Creutzfeldt-Jakob Disease Research - Mad Cow Disease, Symptoms, Causes, Variants

Creutzfeldt-Jakob Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Creutzfeldt-Jakob Disease, including details on mad cow disease, symptoms, causes, variants.


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Influence of timing on CSF tests value for Creutzfeldt-Jakob disease diagnosis.

Sanchez-Juan P, Sánchez-Valle R, Green A, Ladogana A, Cuadrado-Corrales N, Mitrová E, Stoeck K, Sklaviadis T, Kulczycki J, Hess K, Krasnianski A, Equestre M, Slivarichová D, Saiz A, Calero M, Pocchiari M, Knight R, van Duijn CM, Zerr I

Dept. of Epidemiology and Biostatistics, Erasmus University Medical Center, 1738, 3000 DR, Rotterdam, The Netherlands.

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.

Published 6 August 2007 in J Neurol, 254(7): 901-6.
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Creutzfeldt-Jakob Disease Research Today Archive:

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