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Abstract
The study aimed to evaluate the fluorescent molecular-imaging probe 2-(1-[6-[(2-fluoroethyl)(methyl)amino]-2-naphthyl]ethylidene)malononitrile (FDDNP) for its ability to selectively and reproducibly label prion plaques in fixed, paraffin-embedded cerebellar sections from patients of confirmed Gerstmann-Sträussler-Scheinker disease, sporadic Creutzfeldt-Jacob disease (CJD) with kuru plaques, and variant CJD (vCJD). FDDNP is a highly hydrophobic, viscosity-sensitive, solvent-sensitive, fluorescent substance, whose radiofluorinated analog [18F]FDDNP has recently been successfully used to label senile plaques and neurofibrillary tangles in the living brain of Alzheimer's disease patients with positron emission tomography. Our results show that FDDNP reliably identifies all prion plaques, including small cluster-plaques in vCJD. This finding may open new in vivo diagnostic possibilities for vCJD.
Highlights
Transmissible spongiform encephalopathies (TSE), known as prion diseases, are a group of invariably fatal neurodegenerative disorders that affect both humans and animals (Prusiner, 1998)
Our results show that FDDNP reliably identifies all prion plaques, including small cluster-plaques in variant CJD (vCJD)
Concerns of possible transmission of prion diseases through surgical and dental procedures, tissue transplantation, and blood transfusion have been fueled by a recent emergence of a new variant Creutzfeldt–Jacob disease (Will et al, 1996) and evidence of its association with the epidemic of bovine spongiform encephalopathy
Summary
Transmissible spongiform encephalopathies (TSE), known as prion diseases, are a group of invariably fatal neurodegenerative disorders that affect both humans and animals (Prusiner, 1998). Despite their rarity (ϳ1 case per million per year), human TSE have a dramatic impact because of their clinical profile and complete absence of effective treatment. There are currently a few antemortem investigations that can consolidate a clinical suspicion of a prion disease
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