Nutritional status is a prognostic factor for survival in ALS patients

Desport, J.C.; Preux, P.M.; Truong, Tri; Vallat, Jean Michel; Sautereau, Denis; Couratier, Philippe

doi:10.1212/wnl.53.5.1059

Cited by 414 publications

(271 citation statements)

References 25 publications

Supporting

Mentioning

244

Contrasting

Unclassified

Order By: Relevance

“…It is worth emphasizing, however, that at least a subset of ALS patients show a characteristic hypermetabolic phenotype (20) reminiscent of that observed in our mice. From a clinical point of view, the nutritional status is a prognostic factor for survival in ALS (37), and growing evidence now supports that the appropriate, individualized nutritional management of patients may constitute a primary symptomatic treatment for the disease (38). In this scenario, our present findings may lend clues to further improve our knowledge about the pathogenic mechanisms underlying ALS and to explore new approaches of nutritional intervention.…”

Section: Resultsmentioning

confidence: 99%

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

Dupuis

Oudart

Frydman

et al. 2004

Proc. Natl. Acad. Sci. U.S.A.

474

472

View full text Add to dashboard Cite

Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by selective loss of motor neurons and progressive muscle wasting. Growing evidence indicates that mitochondrial dysfunction, not only occurring in motor neurons but also in skeletal muscle, may play a crucial role in the pathogenesis. In this regard, the life expectancy of the ALS G93A mouse line is extended by creatine, an intracellular energy shuttle that ameliorates muscle function. Moreover, a population of patients with sporadic ALS exhibits a generalized hypermetabolic state of as yet unknown origin. Altogether, these findings led us to explore whether alterations in energy homeostasis may contribute to the disease process. Here, we show important variations in a number of metabolic indicators in transgenic ALS mice, which in all shows a metabolic deficit. These alterations were accompanied early in the asymptomatic phase of the disease by reduced adipose tissue accumulation, increased energy expenditure, and concomitant skeletal muscle hypermetabolism. Compensating this energetic imbalance with a highly energetic diet extended mean survival by 20%. In conclusion, we suggest that hypermetabolism, mainly of muscular origin, may represent by itself an additional driven force involved in increasing motor neuron vulnerability.

show abstract

Section: Resultsmentioning

confidence: 99%

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

Dupuis

Oudart

Frydman

et al. 2004

Proc. Natl. Acad. Sci. U.S.A.

474

472

View full text Add to dashboard Cite

show abstract

“…Malnutrition in ALS is closely associated to dysphagia and thus an early finding in patients with progressive bulbar palsy occurrance 25 . Due to oro-pharyngeal muscle weakness, measurements such as MIP and MEP are less reliable in those patients.…”

Section: Discussionmentioning

confidence: 99%

Amyotrophic lateral sclerosis: combined nutritional, respiratory and functional assessment

Silva

Mourão

Silva

et al. 2008

Arq. Neuro-Psiquiatr.

View full text Add to dashboard Cite

-Objective:To establish correlations between nutritional, functional and respiratory indices of patients with amyotrophic lateral sclerosis (ALS). Method: Twenty patients (13 appendicular -GA and 7 bulbar -GB) were included in the multidisciplinary study at the Neurological Clinic Ambulatory of the University of Campinas Hospital. Results: Among the GA type significant correlation was observed between maximal inspiratory (MIP) and expiratory (MEP) pressure (r= -0.76), MEP and pulse oxymetry (r=0.58), MIP and percent weight loss (%WL; r=0.59), and between MIP, total and subscale respiratory scores (ALSFRS-R) with %WL. With regard to the GB, correlation was found between MEP and body mass index (BMI) (r=0.97). In both GA and GB correlations were noticed between the BMI and the variables mass (kg), fat (%), arm and wrist circumference (cm), and tricipital, subscapular and supra-iliac skinfolds (mm), as well as the arm muscle circumference (cm) and fatty arm muscular area (mm 2 ). Conclusion: It is suggested that the application of simple anthropometric measurements could be useful in routine monitoring of patients with ALS.KEY WORDS: amyotrophic lateral sclerosis, nutritional support, respiratory tests, ALSFRS scale. esclerose lateral amiotrófica: correlações dos indicadores da avaliação nutricional, funcional e respiratóriaResumo -Objetivo: Correlacionar os indicadores utilizados na avaliação nutricional, funcional e respiratória de indivíduos com esclerose lateral amiotrófica (ELA). Método: Vinte pacientes (13 apendiculares -GA e 7 bulbares -GB) foram incluídos no estudo usando parâmetros nutricionais, respiratórios e escala funcional (ALSFRS-R). Resultados: Entre os pacientes do GA, as correlações observadas foram: pressão inspiratória máxima (PI max ) e expiratória máxima (PE max ) (r= -0,76); PE max e oximetria de pulso (r=0,58); PI max e porcentagem de perda de peso (%PP) (r=0,59); e entre PI max , escore ALSFRS-R com %PP. No GB, houve correlação entre MEP e índice de massa corporal (IMC) (r=0,97). Em GA e GB, observaram-se correlação entre IMC e as variáveis: massa, gordura (%), circunferência braquial e punho, pregas cutâneas tricipital, subescapular e supra-ilíaca, circunferência muscular do braço (cm), área muscular gordurosa do braço (mm 2 ). Conclusão: Sugere-se a aplicação deste conjunto de medidas durante a evolução clínica de indivíduos com ELA. PALAVRAS-CHAVE: esclerose lateral amiotrófica, suporte nutricional, testes respiratórios, escala ALSFRS-R.

show abstract

“…The etiology contributing to this includes dysphagia, impaired motor function (involving bulbar and extremity muscles), and the possibility that a hypermetabolic state exists [101][102][103][104]. The association of weight loss, impaired nutrition, and shortened survival is commonly observed and actively targeted for proactive treatment [105][106][107][108][109][110]. Multiple interventions to address these significant determinants of health and longevity have implicated a multidisciplinary approach involving 1) dieticians (include altering food consistency, high-calorie supplements, and adequate hydration); 2) occupational therapy (hand braces, altered utensils, mobile arm supports, home modifications); 3) physical therapy (range of motion, bracing, seating, maintaining strength); 4) social work (home care assistance, financial support, emotional health); and 5) respiratory therapy (adequate ventilation, secretion management).…”

Section: Advances In Treatment Affecting Disease Coursementioning

confidence: 99%

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

Rosenfeld¹,

Strong

2015

Neurotherapeutics

View full text Add to dashboard Cite

With the acceleration in our understanding of ALS and the related motor neuron disease has come even greater challenges in reconciling all of the proposed pathogenic mechanisms and how this will translate into impactful treatments. Fundamental issues such as diagnostic definition(s) of the disease spectrum, relevant biomarkers, the impact of multiple novel genetic mutations and the significant effect of symptomatic treatments on disease progression are all areas of active investigation. In this review, we will focus on these key issues and highlight the challenges that confront both clinicians and basic science researchers.

show abstract

Nutritional status is a prognostic factor for survival in ALS patients

Abstract: Nutritional surveillance of ALS patients is very important, both in bulbar-onset and spinal-onset patients.

Cited by 414 publications

References 25 publications

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

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

Amyotrophic lateral sclerosis: combined nutritional, respiratory and functional assessment

Challenges in the Understanding and Treatment of Amyotrophic Lateral Sclerosis/Motor Neuron Disease

Contact Info

Product

Resources

About