J Neurol Neurosur Ps:肌萎缩侧索硬化早期体重减轻的意义

2019-01-12 xing.T MedSci原创

由此可见,从发病到诊断的体重减轻率是ALS患者一个强有力且独立的预后因素。体重减轻主要是由于与吞咽困难相关的营养摄入减少所致,但是在诊断时没有吞咽困难的脊柱发病患者具有严重的体重减轻,并且结局类似于延髓发病的患者。根据该研究结果,研究人员建议在临床试验中,患者应根据入组时有无吞咽困难而不是根据症状发作部位进行分层。

近日,神经病学领域权威取杂志Journal Of Neurology Neurosurgery And Psychiatry上发表了一篇研究文章,该研究旨在评估体重指数(BMI)和体重减轻率作为肌萎缩侧索硬化症(ALS)预后因素的作用,并探讨疾病早期体重减轻的临床相关性。

该研究队列纳入了2007-2011期间Piemonte/Valle d'Aosta的所有ALS患者。研究人员计算了受试者的总体存活率和18个月时死亡/气管造口术的概率(Logistic回归模型)。

在712例患者中,620例(87.1%)患者被纳入研究。患者的生存率与诊断时体重减轻的平均月百分比(p<0.0001)有关,但与诊断时BMI或病态前BMI无关。诊断时存在吞咽困难的脊柱发病患者的中位生存期与延髓发病患者相似。在诊断时没有吞咽困难的脊柱发病患者中约20%具有严重的体重减轻和初始呼吸障碍,并且具有与延髓发作患者类似的中位存活时间。

由此可见,从发病到诊断的体重减轻率是ALS患者一个强有力且独立的预后因素。体重减轻主要是由于与吞咽困难相关的营养摄入减少所致,但是在诊断时没有吞咽困难的脊柱发病患者具有严重的体重减轻,并且结局类似于延髓发病的患者。根据该研究结果,研究人员建议在临床试验中,患者应根据入组时有无吞咽困难而不是根据症状发作部位进行分层。

原始出处:

Cristina Moglia,et al. Early weight loss in amyotrophic lateral sclerosis: outcome relevance and clinical correlates in a population-based cohort. Journal Of Neurology Neurosurgery And Psychiatry. 2018. https://jnnp.bmj.com/content/early/2019/01/10/jnnp-2018-319611

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    2019-04-26 chendoc252
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    2019-03-07 yinhl1978
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    2019-01-14 lsndxfj
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    2019-01-12 坚强007

    向科研人员致敬!!!

    0

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    2019-01-12 天地飞扬

    了解一下,谢谢分享!

    0

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在肌萎缩侧索硬化(ALS)的疾病进展过程中神经性炎症反应发挥了十分关键的作用。在小鼠模型中CD4 Foxp 3调节T细胞(Tregs)通过浸润中枢神经系统抑制神经炎症,促进ALS小鼠神经保护小胶质细胞的激活。近日研究人员就Tregs在调节人ALS的病理生理以及对ALS动物模型的治疗效果进行了考察。研究招募了33名散发性ALS患者进行临床和功能评估,以及免疫学研究,其结果与38名健康志愿者进行对照。

Lancet Neurol:雷沙吉兰可延缓ALS患者疾病进展但不能延长患者生存期

虽然雷沙吉兰对肌萎缩侧索硬化患者具有极高的安全性并且可以延缓疾病进程,但其联合利鲁唑治疗不能延长ALS患者生存期

AGING CELL:抗衰老因子延缓ALS进展

SIRT1是NAD +依赖性脱乙酰酶,其在多种细胞和组织中起作用以减轻与年龄相关的疾病。

NEJM:下颌阵挛-病例报道

鉴于存在上和下运动神经元体征并且保留了感觉通路,因此诊断为肌萎缩侧索硬化。下颌阵挛通常表示皮质脑桥束中的上运动神经元的损伤。

JAMA Neurol:视黄醇结合蛋白4含量与肌萎缩侧索硬化疾病进展

研究认为视黄醇结合蛋白4含量与肌萎缩侧索硬化疾病进展相关,表明维生素A代谢和胰岛素信号通路在ALS疾病生理过程中发挥重要作用

J Neurol Neurosur Ps:饮酒与肌萎缩侧索硬化风险之间的关系

由此可见,除少数例外,饮酒与ALS之间未发现显著相关性。对酒精与ALS之间关联的研究需要进行彻底地探索,特别是考虑到不同类型酒精饮料的作用时。