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Creutzfeldt-Jakob's Disease

Sporadic Creutzfeldt-Jakob's Disease (sCJD) is a devastating neurodegenerative disorder that affects approximately 1 in every 1,000,000 people. It is a very rapid progressive disease that usually leads to death within months after the diagnosis has been made. Antemortem diagnosis includes, amongst others, analysis of cerebrospinal fluid (CSF). It is widely recommended to analyse the so-called 14-3-3 protein in CSF. The most commonly used technique for this is Western blot analysis. Since this technique is prone to inter-assay and inter-operator variability and it is a non-quantitative technique, other biomarkers in CSF may be more sensitive and specific.

Our lab offers a "sCJD panel" of analyses in CSF, which comprises:

- Total tau protein (t-tau). Usually levels in sCJD are (much) higher than 1500 ng/L, which is strongly elevated compared to the < 350 ng/L levels in controls.

- 14-3-3 protein; a positive result on Western blot is observed in sCJD, but also in other rapidly progressive neurodegenerative disorders

- Neuron-specific enolase (NSE). Levels are (strongly) elevated in sCJD.

- S-100b protein. Levels are (strongly) elevated in sCJD.

- Phosphorylated tau (p-tau) protein. Levels are normal (or marginally elevated) in sCJD. This is in contrast to Alzheimer's disease, where both t-tau and p-tau are elevated. As a consequence, in sCJD the t-tau/p-tau ratio is > 20, whereas in AD this ratio is < 10.

- Cell count and total protein levels are usually normal in CJD; oligoclonal IgG bands are usually absent. The presence of any of these abnormalities may sugegst a paraneoplastic syndrome / limbic encephalitis.

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