与家族性额颞叶痴呆相关的痴呆中的淀粉样蛋白：并非无辜旁观者。
Amyloid in dementia associated with familial FTLD: not an innocent bystander.
作者信息
Naasan Georges, Rabinovici Gil D, Ghosh Pia, Elofson Jonathan D, Miller Bruce L, Coppola Giovanni, Karydas Anna, Fong Jamie, Perry David, Lee Suzee E, Yokoyama Jennifer S, Seeley William W, Kramer Joel H, Weiner Michael W, Schuff Norbert, Jagust William J, Grinberg Lea T, Pribadi Mochtar, Yang Zhongan, Sears Renee, Klein Eric, Wojta Kevin, Rosen Howard J
机构信息
a Memory and Aging Center, Department of Neurology , University of California , San Francisco , CA , USA.
d Department of Psychiatry, Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine , University of California , Los Angeles , CA , USA.
出版信息
Neurocase. 2016;22(1):76-83. doi: 10.1080/13554794.2015.1046458. Epub 2015 Jun 4.
Patients with frontotemporal lobar degeneration (FTLD) can show superimposed amyloid pathology, though the impact of amyloid on the clinical presentation of FTLD is not well characterized. This cross-sectional case-control study compared clinical features, fluorodeoxyglucose-positron emission tomography metabolism and gray matter volume loss in 30 patients with familial FTLD in whom amyloid status was confirmed with autopsy or Pittsburgh compound B-PET. Compared to the amyloid-negative patients, the amyloid-positive patients performed significantly worse on several cognitive tests and showed hypometabolism and volume loss in more temporoparietal regions. Our results suggest that in FTLD amyloid positivity is associated with a more Alzheimer's disease-like pattern of neurodegeneration.
额颞叶变性（FTLD）患者可能会出现叠加的淀粉样蛋白病变，尽管淀粉样蛋白对FTLD临床表现的影响尚未得到充分描述。这项横断面病例对照研究比较了30例家族性FTLD患者的临床特征、氟脱氧葡萄糖正电子发射断层扫描代谢和灰质体积损失情况，这些患者的淀粉样蛋白状态通过尸检或匹兹堡化合物B-PET得以确认。与淀粉样蛋白阴性的患者相比，淀粉样蛋白阳性的患者在多项认知测试中的表现明显更差，并且在更多的颞顶叶区域表现出代谢减退和体积损失。我们的结果表明，在FTLD中，淀粉样蛋白阳性与更类似阿尔茨海默病的神经退行性变模式相关。
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