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