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BACKGROUND:The analysis of markers in the cerebrospinal fluid (CSF) is useful in the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD). However, the time at which the study of these markers is most sensitive remains controversal. OBJECTIVE:To assess the influence of time of sampling on the value of CSF tests in the diagnosis of sCJD. METHOD:In the framework of a multinational European study, we studied the results of 14-3-3, S100b, neurone specific enolase (NSE) and tau protein in 833 CSF samples from sCJD patients at different stages of disease and in 66 sequentially repeated lumbar punctures (LP). RESULTS:14-3-3 and tau protein tended to increase in sensitivity from onset (88%, 81%) to the advanced stage (91%, 90%). This was significant only in the methionine-valine (MV) heterozygous group of patients at codon 129. The absolute levels of S100b (p < 0.05), NSE and tau protein increased in the last stage of disease. High levels of tau protein, NSE and S100b were associated with shorter survival times (p < 0.01). Sixty-six sCJD patients underwent repeated LP. These sCJD patients were younger, had longer disease durations and were more frequently MV at codon 129 (p < 0.001) than the whole group. 14-3-3 sensitivity increased from 64% to 82% in the second LP (p = 0.025) and 88% sCJD patients had at least one positive result. CONCLUSIONS:Sensitivity and absolute levels of CJD markers increased with disease progression and were modulated by the codon 129 genotype. Early negative results should be inter-preted with caution, especially in young patients or those who are MV at codon 129.

Original publication

DOI

10.1007/s00415-006-0472-9

Type

Journal article

Journal

Journal of neurology

Publication Date

07/2007

Volume

254

Pages

901 - 906

Addresses

Department of Epidemiology and Biostatistics, Erasmus University Medical Center, 3000 DR Rotterdam, The Netherlands.

Keywords

Humans, Creutzfeldt-Jakob Syndrome, Disease Progression, Phosphopyruvate Hydratase, Nerve Growth Factors, 14-3-3 Proteins, S100 Proteins, tau Proteins, Statistics, Nonparametric, Retrospective Studies, Age of Onset, Time Factors, International Cooperation, Aged, Middle Aged, Europe, Female, Male, S100 Calcium Binding Protein beta Subunit