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> CJD test came back negative

So a CJD test is usually staining a biopsy with congo red and observing apple-green birefringence in the image. This generally works to detect amyloid plaques, which is common to all prions. So unless there was a lab mistake, the prion is localized elsewhere from where the biopsies are being done (FFI, for example, localizes to a different part of the brain) or they were using some other procedure, it's seems pretty unlikely this is a prion disease.




Are there other tests to determine prion disease related brain degeneration? Seems hard to believe they can't identify at all what the cause is.


Intracranial inoculation of infected tissue is the old-school way to assay for prion activity. Sometimes it takes a few years to get an answer though, especially in cross-species assays [0].

[0] https://link.springer.com/chapter/10.1007/978-1-4614-5338-3_...




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