Summary Sporadic Creutzfeldt-Jakob disease is a fatal neurodegenerative disease caused
by misfolded prion proteins (PrP Sc). Effective therapeutics are currently not available and …

The most definitive classification systems for dementia are based on the underlying
pathology which, in turn, is categorized largely according to the observed accumulation of …

Objective Several prion amplification systems have been proposed for detection of prions in
cerebrospinal fluid (CSF), most recently, the measurements of prion seeding activity with …

Abstract Purpose of Review: This article presents an update on the clinical aspects of human
prion disease, including the wide spectrum of their presentations. Recent Findings: Prion …

Abstract Sporadic Creutzfeldt–Jakob disease (CJD), the most common human prion
disease, is generally regarded as a spontaneous neurodegenerative illness, arising either …

Importance Fluid biomarkers that can predict survival time in sporadic Creutzfeldt-Jakob
disease (sCJD) will be critical for clinical care and for treatment trials. Objective To assess …

Although prion diseases are generally thought to present as rapidly progressive dementias
with survival of only a few months, the phenotypic spectrum for genetic prion diseases …

Importance Early diagnosis is a requirement for future treatment of prion diseases. Magnetic
resonance imaging (MRI) with diffusion-weighted images and improved real-time quaking …

Prion diseases (PrDs) are a group of neurodegenerative conditions resulting from the
conversion of the normal brain prion protein, the cellular form of prion-related protein (PrPC) …

Objective Real‐time quaking‐induced conversion (RT‐QuIC) assays detect prion‐seeding
activity in a variety of human biospecimens, including cerebrospinal fluid and olfactory …