在REM睡眠行为障碍的172例临床病理的相关性±共存神经障碍(PL01.003)

文摘

摘要目的:确定病理基质患者REM睡眠行为障碍(RBD)±共存神经障碍。

背景:而RBD的嗜睡症是一种常见的表现,经常与痴呆有关,帕金森症和/或自主功能障碍,有有限的证据的基本病理基质这些关联。

设计/方法:分析了临床和neuropathologic发现合作的所有解剖情况下从一个网站在北美和欧洲,评估从1990年1月至2012年3月,他被诊断出患有polysomnogram (PSG)证明或可能的RBD±共存神经障碍。临床和neuropathologic诊断是基于发布标准。

结果:172病例,其中有143(83%)人。均值±SD发病年龄年核心功能如下——RBD: 62±14(20 - 93),认知障碍(n = 147): 69±10(范围22 - 90),震颤麻痹(n = 151): 68±9(20 - 92)和自主功能障碍(n = 42): 62±12(范围23 - 81)。死亡是75±9岁(范围24 - 96)。主要的临床诊断和路易体痴呆(n = 97)、帕金森病±轻度认知障碍或痴呆(n = 32)、多系统萎缩(MSA, n = 19),阿尔茨海默氏症(广告,n = 9)和其他各种疾病包括二级嗜睡症(n = 2)与大脑和神经退行性变的铁accumulation-type 1 (NBAI-1 n = 1)。neuropathologic诊断是路易的身体疾病(精神的小黑裙),n = 77),小黑裙和广告(n = 59)相结合,MSA (n = 19),广告(n = 6), PSP (n = 2),其他神经退行性疾病(n = 6)混合,NBIA-1小黑裙/ tauopathy (n = 1),和下丘脑结构病变(n = 2)。相关的神经退行性疾病中RBD (n = 170), 160(94%)被synucleinopathies。

结论:在这个大系列PSG-confirmed和概率RBD的案件进行了尸检,强大的协会的RBD synucleinopathies进一步证实,和更广泛的频谱紊乱可以构成RBD是更加明显。

支持:国家卫生研究院的基金AG015866、AG006786 AG016574, NS072187, NS072187-01S2, NS072026, AG19610;亚利桑那州卫生服务(合同211002,亚利桑那州阿尔茨海默氏症研究中心),亚利桑那州的生物医学研究委员会(合约4001、0011、05 - 901年和1001年亚利桑那州帕金森病财团);Mangurian基金会;国家帕金森基金会;迈克尔·j·福克斯帕金森氏症研究基金会;和洋底de Investigaciones疗养地(费斯)和Instituo de Salud卡洛斯三世的Maraton TV3基础。

披露:Boeve博士收到头研究支持公司。Allon疗法;和通用电气医疗集团。西尔柏博士没有披露。Ferman博士没有披露。林博士没有披露。Benarroch博士已经收到了个人在一篇社论中补偿神经病学的能力。首页舒梅切尔没有披露博士。Ahlskog博士没有披露。Caselli博士已经收到个人薪酬在一篇社论中临床神经学新闻的能力。首页 Dr. Jacobson has received research support from Avid, Baxter, Bayer, Bristol-Myers Squibb Company, Celgene, Eisai Inc, Elan Corporation, Eli Lilli & Company, General Electric, Genentech, Inc., Janssen, and Pfizer Inc. Dr. Sabbagh has received personal compensation for activities with Amerisciences, Pfizer, Eisai, Lilly, Avid, BMS, Allon. Dr. Sabbagh has received royalty payments from Amerisciences for the development of Cognivite Complete and Wiley. Dr. Sabbagh has received research support from Avid, Baxter, Bayer, BMS, Celgene, Eisai, Elan, Eli Lilly, GE, Genentech, Janssen/Wyeth, and Pfizer. Dr. Adler has received personal compensation for activities with Ipsen, Merz Pharma, and Impax. Dr. Adler has received research support from Avid Radiopharmaceuticals and Phytopharm. Dr. Woodruff has nothing to disclose. Dr. Beach has received research support from Avid Radiopharmaceuticals/Eli Lilly Corporation, Bayer Healthcare, GE Healthcare, GlaxoSmithKline, Inc., Elan Corporation, Janssen, Iperian, Signum, Biogen Idec, HTG Molecular and Neotope. Dr. Iranzo has nothing to disclose. Dr. Gelpi has nothing to disclose. Dr. Santamaria has nothing to disclose. Dr. Tolosa has received personal compensation for activities with Teva Neuroscience, Lundbeck Research USA, Novartis, Boehringer Ingelheim Pharmaceuticals, Merck Serono, UCB and Solvay S.A. as a consultant, speaker and/or participant on an advisory board. Dr. Singer has received personal compensation for activities with Ipsen, Allergan, Merz, IMPAX and Lundbeck. Dr. Singer has received research support from Schwarz Pharma , Synosia/Biotie, Allergan, Astellas, IMPAX and Boehringer Ingelheim. Dr. Mash has nothing to disclose. Dr. Luca has nothing to disclose. Dr. Arnulf has received research support from Bioprojet. Dr. Duyckaerts has nothing to disclose. Dr. Schenck has nothing to disclose. Dr. Mahowald has nothing to disclose. Dr. Dauvilliers has nothing to disclose. Dr. Graff-Radford has received personal compensation for activities with Codman. Dr. Graff-Radford has received personal compensation in an editorial capacity for the Neurologist. Dr. Graff-Radford has received research support from Janssen, Pfizer Pharmaceuticals, Medivation, Forrest, and Allon. Dr. Wszolek has received personal compensation in an editorial cqapacity for Parkinsonism and Related Disorders and the European Journal of Neurology. Dr. Wszolek has received research support from the NIH/NINDS, Mayo Clinical Florida Research Committee CR Program, and Dystonia Medical Research Foundation. Dr. Parisi has nothing to disclose. Dr. Dugger has nothing to disclose. Dr. Murray has nothing to disclose. Dr. Dickson has received personal compensation for activities with Neotope, Inc. as a consultant.