Lehéricey Guillaume, Le Forestier Nadine, Dupuis Luc, Gonzalez-Bermejo Jésus, Meininger Vincent, Pradat Pierre-François
AP-HP, groupe hospitalier Pitié-Salpêtrière, service de diététique, 75651 Paris cedex 13, France.
Presse Med. 2012 Jun;41(6 Pt 1):560-74. doi: 10.1016/j.lpm.2011.09.024. Epub 2011 Dec 1.
Malnutrition and dehydration are common and result from swallowing disorders secondary to degeneration of brainstem motor neurons. Recent knowledge argues in favor of the associated primary metabolism abnormalities. Though muscle atrophy, a paradoxical hypermetabolism at rest has often been observed. Hyperlipidemia and glucose intolerance are more frequent than in general population. The heterogeneity of the nutritional assessment of patients in published series is due, partially at least, to the use of disparate criteria and evaluating procedures. Weight lost is an independent negative survival prognostic factor. Overweight may be beneficial for the survival of ALS patients. A specific nutritional management for ALS is an essential point in the multidisciplinary support. The criteria leading to artificial nutrition indication are medical, mainly based on percentage of weight loss, but also psychological and ethical.
营养不良和脱水很常见，是由脑干运动神经元变性继发的吞咽障碍所致。最新研究支持相关的原发性代谢异常。尽管存在肌肉萎缩，但静息时反常的高代谢现象却经常被观察到。高脂血症和葡萄糖不耐受比普通人群更为常见。已发表系列研究中患者营养评估的异质性至少部分归因于使用了不同的标准和评估程序。体重减轻是独立的负面生存预后因素。超重可能对肌萎缩侧索硬化症（ALS）患者的生存有益。ALS的特定营养管理是多学科支持中的关键环节。导致人工营养指征的标准包括医学标准，主要基于体重减轻的百分比，还包括心理和伦理标准。
