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When prion brain disease, such as Creutzfeldt-Jakob disease (CJD), is suspected, the only definitive diagnostic test is brain biopsy. However, this procedure can cause complications for the patient and risk for the medical team who must handle the biopsy tissue.
A Japanese team developed a test to detect very small amounts of human prion protein (PrPSc). They evaluated the test's accuracy using spinal fluid from several groups of patients with CJD and from control patients with non-prion neurodegenerative diseases. The test was 83% sensitive and 100% specific (i.e., no false-positives occurred) in an initial group of 18 CJD case patients and 35 controls from Japan. In spinal fluid from an additional 25 CJD case patients and 130 controls, the…