Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal, neurodegenerative disorder leading to quadriplegia and aphagia. While swallowing difficulties and increased energy demand lead to malnutrition, increased lipid concentration may correlate with survival and respiratory functions. Objective: To analyze the frequency and type of dyslipidemias in a large population of clinically characterized ALS patients (PALS). Methods: The retrospective study included clinical and laboratory data of 650 consecutive PALS fulfilling the El Escorial criteria and 365 age- and gender-matched hospital controls. Results: 65% of PALS suffered from dyslipidemia independently of concomitant metabolic diseases. The most frequent lipid disorder was hypercholesterolemia (35% PALS, 25% controls), followed by mixed dyslipidemia (24.6%, 14%), with rare cases of hypertriglyceridemia and atherogenic dyslipidemia. Triacylglycerols (TAG) and LDL/HDL correlated with BMI, while LDL/HDL and total cholesterol (TCh) with disease duration. Among PALS with concomitant metabolic diseases, TCh correlated with disease duration and ALSFRS-R, while TAG with respiratory functions (FVC) in patients without metabolic diseases. The highest median concentration of TCh, LDL and LDL/HDL was found in classic ALS and PMA and the lowest in PBP. Conclusion: Dyslipidemia occurs more frequently in PALS compared to controls and independently of concomitant metabolic diseases. Similar to the general population, the most frequent lipid disturbance is hypercholesterolemia, followed by mixed dyslipidemia. Although particular lipid parameters correlate with BMI and disease duration, they do not show strong correlations with disease progression rate. There is a need of randomized control trials assessing the risk and benefits of the use of lipid lowering agents in ALS.

摘要

肌萎缩侧索硬化 (ALS) 是一种致命的神经退行性疾病，可导致四肢瘫痪和吞咽障碍。虽然吞咽困难和能量需求增加导致营养不良，但脂质浓度增加可能与生存和呼吸功能相关。目的：分析大量具有临床特征的 ALS 患者 (PALS) 中血脂异常的频率和类型。方法：回顾性研究包括 650 名符合 El Escorial 标准的连续 PALS 和 365 名年龄和性别匹配的医院对照的临床和实验室数据。结果：65% 的 PALS 患有与伴随代谢疾病无关的血脂异常。最常见的脂质紊乱是高胆固醇血症（35% PALS，25% 对照组），其次是混合性血脂异常（24.6%，14%），罕见的高甘油三酯血症和致动脉粥样硬化性血脂异常。三酰甘油 (TAG) 和 LDL/HDL 与 BMI 相关，而 LDL/HDL 和总胆固醇 (TCh) 与病程相关。在伴有代谢性疾病的 PALS 中，TCh 与病程和 ALSFRS-R 相关，而 TAG 与无代谢疾病患者的呼吸功能（FVC）相关。在经典 ALS 和 PMA 中发现 TCh、LDL 和 LDL/HDL 的中值浓度最高，而在 PBP 中最低。结论：与对照组相比，在 PALS 中血脂异常发生的频率更高，并且与伴随的代谢疾病无关。与一般人群相似，最常见的脂质紊乱是高胆固醇血症，其次是混合性血脂异常。尽管特定的脂质参数与 BMI 和疾病持续时间相关，但它们与疾病进展率没有很强的相关性。需要进行随机对照试验来评估在 ALS 中使用降脂药物的风险和益处。