Changes of tau protein in cerebrospinal fluid of sporadic Creutzfeldt-Jakob disease

Xinying Huang; Chenhui Mao; Longze Sha; Caiyan Liu; Liling Dong; Yan Zhou; Jie LI; Dan Lei; Mengyu Zhang; Dongchao Shen; Qin LI; Shanshan Chu; Qi XU; Bin Peng; Liying Cui; Jing Gao

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Changes of tau protein in cerebrospinal fluid of sporadic Creutzfeldt-Jakob disease

VernacularTitle: 散发型克雅病脑脊液tau蛋白的改变
Author: Xinying HUANG ^{1
,

2} ; Chenhui MAO ¹ ; Longze SHA ³ ; Caiyan LIU ¹ ; Liling DONG ¹ ; Yan ZHOU ¹ ; Jie LI ¹ ; Dan LEI ¹ ; Mengyu ZHANG ¹ ; Dongchao SHEN ¹ ; Qin LI ¹ ; Shanshan CHU ¹ ; Qi XU ³ ; Bin PENG ¹ ; Liying CUI ^{1
,

4} ; Jing GAO ^{1
,

2}
Author Information

1. Department of Neurology, Peking Union Medical College Hospital, Beijing 100730, China
2. Peking Union Medical College, Beijing 100730, China
3. Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, Beijing 100730, China
4. Neuroscience Center, Chinese Academy of Medical Sciences, Beijing 100730, China
Publication Type:Journal Article
Keywords: Creutzfeldt-Jakob syndrome; Dementia; Biological markers; Neurodegenerative diseases
From: Chinese Journal of Neurology 2020;53(1):25-30
CountryChina
Language:Chinese
Abstract: Objective:To evaluate the value of cerebrospinal fluid markers expecially total-tau protein (T-tau), phosphorylated-tau protein (P-tau) in diagnosis and differentiation of sporadic Creutzfeldt-Jakob disease (sCJD).
Methods:sCJD (according to 2009 Brain criteria, 2018 Neurology amended criteria), Alzheimer's disease (AD; the National Institute on Aging at National Institutes of Health and the Alzheimer's Association revised guidelines 2011 criteria) and other patients without cognitive impairment, matched for sex and age, in the Department of Neurology, Peking Union Medical College Hospital from 2018 to 2019 were enrolled. Twelve sCJD patients, 49 AD patients and 14 normal controls were enrolled. Cerebrospinal fluid (CSF) specimens were collected through gravity dropping directly, and further stored in -80 ℃ and disposed according to widely used standards. The levels of T-tau and P-tau were measured by ELISA. The data on electroencephalogram and neuroimaging findings of sCJD patients were recorded. Moreover, specimens of sCJD patients were sent to the Chinese Center for Disease Control and Prevention to test 14-3-3 protein and PRNP genotype.
Results:Using Mann-Whitney U test, T-tau concentrations were found higher in patients with sCJD (1 211(448, 2 227) pg/ml) than in AD patients (549(314, 1 078) pg/ml; U=178, P=0.034 9), and both groups had higher T-tau than the control group (127(79, 192) pg/ml; U=20, 73, P<0.01). The level of P-tau was significantly increased in AD patients (72(58,109) pg/ml) compared to the control group (27(15, 42) pg/ml; U=82, P<0.01), but not in sCJD patients (32(24, 47) pg/ml). The T-tau/P-tau ratio was higher in sCJD patients (29.77(20.01, 54.53)) than in AD patients (7.45(4.79, 10.43); U=87, P<0.01). Twelve sCJD patients had cotical hyperintensity on diffusion weighted imaging and five had periodic three-phase waves on electroencephalogram. Nine sCJD patients, whose CSF samples were tested in the Chinese Center for Disease Control and Prevention, carried an M/M genotype at codon 129 and E/E at codon 219.
Conclusion:The CSF tau level and T-tau/P-tau ratio are significantly increased in sCJD, which may promote the diagnosis and differentiation of sCJD in routine clinical setting.