The importance of ongoing international surveillance for Creutzfeldt–Jakob disease

N Watson, JP Brandel, A Green, P Hermann… - Nature Reviews …, 2021 - nature.com
Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible
neurodegenerative disease associated with the accumulation of misfolded prion protein in …

Are synucleinopathies prion-like disorders?

E Angot, JA Steiner, C Hansen, JY Li… - The Lancet …, 2010 - thelancet.com
A shared neuropathological feature of idiopathic Parkinson's disease, dementia with Lewy
bodies, and multiple system atrophy is the development of intracellular aggregates of α …

Evidence-based guideline: diagnostic accuracy of CSF 14-3-3 protein in sporadic Creutzfeldt-Jakob disease: report of the guideline development subcommittee of the …

T Muayqil, G Gronseth, R Camicioli - Neurology, 2012 - AAN Enterprises
Objective: To assess the available evidence for the diagnostic accuracy of CSF testing for
protein 14-3-3 in patients with suspected sporadic Creutzfeldt-Jakob disease (sCJD) …

Analysis of clinical features, diagnostic tests, and biomarkers in patients with suspected Creutzfeldt-Jakob disease, 2014-2021

D Shir, EB Lazar, J Graff-Radford, AJ Aksamit… - JAMA Network …, 2022 - jamanetwork.com
Importance Detection of prion proteins in cerebrospinal fluid (CSF) using real-time quaking-
induced conversion (RT-QuIC) assays has transformed the diagnostic approach to sporadic …

Revisiting the Heidenhain variant of Creutzfeldt-Jakob disease: evidence for prion type variability influencing clinical course and laboratory findings

S Baiardi, S Capellari, A Ladogana… - Journal of …, 2016 - content.iospress.com
The Heidenhain variant defines a peculiar clinical presentation of sporadic Creutzfeldt-
Jakob disease (sCJD) characterized by isolated visual disturbances at disease onset and …

Prion diseases

LT Takada, MD Geschwind - Seminars in neurology, 2013 - thieme-connect.com
Prion diseases are a group of diseases caused by abnormally conformed infectious
proteins, called prions. They can be sporadic (Jakob-Creutzfeldt disease [JCD]), genetic …

A novel subtype of sporadic Creutzfeldt–Jakob disease with PRNP codon 129MM genotype and PrP plaques

R Bayazid, C Orru', R Aslam, Y Cohen… - Acta …, 2023 - Springer
The presence of amyloid kuru plaques is a pathological hallmark of sporadic Creutzfeldt–
Jakob disease (sCJD) of the MV2K subtype. Recently, PrP plaques (p) have been described …

Subtype and regional-specific neuroinflammation in sporadic Creutzfeldt–Jakob disease

F Llorens, I López-González, K Thüne… - Frontiers in aging …, 2014 - frontiersin.org
The present study identifies deregulated cytokines and mediators of the immune response in
the frontal cortex and cerebellum of sporadic Creutzfeldt–Jakob disease (sCJD) MM1 and …

Neuropathologically directed profiling of PRNP somatic and germline variants in sporadic human prion disease

GA McDonough, Y Cheng, KS Morillo, RN Doan… - Acta …, 2024 - Springer
Abstract Creutzfeldt–Jakob Disease (CJD), the most common human prion disease, is
associated with pathologic misfolding of the prion protein (PrP), encoded by the PRNP gene …

Variably protease-sensitive prionopathy

S Notari, BS Appleby, P Gambetti - Handbook of clinical neurology, 2018 - Elsevier
Variably protease-sensitive prionopathy (VPSPr), originally identified in 2008, was further
characterized and renamed in 2010. Thirty-seven cases of VPSPr have been reported to …