title: Influence of timing on CSF tests value for Creutzfeldt-Jakob disease diagnosis.
abstract: 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.
  
  
  METHODS
  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.
authors: Department of Epidemiology and Biostatistics, Erasmus University Medical Center, 3000 DR Rotterdam, The Netherlands.
authors: Pascual
authors: P
authors: Sanchez-Juan
authors: Raquel
authors: R
authors: Sánchez-Valle
authors: Alison
authors: A
authors: Green
authors: Anna
authors: A
authors: Ladogana
authors: Natividad
authors: N
authors: Cuadrado-Corrales
authors: Eva
authors: E
authors: Mitrová
authors: Kathrina
authors: K
authors: Stoeck
authors: Theodoros
authors: T
authors: Sklaviadis
authors: Jerzy
authors: J
authors: Kulczycki
authors: Klaus
authors: K
authors: Hess
authors: Anna
authors: A
authors: Krasnianski
authors: Michele
authors: M
authors: Equestre
authors: Danka
authors: D
authors: Slivarichová
authors: Albert
authors: A
authors: Saiz
authors: Miguel
authors: M
authors: Calero
authors: Maurizio
authors: M
authors: Pocchiari
authors: Richard
authors: R
authors: Knight
authors: Cornelia M
authors: CM
authors: van Duijn
authors: Inga
authors: I
authors: Zerr
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D017668:Age of Onset; D000368:Aged; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid* / Q000175:diagnosis*; D018450:Disease Progression; D005060:Europe; D005260:Female; D006801:Humans; D007391:International Cooperation; D008297:Male; D008875:Middle Aged; D009414:Nerve Growth Factors / Q000134:cerebrospinal fluid; D010751:Phosphopyruvate Hydratase / Q000134:cerebrospinal fluid; D012189:Retrospective Studies; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins / Q000134:cerebrospinal fluid; D018709:Statistics, Nonparametric; D013997:Time Factors; D016875:tau Proteins / Q000134:cerebrospinal fluid
chemical_list: D048948:14-3-3 Proteins; D009414:Nerve Growth Factors; D064568:S100 Calcium Binding Protein beta Subunit; D009418:S100 Proteins; C579160:S100B protein, human; D016875:tau Proteins; D010751:Phosphopyruvate Hydratase