文摘
摘要目的:定义神经退化的神经退行性疾病风险和预测在一个大型多中心的特发性REM睡眠行为障碍(RBD)。
背景:特发性RBD对帕金森病已知的最强预测,与路易体痴呆,多系统萎缩。这提供了一个前所未有的机会,直接观察到有前驱症状的神经退行性状态和神经保护治疗干预。神经保护试验,有必要准确地估计神经退化和识别风险预测指标的下降。
设计/方法:我们结合前瞻性随访数据从21中心国际RBD的研究小组。我们没有神经退行性疾病包括polysomnographic-proven RBD患者,在基线,至少1年的随访。每个中心评估睡眠、运动、认知、自主和特殊的感官测试基线。我们用kaplan meier痴呆和帕金森症的风险评估转换分析。预测转换和Cox比例风险分析评估。
结果:从21中心招募了1130名病人。平均年龄为68.1岁,82%是男性,平均随访时间为4.1年(范围= -)。取决于分析方法,痴呆、帕金森症的转化率是12 - 18%,3年,5年24 - 36%,51 - 63%在10年。与嗅觉异常疾病转换风险显著增加(HR = 2.6, p < 0.001),运动症状(HR = 2.6, p < 0.001),微妙的运动迹象(HR = 2.0, p < 0.001),异常定量马达测试(HR = 3.5, p < 0.001),便秘(HR = 1.6, p = 0.003),增加快速眼动语气(HR = 1.6, p = 0.04),和异常的DAT扫描(HR = 3.2, p < 0.001)。没有预测价值嗜睡、失眠、泌尿功能障碍,勃起功能障碍,收缩压下降,抑郁,焦虑,或黑质超声波。最有预测力的标记类似初级痴呆和帕金森症之间的转换。认知和色觉异常预测主痴呆(HR = 11.2, p < 0.001, 3.6, p < 0.002)但不是原发性震颤麻痹。
结论:这个大型多中心研究证实高转化率的特发性RBD神经退行性疾病。我们建立了众多的前驱的标记的预测价值,可用于分层患者神经保护试验。
研究支持:加拿大健康研究所,昏聩de la矫揉造作的桑特魁北克省
披露:Postuma博士已经收到个人赔偿咨询、担任科学顾问委员会说,与Biotie或其他活动,罗氏/ Prothena Teva神经科学,爵士乐制药、诺华加拿大Theranexus,通用电气医疗集团。Iranzo博士没有披露。胡博士没有披露。它没有披露博士。Oertel博士已经收到个人赔偿咨询、担任科学顾问委员会说,与Abbvie或其他活动,金刚石,Desitin, Mundipharma,诺华,Prothena,联合银行。Oertel博士已经收到赔偿服务BiogenIdec董事会,Medigene,默克达姆施塔特,罗氏。Oertel博士已经收到了来自诺华制药研究支持德国。Boeve博士接到GE保健和Axovant研究支持。Hogl博士没有披露。阿努尔夫博士没有披露。 Dr. Arnaldi has nothing to disclose. Dr. Cochen has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with LVL medicla, Orkyn, Eole santé and SOS oxygen. Dr. Mollenhauer has nothing to disclose. Dr. Puligheddu has nothing to disclose. Dr. Sonka has nothing to disclose. Dr. Jung has nothing to disclose. Dr. Videnovic has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Pfizer, Acorda, and Wilsons Therapeutics. Dr. Provini has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Fidia and Vanda Pharmaceutical. Dr. Dauvilliers has nothing to disclose. Dr. Lewis has nothing to disclose. Dr. Heidbreder has nothing to disclose. Dr. Kunz has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Durable, Actelion, Medice. Dr. Pavlova has nothing to disclose. Dr. Montplaisir has nothing to disclose. Dr. Dawson has nothing to disclose. Dr. Pelletier has nothing to disclose. Dr. Gagnon has nothing to disclose. Dr. Santamaria has nothing to disclose. Dr. Barber has nothing to disclose. Dr. Terzaghi has nothing to disclose. Dr. Janzen has nothing to disclose. Dr. St. Louis has nothing to disclose. Dr. Stefani has nothing to disclose. Dr. Nobili has nothing to disclose. Dr. Sixel-Doring has nothing to disclose. Dr. Dusek has nothing to disclose. Dr. Cortelli has nothing to disclose. Dr. Martens has nothing to disclose. Dr. Latreille has nothing to disclose. Dr. Gaig has nothing to disclose. Dr. Trenkwalder has nothing to disclose. Dr. Mahlknecht has nothing to disclose. Dr. Holzknecht has nothing to disclose.
