2008年多系统萎缩诊断标准的敏感性和特异性-临床病理研究(P16-11.003)

摘要目的:我们的目的是在一个神经病理学定义的队列中建立2008年多系统萎缩(MSA)共识标准的诊断性能。

背景:2008年MSA的诊断标准在研究和临床实践中被接受，然而，使用神经病理学作为金标准来验证这些标准尚未进行。

设计/方法:从1995年至2020年梅奥诊所提交的251例神经退行性帕金森病或小脑疾病病理病例的数据库中，有83例患者被一位作者(EAC)确定为MSA的明确病理诊断，130例因数据不足而被排除。一位不知道神经病理学诊断的神经科医生(GL)对121例患者的症状、检查结果、实验室和影像学进行了回顾性图表回顾(由FA进行质量检查)。记录初始评估后的前6个月(T1)和最终评估前的最后6个月(T2)的变量。

结果:病理诊断为MSA(83例)、PD(4例)、DLB(20例)、PSP(10例)、血管性帕金森病(3例)、阿尔茨海默病(1例)。63例患者(49.2%)获得随访数据。从症状出现到T1、T1至T2和T2至死亡的中位时间分别为35.6个月、34.6个月和52.2个月。在T1时，72/83(86.7%)的MSA患者符合MSA临床标准(可能N=52，可能N=20)，而11/38(28.9%)的其他疾病患者(可能N=5，可能N=6)。T2时，40/41 (97.6%)MSA患者符合MSA的临床标准(可能N=36，可能N=4)，而7/22(31.8%)其他疾病患者(可能N=1，可能N=6)。

结论:经专家评估的患者，2008年标准诊断MSA的敏感性和特异性分别为86.7%和71.1%。随着随访，敏感性增加到97.6%，而特异性仍然很低，为68.2%。

披露:拉莫特医生没有什么可透露的。艾莉医生没什么要透露的Ahlskog博士从一份与医疗保健有关的出版物中获得了出版版税。鲍尔博士所在的机构得到了艾伯维的研究支持。Hassan博士的机构得到了Intrabio的研究支持。哈桑博士作为韩国运动障碍学会的特邀演讲者，获得了500至4999美元不等的个人补偿。Boeve博士因担任Rainwater Charitable Foundation的官员或董事会成员而获得了10,000- 49,999美元的个人薪酬。Boeve博士的机构得到了Biogen的研究支持。Boeve博士的机构得到了EIP Pharma的研究支持。Boeve博士的机构得到了Alector的研究支持。 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.