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Abstract

Diagnostic criteria of Creutzfeldt–Jakob disease (CJD), a rare and fatal transmissible nervous system disease with public health implications, are determined by clinical data, electroencephalogram (EEG), detection of 14-3-3 protein in cerebrospinal fluid (CSF), brain magnetic resonance imaging and prion protein gene examination. The specificity of protein 14-3-3 has been questioned. We reviewed data from 1,572 autopsied patients collected over an 18-year period (1992–2009) and assessed whether and how 14-3-3 detection impacted the diagnosis of sporadic CJD in France, and whether this led to the misdiagnosis of treatable disorders. 14-3-3 detection was introduced into diagnostic criteria for CJD in 1998. Diagnostic accuracy decreased from 92% for the 1992–1997 period to 85% for the 1998–2009 period. This was associated with positive detections of 14-3-3 in cases with negative EEG and alternative diagnosis at autopsy. Potentially treatable diseases were found in 163 patients (10.5%). This study confirms the usefulness of the recent modification of diagnosis criteria by the addition of the results of CSF real-time quaking-induced conversion, a method based on prion seed-induced misfolding and aggregation of recombinant prion protein substrate that has proven to be a highly specific test for diagnosis of sporadic CJD.

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/content/10.2807/1560-7917.ES.2017.22.41.16-00715
2017-10-12
2024-12-22
/content/10.2807/1560-7917.ES.2017.22.41.16-00715
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Accuracy of diagnosis criteria in patients with suspected diagnosis of sporadic Creutzfeldt-Jakob disease and detection of 14-3-3 protein, France, 1992 to 2009
<p class="citation"> <span>Citation style for this article:</span> <span class="meta-value authors"> <a href="/search?value1=Laurene+Peckeu&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Peckeu Laurene</a>, <a href="/search?value1=Nicole+Delasnerie-Laupr%C3%A8tre&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Delasnerie-Lauprètre Nicole</a>, <a href="/search?value1=Jean-Philippe+Brandel&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Brandel Jean-Philippe</a>, <a href="/search?value1=Dominique+Salomon&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Salomon Dominique</a>, <a href="/search?value1=V%C3%A9ronique+Sazdovitch&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Sazdovitch Véronique</a>, <a href="/search?value1=Jean-Louis+Laplanche&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Laplanche Jean-Louis</a>, <a href="/search?value1=Charles+Duyckaerts&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Duyckaerts Charles</a>, <a href="/search?value1=Danielle+Seilhean&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Seilhean Danielle</a>, <a href="/search?value1=St%C3%A9phane+Ha%C3%AFk&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Haïk Stéphane</a>, <a href="/search?value1=Jean-Jacques+Hauw&option1=author&noRedirect=true" class="nonDisambigAuthorLink">Hauw Jean-Jacques</a></span>. Accuracy of diagnosis criteria in patients with suspected diagnosis of sporadic Creutzfeldt-Jakob disease and detection of 14-3-3 protein, France, 1992 to 2009. <a href="/content/ecdc">Euro Surveill.</a> 2017;22(41):pii=16-00715. <a href="https://doi.org/10.2807/1560-7917.ES.2017.22.41.16-00715" title="doi link" target="_blank">https://doi.org/10.2807/1560-7917.ES.2017.22.41.16-00715</a> <span class="ES_Article_citation"> <span class="generated">Received</span>: 03 Nov 2016;&nbsp;&nbsp; <span class="generated">Accepted</span>: 02 Apr 2017 </span> </p>
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