254 Ch 254. Prion Diseases

Protein-only infectious agents (PrPSc); cause transmissible spongiform encephalopathies (TSE); rapidly progressive neurodegeneration + dementia + ataxia + myoclonus + death; CJD (sporadic, genetic, iatrogenic, variant), GSS, FFI, Kuru in humans; BSE → vCJD human transmission via beef consumption (UK 1990s+); No treatment, universally fatal; CSF RT-QuIC + MRI + tonsil biopsy (vCJD) for diagnosis。