to identify patients with a poor prognosis.

Introduction

Amyotrophic lateral sclerosis (ALS) is a severe disease characterized by progressive degeneration of upper and lower motor neurons that has a median survival of approximately 36 months [1]. During the course of ALS, malnutrition and loss of weight, primarily due to loss of muscle tissue, are common and can have direct consequences on disease duration. The causes of malnutrition are multiple and include reduction in caloric and protein intake due to dysphagia or arm weakness and hypermetabolism, which leads to an increase in resting energy expenditure [2], [3]. A more than seven-fold increased risk of death occurs in patients who become malnourished during the course of the disease [3]. Based on the results of experimental studies in animal models [4], [5], altered lipid metabolism has been suspected to partially explain the observed hypermetabolism. In the French ALS population, Dupuis et al. observed longer survivals in patients with hyperlipidemia [6], but this observation was not confirmed in an Italian cohort using a different method. However, Chio et al. found that respiratory impairment is related to lower blood lipid levels [7].

Thus, nutritional assessment is an important component of patient management. The body mass index (BMI), body weight and percentage of premorbid weight loss are all used to assess nutritional status [8]. The BMI has been used in large-scale nutrition surveys and epidemiologic studies of adults because it is easily administered and non-invasive. Recent data suggest that a single measure of BMI does not capture the significance of weight loss or reveal body composition changes [9]. Alternative methods, such as Mildram anthropometry, bioelectrical impedance analysis and dual-energy X-ray absorptiometry, have been used to assess body composition, but these techniques are not always available in daily practice.

The aim of our study was to test several clinical nutritional parameters available in daily practice to determine their importance as prognostic factors for ALS.

Section snippets

Methods

One author (NL) reviewed the database records of all cases discharged from our centre between January 2002 and January 2006 with a diagnosis of definite or probable ALS according to the El Escorial criteria [10].

Cases with no regular follow-up in our centre (less than 2 examinations) were excluded. All patients were treated with riluzole. Non-invasive ventilation (NIV) was used in symptomatic patients based on consensus recommendations [11]. Data on gender, age at symptom onset, site of onset,

Results

A total of 63 patients matching inclusion criteria were identified from our database. Diagnostic certainty was definite in 67% and probable in 33% of cases [10]. The clinical characteristics of our population are shown in Table 1. Five patients had missing data for time between onset and diagnosis. Six patients had a familial history of ALS. None had mutations of the SOD1 gene. Four patients had tracheostomies. Fifteen patients refused NIV and fifteen others refused PEG.

Nutritional data are

Discussion

We aimed to evaluate several available nutritional parameters as prognostic factors in ALS patients. Use of PEG and weight loss exceeding 10% at the time of diagnosis were associated with a shortened lifespan.

In ALS, malnutrition is an independent factor for worse prognosis [3]. Usually, malnutrition is defined by one of the two following criteria: weight loss exceeding 10% or BMI < 18.5 kg/m². Occurrence of malnutrition at all stages of the disease was similar in our population (48%) to other

Conclusions

Few studies have examined ALS patients' weight over the course of their illness. ALS patients with weight loss exceeding 10% at the time of diagnosis seem to have shorter disease duration in our preliminary retrospective study. A more detailed, larger, longitudinal study might be warranted to confirm our results and to determine causes of weight loss and the effect of specific nutritional management in those patients.

Disclosure

The authors have reported no conflict of interest.

References (25)

E.J. Kasarskis et al.
Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death
Am J Clin Nutr
(1996)
B.R. Brooks
El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial “Clinical limits of amyotrophic lateral sclerosis” workshop contributors
J Neurol Sci
(1994)
E.J. Kasarskis et al.
A retrospective study of percutaneous endoscopic gastrostomy in ALS patients during the BDNF and CNTF trials
J Neurol Sci
(1999)
A.C. Ludolph
135th ENMC International Workshop: nutrition in amyotrophic lateral sclerosis 18–20 of March 2005, Naarden, The Netherlands
Neuromuscul Disord
(2006)
H. Mitsumoto et al.
Course and prognosis. Amyotrophic lateral sclerosis
(1998)
J.C. Desport et al.
Nutritional status is a prognostic factor for survival in ALS patients
Neurology
(1999)
L. Dupuis et al.
Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model
Proc Natl Acad Sci USA
(2004)
M.P. Mattson et al.
Energy intake and amyotrophic lateral sclerosis
Neuromolecular Med
(2007)
L. Dupuis et al.
Dyslipidemia is a protective factor in amyotrophic lateral sclerosis
Neurology
(2008)
A. Chiò et al.
Lower serum lipid levels are related to respiratory impairment in patients with ALS
Neurology
(2009)

E.J. Kasarskis et al.

Management of ALS: nutritional care

Neurology

(1996)

A. Rio et al.

Nutritional advice and treatment by dietitians to patients with amyotrophic lateral sclerosis/motor neurone disease: a survey of current practice in England, Wales, Northern Ireland and Canada

J Hum Nutr Diet

(2007)

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In Abdelnour-Mallet et al. [25] patients visited the ALS clinic every 3 months for neurological, nutritional and respiratory monitoring by a MDT. In Limousin et al. [71] BMI was determined at premorbid, time of diagnosis and final study examination. In Fasano et al. [49] BMI was measured at diagnosis, at the time percutaneous endoscopic gastrostomy (PEG) was first required and at three and six months post-PEG.
Poor nutritional outcomes are observed in people with Amyotrophic Lateral Sclerosis (pwALS) including weight loss and poor dietary intake. Surveys of healthcare professionals have highlighted the lack of evidence and knowledge regarding nutritional management of ALS throughout the disease course. Furthermore, national evidence-based guidance is lacking. This mapping review aims to understand the structure and input of nutritional management services for pwALS.
Systematic searches were conducted across eight electronic databases to identify qualitative and quantitative research on structure and input of nutritional care in ALS. Supplementary searches included grey literature, citation and reference list searching of included studies and key reviews, web searching and contacting experts and organisations that provide ALS services to identify guidelines. Study selection and data extraction were undertaken independently by at least two reviewers. Data was synthesised using a narrative approach.
One hundred and nine documents were identified. These consisted of journal articles, guidelines and related documents that contributed evidence towards mapping of nutritional management of pwALS. No evidence on commissioning of nutritional care was identified. Guidelines provided high-level overviews and gave general guidance or recommendations for care; however, these typically focused on gastrostomy with limited guidance on broader aspects, including oral nutrition support. Evidence from primary studies found nutritional care delivery in ALS consisted of multiple types of nutritional management, at different time points during the disease course and involving a range of professionals. There was little evidence relating to proactive nutritional care. Details of healthcare setting, number of professionals involved in care, team composition and how services were delivered in community settings were sparse. Although the role of the speech and language therapist in swallowing assessment and provision of advice on the management of swallowing difficulties was consistent; there was limited evidence on care provided by dietitians. In addition, a small number of studies reported on the use of screening tools. Overall, evidence was consistent that weight management, including monitoring of weight change by professionals and patients, was central and recommended that this should be part of nutritional assessment and follow-up.
The evidence identified in this mapping review has highlighted the requirement for further primary research providing specific details on how nutritional management of pwALS is structured and delivered.
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Dysphagia leads to malnutrition, a major cause of morbidity and mortality in ALS. Weight loss and malnutrition are common presentations and predict a poor outcome in ALS [6]. Respiratory failure is the most common cause of death in ALS [7], and the presence of respiratory muscle weakness serves as a major poor predictor for survival [1].
Knowledge of prognostic factors related to the survival of Motor Neuron Diseases (MND) remains scarce in Southeast Asia.
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One hundred and four MND patients from the Singapore General Hospital (SGH) between January 2004 and December 2017 were reviewed. All relevant clinical data, demographic information were collected. Kaplan-Meier and Cox regression model were performed to identify potential prognostic factors for crucial outcomes (survival, need for feeding support and ventilation support).
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A cohort study was performed in 43 patients with ALS who were referred to the Nutrition Unit between April 2015 and April 2017. Anthropometric parameters and diagnostic times were collected, and the nutritional status was studied through subjective global assessment (SGA). Patients who were included before (control cohort [NoP]) and after (protocol cohort [P]) a multidisciplinary protocol were compared. The mean age of the participants was 66.79 y (10.86 y). Of the patients, 62.8% belonged to the protocol cohort.
Patients who started the protocol had a lower delay in initial assessment by a nutrition specialist (P:2 [1–6] mo/NoP:12 [10–29] mo; P = 0.03). When the nutritional status was analyzed according to the SGA, more patients who did not initiate protocol were in the state of severe malnutrition (C) (P 22.2% versus NoP 60%; P = 0.01). Entry into the protocol was an independent protective factor of the presence of severe malnutrition at the beginning of the nutritional follow-up (odds ratio, 0.20; 95% confidence interval, 0.03–0.73; P = 0.02).
The implementation of a multidisciplinary protocol in ALS allowed patients to present a lower percentage of severe malnutrition in an initial assessment by the nutrition specialist. This protocol is a protective factor for the presence of malnutrition at the beginning of support.
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Malnutrition at the time of diagnosis is associated with a shorter disease duration in ALS

Abstract

Background

Methods

Results

Conclusions

Introduction

Section snippets

Methods

Results

Discussion

Conclusions

Disclosure

Am J Clin Nutr

J Neurol Sci

J Neurol Sci

Neuromuscul Disord

Course and prognosis. Amyotrophic lateral sclerosis

Nutritional status is a prognostic factor for survival in ALS patients

Neurology

Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model

Proc Natl Acad Sci USA

Energy intake and amyotrophic lateral sclerosis

Neuromolecular Med

Dyslipidemia is a protective factor in amyotrophic lateral sclerosis

Neurology

Lower serum lipid levels are related to respiratory impairment in patients with ALS

Neurology

Management of ALS: nutritional care

Neurology

Nutritional advice and treatment by dietitians to patients with amyotrophic lateral sclerosis/motor neurone disease: a survey of current practice in England, Wales, Northern Ireland and Canada

J Hum Nutr Diet