Cohen O S, Chapman J, Korczyn A D, Warman-Alaluf N, Nitsan Z, Appel S, Kahana E, Rosenmann H
Department of Neurology, The Sagol Neuroscience Center, Chaim Sheba Medical Center, Tel-Hashomer, Israel.
Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
Acta Neurol Scand. 2016 Feb;133(2):119-123. doi: 10.1111/ane.12441. Epub 2015 May 25.
Creutzfeldt-Jakob disease (CJD) is the most common prion disease in humans. The clinical diagnosis of CJD is supported by a combination of electroencephalogram, MRI, and the presence in the CSF of biomarkers. CSF tau is a marker for neuronal damage and tangle pathology, and is correlated with cognitive status in Alzheimer's disease (AD).
The aim of this study was to test whether tau levels in the CSF also correlate with the degree of the neurological deficit and cognitive decline in patients with CJD as reflected by various clinical scales that assess disease severity and cognitive performance.
Consecutive patients with familial CJD (fCJD) were examined by a neurologist who performed several tests including minimental status examination (MMSE), frontal assessment battery (FAB), NIH stroke scale (NIHSS), CJD neurological scale (CJD-NS), and the expanded disability status scale (EDSS). CSF tau was tested as part of the workout, and the correlation was tested using Pearson correlation.
Fifty-two patients with fCJD were recruited to the study (35 males, mean age 59.4 ± 5.7, range 48-75 years). A significant negative correlation was found between CSF tau levels and the cognitive performance of the patients as reflected by their MMSE and FAB scores. In addition, a significant positive correlation was found between tau levels and the clinical disease severity scales of CJD-NS, NIHSS, and EDSS.
The correlation between tau levels and the disease severity and degree of cognitive decline in patients with fCJD suggests that tau can be a biomarker reflecting the extent of neuronal damage.
克雅氏病（CJD）是人类最常见的朊病毒病。脑电图、磁共振成像（MRI）以及脑脊液中生物标志物的存在共同支持CJD的临床诊断。脑脊液tau蛋白是神经元损伤和缠结病理的标志物，与阿尔茨海默病（AD）的认知状态相关。
本研究旨在测试脑脊液中tau蛋白水平是否也与CJD患者神经功能缺损程度和认知衰退程度相关，这些程度由评估疾病严重程度和认知表现的各种临床量表反映。
由神经科医生对连续的家族性克雅氏病（fCJD）患者进行检查，医生进行了多项测试，包括简易精神状态检查表（MMSE）、额叶评估量表（FAB）、美国国立卫生研究院卒中量表（NIHSS）、CJD神经量表（CJD-NS）和扩展残疾状态量表（EDSS）。脑脊液tau蛋白作为检查的一部分进行检测，并使用Pearson相关性检验相关性。
52例fCJD患者被纳入研究（35例男性，平均年龄59.4±5.7岁，范围48 - 75岁）。脑脊液tau蛋白水平与患者的认知表现（由MMSE和FAB评分反映）之间存在显著负相关。此外，tau蛋白水平与CJD-NS、NIHSS和EDSS的临床疾病严重程度量表之间存在显著正相关。
fCJD患者中tau蛋白水平与疾病严重程度和认知衰退程度之间的相关性表明，tau蛋白可以作为反映神经元损伤程度的生物标志物。
