文摘
摘要目的:我们旨在建立的诊断性能2008共识标准多系统萎缩(MSA)的神经病理学定义的队列。
背景:接受2008年的诊断标准为MSA研究和临床实践,然而,这些标准的验证使用神经病理学作为黄金标准还没有被执行。
设计/方法:从数据库的251例病理神经退行性震颤麻痹或小脑障碍指的是梅奥诊所在1995年至2020年之间,83年一个明确的病理诊断MSA患者被报告的作者之一(EAC)和130例排除由于数据不足。神经学家(GL),蒙蔽neuropathologic诊断,进行了回顾性的图表总结的症状,检查发现,实验室,和成像121例(质量检查由FA)。变量记录第一初步评估后6个月(T1)和6个月前最后的评估(T2)。
结果:患者的病理诊断MSA (N = 83), PD (N = 4),下文(N = 20), PSP (N = 10),血管性帕金森症(N = 3),和阿尔茨海默病(N = 1)。后续数据可供63名患者(49.2%)。中位数乘以从出现症状到T1, T1, T2,和T2死是35.6,34.6,和52.2个月。T1, 72/83(86.7%)的MSA会见了MSA患者临床标准(N = 52岁,可能可能N = 20)与11/38(28.9%)相比其他疾病患者(N = 5,可能N = 6)。T2, 40/41(97.6%)的MSA患者遇到的临床标准MSA (N = 36岁,可能可能N = 4)与7/22(31.8%)相比其他疾病患者(可能N = 1, N = 6)。
结论:对患者由专家评估,2008年的敏感性和特异性诊断标准MSA是86.7%和71.1%,分别。后续的敏感性增加到97.6%而特异性仍低68.2%。
披露:博士可能没有披露。阿里博士没有披露。Ahlskog博士已经收到相关出版物的出版版税卫生保健。鲍尔博士的机构已经接到Abbvie研究支持。哈桑博士的机构已经接到Intrabio研究支持。哈桑博士已经收到个人薪酬在500 - 4999美元的范围作为邀请演讲者与韩国社会运动障碍。Boeve博士已经收到个人薪酬在10000 - 49999美元的范围作为一个军官或雨水慈善基金会的董事会成员。Boeve博士的机构收到生原体的研究支持。Boeve博士的机构收到EIP制药的研究支持。Boeve博士的机构已经接到Alector研究支持。 The institution of Dr. Boeve has received research support from GE Healthcare. Dr. Boeve has received publishing royalties from a publication relating to health care. Dr. Cheshire has received personal compensation in the range of $0-$499 for serving as a Consultant for oxford university press. Dr. Cheshire has received personal compensation in the range of $500-$4,999 for serving as a Consultant for elsevier. Dr. Cheshire has received research support from Biohaven. Dr. Cheshire has received personal compensation in the range of $0-$499 for serving as a Visiting professor with Trinity International University. Dr. Cheshire has a non-compensated relationship as a Board of Directors with American Autonomic Society that is relevant to AAN interests or activities. Dr. Cheshire has a non-compensated relationship as a Associate Editor with Clinical Autonomic Research that is relevant to AAN interests or activities. Dr. Dickson has nothing to disclose. Dr. Parisi has nothing to disclose. Dr. Josephs has nothing to disclose. Ann M. Schmeichel has nothing to disclose. Dr. Low has nothing to disclose. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biohaven. Dr. Singer has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Lundbeck. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Catalyst. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Icon PLC. The institution of Dr. Singer has received research support from NIH. Dr. Singer has received intellectual property interests from a discovery or technology relating to health care. Dr. Coon has nothing to disclose.
