CSF biomarkers in frontotemporal lobar degeneration with known pathology

CSF biomarkers in frontotemporal lobar degeneration with known pathology
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DOI:
10.1212/01.wnl.0000311445.21321.fc
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发表时间:
2008-05-06
期刊:
影响因子:
9.9
通讯作者:
Grossman, M.
Grossman, M.
中科院分区:
医学1区
文献类型:
--
作者:
Bian, H.;Van Swieten, J. C.;Grossman, M.

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目的:评估脑脊液生物标志物对已知病理学的额颞叶变性 (FTLD) 患者的诊断价值。背景:将 FTLD 与阿尔茨海默病 (AD) 等其他神经退行性疾病区分开来很重要,但由于表现不典型,这在临床上可能很困难。方法:通过尸检或宾夕法尼亚大学和伊拉斯姆斯大学的基因检测来鉴定 FTLD (n = 30) 和 AD (n = 19) 患者医疗中心。脑脊液是在诊断性腰椎穿刺过程中获得的,并使用总 tau 和淀粉样蛋白-β 1-42 (A beta(42)) 测定进行分析。患者还接受了简短的神经心理电池评估。结果:FTLD 中的 CSF 总 tau 水平以及 CSF 总 tau 与 A beta(42) 的比率(tau/A beta(42))显着低于 AD 患者。受试者工作特征曲线分析证实,CSF tau/A beta(42) 比率在区分 FTLD 和 AD 方面具有敏感性和特异性,并且比单独使用 CSF 总 tau 更成功。尽管在尸检证实的 FTLD 和 AD 中,一些神经心理学指标存在显着差异,但将这些神经心理学指标与 CSF 生物标志物相结合并没有提高区分 FTLD 和 AD 的能力。结论:CSF tau/A beta(42) 的比值是区分已知病理学患者的额颞叶变性与阿尔茨海默病的敏感且特异的生物标志物。
Objective: To evaluate the diagnostic value of CSF biomarkers in patients with known pathology due to frontotemporal lobar degeneration (FTLD).Background: It is important to distinguish FTLD from other neurodegenerative diseases like Alzheimer disease (AD), but this may be difficult clinically because of atypical presentations.Methods: Patients with FTLD (n = 30) and AD (n = 19) were identified at autopsy or on the basis of genetic testing at University of Pennsylvania and Erasmus University Medical Center. CSF was obtained during a diagnostic lumbar puncture and was analyzed using assays for total tau and amyloid-beta 1-42 (A beta(42)). Patients also were assessed with a brief neuropsychological battery.Results: CSF total tau level and the ratio of CSF total tau to A beta(42) (tau/A beta(42)) were significantly lower in FTLD than in AD. Receiver operating characteristic curve analyses confirmed that the CSF tau/A beta(42) ratio is sensitive and specific at discriminating between FTLD and AD, and is more successful at this than CSF total tau alone. Although some neuropsychological measures are significantly different in autopsy-proven FTLD and AD, combining these neuropsychological measures with CSF biomarkers did not improve the ability to distinguish FTLD from AD.Conclusions: The ratio of CSF tau/A beta(42) is a sensitive and specific biomarker at discriminating frontotemporal lobar degeneration from Alzheimer disease in patients with known pathology.