TY - T1的纵向球和呼吸下降肌萎缩性脊髓侧索硬化症(ALS) (4786) JF -神经学乔-神经学六世- 94 - 15补充SP - 4786 AU - Nnenna Asidianya AU -劳伦斯Korngut首页盟安琪拉其全称盟Agessandro Abrahao AU - Sanjay卡尔拉盟Lorne Zinman盟雅娜·于Y1 - 2020/04/14 UR - //www.ez-admanager.com/content/94/15_Supplement/4786.abstract N2 -目的:本研究的主要目的是估计的影响逐步呼吸下降进步球下降。第二个目标是确定哪些其他因素可能预测球疾病的发展。背景:肌萎缩性脊髓侧索硬化(ALS)是一种神经退行性疾病,导致运动功能的逐步丧失。呼吸肌肉无力发生在所有患者中,最终导致呼吸衰竭和死亡。球(演讲和吞咽)肌肉也影响多达85%的病人。球和呼吸障碍相关在生理层面上通过舌下神经核运动神经元的损失和功能,同时说话和吞咽呼吸是紧密耦合的。由于这种紧密耦合,通常很难描绘球疾病进展的程度与快速合并呼吸功能下降。设计/方法:471例患者随访纵向(会话总数= 1022)。在每个会话完成ALS患者-功能评定量表修订功能肺活量测试(% FVC),以及一个详细的球评估协议,其中包括一个句子可解性测试和通过阅读。有序的物流用固定效应和随机效应模型使用了随机拦截。球疾病进展的阶段0是实施坐说话速度比; 155 words per minute (WPM), and stage 1 (active) as SIT speaking rate <155 WPM. The stage 0 of respiratory disease was operationalized as %FVCResults: The preliminary results indicated that %FVC was highly predictive of advancing bulbar disease to its active stage; the odds of a patient moving to stage 1 of bulbar disease given that they were at stage 1 of their FVC decline were 3.9 fold. Ongoing work is focused on incorporating the fixed effects (e.g., patient demographics) into the model.Conclusions: NADisclosure: Dr. Asidianya has nothing to disclose. Dr. Korngut has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Alexion, Novartis, Mitsubishi Tanabe Pharma, Sarepta, Biogen, CSL Behring. Dr. Korngut has received compensation for serving on the Board of Directors of Dataffinity Health. Dr. Korngut holds stock and/or stock options in Dataffinity Health. Dr. Korngut has received research support from Biogen Idec, Sanofi Genzyme, Cytokinetics. Dr. Genge has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with AB Sciences, AL-S Pharma, Avexis, Biogen, Cytokinetics, MTPA, and Roche. Dr. Genge has received research support from Sanofi-Genzyme. Dr. Abrahao has nothing to disclose. Dr. Kalra has nothing to disclose. Dr. Zinman has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Mitsubishi Tanabe Pharma Canada.Dr. Yunusova has nothing to disclose. ER -