The role of cerebrospinal fluid 14-3-3 and other proteins in the diagnosis of sporadic Creutzfeldt-Jakob disease in the UK: a 10-year review

Abstract

CSF 14-3-3 had the greatest sensitivity (86%) when compared with tau protein (81%) and S100b (65%). The combination of a positive CSF 14-3-3 or an elevated tau protein with a raised S100b had the highest positive predictive power for sCJD. During the study period, 100 patients were classified as probable sCJD solely on the basis of the clinical features and a positive CSF 14-3-3. The most sensitive marker for sCJD was a positive CSF 14-3-3. The analysis of CSF 14-3-3 plays a crucial role in the case classification of sCJD.

Cerebrospinal fluid real‐time quaking‐induced conversion is a robust and reliable test for sporadic creutzfeldt–jakob disease: An international study

“…Current diagnostic criteria for sCJD rely on clinical features, the results of electroencephalography and magnetic resonance imaging, and the presence of 14‐3‐3 protein in the cerebrospinal fluid (CSF) 1, 2. These tests are not specific for CJD, and none is able to detect all forms of CJD 3, 4…”

Imaging of prion diseases

“…In addition to EEG, multiple CSF markers have been identified; the most frequently cited is the 14-3-3 protein, a marker of neuronal damage. This CSF marker appears more sensitive than EEG with a reported sensitivity of approximately 85% (34,35). However the specificity is relatively low, with multiple false positive results including Hashimoto encephalopathy (36), paraneoplastic encephalopathy (37), herpes encephalitis, cerebrovascular disorders (38), Alzheimer's disease and lymphoma (34) among others.…”

“…GSS disease is another inherited neurodegenerative disease associated with mutations in the PNRP gene characterized by deposition of large amyloid plaque in the cerebellar cortex, as well as the basal ganglia and cerebral cortex (34). Multiple different point mutation and octapeptide repeat insertions are identified, but the most frequent cause is the P102L mutation (1).…”

Subtype Specific CSF Biomarkers in Sporadic Creutzfeldt-Jakob Disease

“…S100B showed similar trend like NSE levels in sCJD homozygous and heterozygous groups. Sensitivities and specificities of CSF S100b range from 65 to 98% and from 29 to 90%, with an area under the ROC of 0.98% [21,45,52,53]. Alone it does not have better predictive potential than the already used clinical markers or CSF 14-3-3, but the combination of S100b with other markers including CSF 14-3-3 may improve diagnostic capability.…”