Results 51 to 60 of about 11,518 (218)

The most problematic symptoms of prion disease - an analysis of carer experiences [PDF]

, 2019
Objectives: Prion diseases are rare dementias that most commonly occur sporadically, but can be inherited or acquired, and for which there is no cure.
Collinge, John, Ford, Liz, Gorham, Michele, Mead, Simon, Robinson, Kathy, Rudge, Peter   +5 more
core   +2 more sources

Molecular classification of sporadic Creutzfeldt–Jakob disease [PDF]

Brain, 2003
According to the protein-only hypothesis of prion propagation, an abnormal isoform (designated PrP(Sc)) of the cellular prion protein (PrP(C)) is the principal or sole component of transmissible prions. However, the existence of multiple prion strains has been difficult to accommodate within this hypothesis.
Hill, Andrew F, Joiner, Susan, Wadsworth, Jonathan DF, Sidle, Katie CL, Bell, Jeanne E, Budka, Herbert, Ironside, James W, Collinge, John   +7 more
openaire   +3 more sources

Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease [PDF]

, 2017
Several molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype.
Begue, C., Brandel, J. P., Breithaupt, M., Collie, D., Collins, S. J., Cras, P., Haik, S., Heinemann, U., Hewer, E., Jansen, Gerard H., Kallenberg, K., Ladogana, A., Meissner, B., Pimentel, J., Pocchiari, M., Roberts, H., Romero, C., Sanchez-Juan, P., Schuur, M., Smith, P., Stokin, G. B., Summers, D. M., Taratuto, A., van Duijn, C., Varges, D., Will, R. G., Zerr, I.   +26 more
core  

Tissue classification for the epidemiological assessment of surgical transmission of sporadic Creutzfeldt-Jakob disease. A proposal on hypothetical risk levels

BMC Public Health, 2005
Background Epidemiological studies on the potential role of surgery in Creutzfeldt-Jakob Disease transmission have disclosed associations with history of specific surgical interventions or reported negative results.
Calero Miguel, Siden Åke, Mølbak Kåre, de Pedro-Cuesta Jesús, Rábano Alberto, Laursen Henning   +5 more
doaj   +1 more source

The first reported case of Creutzfeldt‐Jakob disease from Nepal

Clinical Case Reports, 2020
Creutzfeldt‐Jakob disease (CJD) can also be diagnosed in a resource‐limited setting through good clinical analysis. The diagnosis of CJD should be considered in patients with rapidly evolving neurological signs associated with cognitive disturbances even
Himal Kharel, Pabitra Adhikari, Nishan B. Pokhrel, Zeni Kharel, Gaurav Nepal   +4 more
doaj   +1 more source

The Molecular Pathology of Prion Diseases [PDF]

, 2004
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal ...
Herms, Jochen, Kretzschmar, Hans A., Vassallo, Neville   +2 more
core  

Understanding the Phenotypic Heterogeneity Within the Sporadic Creutzfeldt–Jakob Disease MV1 Subtype

Neuropathology and Applied Neurobiology, Volume 52, Issue 3, June 2026.
We present the clinical, pathological and prion biophysical features of three atypical cases of the MV1 subtype of sporadic Creutzfeldt–Jakob disease (sCJD). We propose that these heterozygous cases exist on a spectrum ranging from MM1‐like to VV1‐like phenotypes and recommend that subtyping be performed at pH 6.9 to avoid missing atypical or mixed ...
Satish K. Nemani, Leonardo M. Cortez, Jennifer Myskiw, Ignazio Cali, Stephanie A. Booth, Gerard H. Jansen, Valerie L. Sim   +6 more
wiley   +1 more source

Parkinsonism Associated with Pathological 123I-FP-CIT SPECT (DaTSCAN) Results as the Initial Manifestation of Sporadic Creutzfeldt-Jakob Disease

Case Reports in Neurological Medicine, 2018
Sporadic Creutzfeldt-Jakob disease (sCJD) is a type of progressive, subacute encephalopathy associated with spongiform degeneration of the central nervous system.
Sira Carrasco García de León, Juan Pablo Cabello, Ramón Ortiz, Julia Vaamonde   +3 more
doaj   +1 more source

A case of sporadic Creutzfeldt-Jakob disease [PDF]

Romanian Journal of Neurology, 2012
Sporadic Creutzfeldt-Jakob disease (CJD) is a very rare and fatal disease caused by prions – proteins with unique characteristics and infective potential.
Raoul Pop, Arta Teodorescu, Maria Tanasie, Ruxandra Joikits, Roxana Gheoca, Mihaela Simu   +5 more
doaj   +1 more source

Evolution of Diffusion-Weighted Magnetic Resonance Imaging Signal Abnormality in Sporadic Creutzfeldt-Jakob Disease, With Histopathological Correlation [PDF]

, 2015
IMPORTANCE: Prion diseases represent the archetype of brain diseases caused by protein misfolding, with the most common subtype being sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia.
Brandner, S, Carswell, CJ, Collinge, J, Eisenmenger, L, Hyare, H, Jäger, HR, Mead, S, Porter, MC, Rudge, P, Thompson, A   +9 more
core   +1 more source