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Paraneoplastic syndromes / limbic encephalitis

In the past few years many auto-immune antigens have been identified that may elicit an auto-immune reaction that leads to a paraneoplastic syndrome.

Antibodies can be found directed against:

- Cytoplasmic antigens, such as:
    Hu, Yo, Ri, Ma1, Ma2, amphipysin, CV2

- Cell-surface antigens, such as:
    NMDA receptor, GABA receptor, AMPA receptor, VGKC, VGCC

Due to overlap in clinical syndromes, a paraneoplastic syndrome and Creutzfeldt-Jakob's disease (CJD) are often considered in the differential diagnosis of a specific patient. Also, in the CSF of patients with a paraneoplastic syndrome, very high concentrations of total tau protein, 14-3-3 protein, NSE and S-100b can be observed, just like in CJD. However, unlike in CJD, in more than 90% of the patients, the CSF of patients with a paraneoplastic syndrome shows signs of inflammation, i.e. at least one of: increased cell count, total protein or unique oligoclonal IgG bands.

Our lab offers a the following analyses in CSF, that may help to distinguish CJD from a paraneoplastic syndrome:
- Total tau protein (t-tau).

- 14-3-3 protein

- Neuron-specific enolase (NSE)

- S-100b protein.

- Phosphorylated tau (p-tau) protein. Levels are normal (or marginally elevated) in sCJD or a paraneoplastic syndrome. This is in contrast to Alzheimer's disease, where both t-tau and p-tau are elevated.

- Anti-Hu, -Yo, -Ri, -Ma1, -Ma2, -CV2 and - amphiphysin antibodies (in serum or CSF)

- 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.

Click here to download analysis protocols