title: CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease.
abstract: OBJECTIVE
  To analyze the diagnostic sensitivity and specificity of various brain-derived proteins (14-3-3, Tau, neuron specific enolase [NSE], and S100b) in the CSF of patients with Creutzfeldt-Jakob disease (CJD) and to analyze biologic factors that modify these parameters.
  
  
  METHODS
  CSF was tested for 14-3-3, Tau, NSE, and S100b in 1,859 patients with sporadic, genetic, iatrogenic, and variant CJD, and in 1,117 controls.
  
  
  RESULTS
  The highest sensitivity was achieved for 14-3-3 and Tau in sporadic CJD (85% and 86%), and a combined determination of 14-3-3 and Tau, S100b, or NSE increased the sensitivity to over 93%. A multivariate analysis showed that the sensitivity of all tests was highest in patients with the shortest disease duration, age at onset >40 years, and homozygosity at codon 129 of the prion protein gene. In a group of patients with repeated lumbar punctures, a second test also increased the diagnostic sensitivity.
  
  
  CONCLUSIONS
  The detection of elevated levels of brain-derived proteins in the CSF in patients with suspected Creutzfeldt-Jakob disease is a valuable diagnostic test. A second lumbar puncture may be of value in patients with atypical clinical course in whom the first test was negative.
authors.affiliation: Department of Epidemiology &s Biostatistics, Erasmus University Medical Center Rotterdam, The Netherlands.
authors.forename: P
authors.initials: P
authors.lastname: Sanchez-Juan
authors.forename: A
authors.initials: A
authors.lastname: Green
authors.forename: A
authors.initials: A
authors.lastname: Ladogana
authors.forename: N
authors.initials: N
authors.lastname: Cuadrado-Corrales
authors.forename: R
authors.initials: R
authors.lastname: Sáanchez-Valle
authors.forename: E
authors.initials: E
authors.lastname: Mitrováa
authors.forename: K
authors.initials: K
authors.lastname: Stoeck
authors.forename: T
authors.initials: T
authors.lastname: Sklaviadis
authors.forename: J
authors.initials: J
authors.lastname: Kulczycki
authors.forename: K
authors.initials: K
authors.lastname: Hess
authors.forename: M
authors.initials: M
authors.lastname: Bodemer
authors.forename: D
authors.initials: D
authors.lastname: Slivarichová
authors.forename: A
authors.initials: A
authors.lastname: Saiz
authors.forename: M
authors.initials: M
authors.lastname: Calero
authors.forename: L
authors.initials: L
authors.lastname: Ingrosso
authors.forename: R
authors.initials: R
authors.lastname: Knight
authors.forename: A C J W
authors.initials: AC
authors.lastname: Janssens
authors.forename: C M
authors.initials: CM
authors.lastname: van Duijn
authors.forename: I
authors.initials: I
authors.lastname: Zerr
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid*; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015415:Biomarkers / Q000134:cerebrospinal fluid; D002648:Child; D002675:Child, Preschool; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid* / Q000175:diagnosis* / Q000453:epidemiology; D003937:Diagnosis, Differential; D005060:Europe / Q000453:epidemiology; D006801:Humans; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000134:cerebrospinal fluid*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity
chemical_list: D048948:14-3-3 Proteins; D015415:Biomarkers; D009419:Nerve Tissue Proteins