Developing early diagnostics for prion diseases

Abstract

SUMMARY The diagnosis of prion disease is typically made late in the clinical course, by which time patients are in an advanced state of neurological decline. This is despite the presence of pathology in many tissues, particularly those of the lymphoreticular and central nervous systems. The recent description of an effective blood assay for variant Creutzfeldt-Jakob disease clearly demonstrates the potential for routine pre-mortem diagnosis, although further progress is required for the detection of sporadic forms of the disease.