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Mol Neurobiol ; 54(8): 5919-5927, 2017 Oct.
Article in English | MEDLINE | ID: mdl-27665282

ABSTRACT

Creutzfeldt-Jakob disease is a rare, but rapidly progressive, up to now untreatable and fatal neurodegenerative disorder. Clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is difficult; however, it can be facilitated by suitable biomarkers. Aim of the present study is to compare levels of cerebrospinal fluid biomarkers (total tau protein, phosphorylated-tau protein, protein 14-3-3 and amyloid beta) in Slovak population of CJD suspect cases, retrospectively in over a 10-year period. One thousand three hundred sixty-four CSF samples from patients with suspect CJD, forming a homogenous group in terms of geographical as well as of equal transport conditions, storage and laboratory processing, were analysed. Definite diagnosis of Creutzfeldt-Jakob disease was confirmed in 101 patients with genetic form, and 60 patients with its sporadic form of the disease. Specificity of protein 14-3-3 and total tau in both forms CJD was similar (87 % for P14-3-3/85 % for total tau), sensitivity to P 14-3-3 and total tau was higher in sporadic Creutzfeldt-Jakob disease (sCJD) (90/95 %) than in genetic Creutzfeldt-Jakob disease (gCJD) (89/74 %). As expected, the total tau levels were significantly higher in CJD patients than in controls, but there was also significant difference between gCJD and sCJD (levels in gCJD were lower; p = 0.003). There was no significant difference in p-tau and Aß 1-42 levels neither between both CJD forms nor between CJD patients and control group.


Subject(s)
Amyloid beta-Peptides/cerebrospinal fluid , Biomarkers/cerebrospinal fluid , Creutzfeldt-Jakob Syndrome/cerebrospinal fluid , tau Proteins/cerebrospinal fluid , 14-3-3 Proteins/cerebrospinal fluid , 14-3-3 Proteins/metabolism , Adult , Aged , Aged, 80 and over , Amyloid beta-Peptides/metabolism , Creutzfeldt-Jakob Syndrome/diagnosis , Diagnosis, Differential , Female , Humans , Male , Middle Aged , Neurodegenerative Diseases/diagnosis , Phosphorylation , Prions/cerebrospinal fluid , Prions/metabolism , Slovakia , Young Adult , tau Proteins/metabolism
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