Protein 14-3-3 Detection in Cerebrospinal Fluid – Clinico-Pathological Correlation

Česká verzia

Authors: R. Matěj ¹; J. Nováková ¹; J. Fiala ²; F. Koukolík ²; R. Rusina ²
Authors place of work: Národní referenční laboratoř pro diagnostiku lidských prionových chorob při Oddělení patologie a molekulární medicíny, Fakultní Thomayerova nemocnice s poliklinikou, Praha ¹; Neurologická klinika IPVZ a Fakultní Thomayerova nemocnice s poliklinikou, Praha ²
Published in the journal: Cesk Slov Neurol N 2008; 71/104(6): 695-699
Category: Krátké sdělení

Summary

Introduction:
The 14-3-3 – protein group is formed by seven isotope signal molecules. The exact function of these molecules is not known yet. Detection of the ß-subunit of the 14-3-3 protein in cerebrospinal fluid is one of the most important paraclinical markers in sporadic Creutzfeldt-Jakob disease (sCJD) diagnostic procedure. This marker is not specific and its specificity and sensitivity are influenced by the timing and technical aspects of sampling and especially by proper indication.

Methods:
Protein 14-3-3 detection was performed by a standardized method using western blot with chemoluminiscent detection of ß-subunit in cerebrospinal fluid samples from patients with clinical suspection of CJD.

Results:
57 cerebrospinal fluid samples were 14-3-3 – positive and 20 were weakly positive from all 280 samples in total. From the total number (275) of patients, 62 were examined neuropathologically (22%) post mortem. In 25 cases and in six weakly positive cases of 14-3-3 positivity, prion disease of CJD type was proven on autopsy. In other 18 CJD autopsy proved cases, nevertheless, the 14-3-3 protein was evaluated as negative. In two cases of 14-3-3 positivity and in two weakly positive cases a different neuropathological substrate than prion disease was found.

Conclusion:
Our results correspond to previously published data, where a predictive value in ß-subunit of protein 14-3-3 detection in CSF is discussed. In comparison to recent publications both sensitivity and specificity of 14-3-3 protein detection for CJD diagnosis is significantly lower in our patient group. This observation can be influenced by our small patient group. Nevertheless, a positive finding of protein 14-3-3 in the cerebrospinal fluid is one of the requirements for CJD diagnosis according to WHO diagnostic criteria. Because of its relatively low specificity it is important to consider the relevance of 14-3-3 positive test in each particular clinical case.

Key words:
14-3-3 protein – prion disease – Creutzfeldt–Jakob disease – neuropathology – diagnostics

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