What are the features of prion associated dementia?
- Generally rapid progression
- May have motor features, e.g., myoclonus, dystonia, chorea, or ataxia
- Biomarkers include CSF 14-3-3 protein
- Stage-dependent electroencephalogram findings include periodic sharp wave complexes
- Magnetic resonance imaging findings (diffusion-weighted imaging) of multifocal cortical and subcortical hyperintensities
