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Laboratory supported diagnosis of Creutzfeldt-Jakob disease


To analyse the possible use of CSF markers in the diagnosis of CJD;
To modify currently used clinical diagnostic criteria;
To establish a bank of cerebrospinal fluid and serum specimens;
To calculate the incidence figures based on laboratory supported diagnostic criteria and results of neuropathological exarnination;
To provide a diagnostic tool to the European CJD-surveillance (BIOMED 2).

Brief description:
Some proteins have already been shown to be increased in cerebrospinal fluid (CSF) of CJD cases, such as neuron-specific enolase (NSE) and S-lOOb. These proteins will be analyzed with established techniques based on immunological principles such as the enzyme linked immuno sorbent assay (ELISA). The recently described CSF test using antibodies against 14-3-3 protein is reported to be very sensitive and specific for CJD. These analyses will be carried out in the cerebrospinal fluid of suspected CJD-cases. The use of this new CSF marker in the diagnosis of sporadic CreutzfeldtJakob disease and perhaps of NVCJD will be evaluated. Once established, the results of the CSF tests will be made available for the paticipants as they are requested.
By currently used clinical diagnostic criteria cases are missed, especially those with atypical clinical symptoms. A CSF test may improve the methodology and increase the sensitivity of the common diagnostic criteria. Additionally a CSF test may allow to indicate cases with atypical clinical symptoms such as NVCJD. Due to insufficient clinical criteria there are still some cases notified to authorities which may not be CJD cases and one quarter of cases are not detected due to the insufficient clinical diagnostic criteria. The applicability of currently used diagnostic criteria and modified criteria including biochemical markers will be evaluated based on results of -histopathological examination of suspected CJD-cases seen in the European epidemiological CJD-surveillance (BIOMED 2, EUROCJD). Incidence figures of CJD in Europe, Canada and Australia will be calculated based on currently used and on modified criteria. During the whole project, a bank of CSF, serum and plasma specimens will be established and may be used for evaluation of possible tests in future. A cerebrospinal fluid sample bank will provide material for further investigations as there are major scientific interests and increasing efforts in developing diagnostic techniques in vivo.

Creutzfeldt-Jakob disease, NVCJD, prion, risk factors, epidemiology, incidence, cerebrospinal fluid test, biochemical markers, diagnostic tools, diagnostic criteria, CSF-bank.


Georg-August-Universität Göttingen
37075 Göttingen