Results 41 to 50 of about 5,376 (165)

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

Movement Disorders in Prionopathies: A Systematic Review

Tremor and Other Hyperkinetic Movements, 2019
Background: Movement disorders are frequent features of prionopathies. However, their prevalence and onset remain poorly described. Methods: We performed a systematic review of case reports and case series of pathologically- and genetically confirmed ...
Federico Rodriguez-Porcel, Vinícius Boaratti Ciarlariello, Alok K. Dwivedi, Lilia Lovera, Gustavo Da Prat, Ricardo Lopez-Castellanos, Ritika Suri, Holly Laub, Ruth H. Walker, Orlando Barsottini, José Luiz Pedroso, Alberto J. Espay   +11 more
doaj   +1 more source

Sporadic Creutzfeldt-Jakob Disease and Other Proteinopathies in Comorbidity [PDF]

Frontiers in Neurology, 2020
Background: Sporadic Creutzfeldt–Jakob disease (sCJD) is the most common type of a group of transmissible spongiform encephalopathies (prion diseases). The etiology of the sporadic form of CJD is still unclear. sCJD can occur in combination with other neurodegenerative diseases, which further complicates the diagnosis.
Eva Parobkova, Eva Parobkova, Julie van der Zee, Julie van der Zee, Lubina Dillen, Lubina Dillen, Christine Van Broeckhoven, Christine Van Broeckhoven, Robert Rusina, Robert Rusina, Radoslav Matej, Radoslav Matej, Radoslav Matej, Radoslav Matej   +13 more
openaire   +4 more sources

Early Remission of Cerebrospinal Fluid Interleukin‐6 After Initial Immunotherapy Predicts a Good Prognosis in Autoimmune Encephalitis

Neurology and Clinical Neuroscience, EarlyView.
ABSTRACT Background Autoimmune encephalitis therapy requires adjusting the regimen based on therapeutic response; however, clinical indicators of this response remain unknown. Aim To determine the predictive capability of cerebrospinal fluid (CSF), electroencephalography (EEG), and brain magnetic resonance imaging (MRI) in identifying responders to ...
Yuta Madokoro, Yuto Uchida, Hiroyasu Inoue, Toyohiro Sato, Teppei Fujioka, Masayuki Mizuno, Shoji Kawashima, Masahiro Oomura, Noriyuki Matsukawa   +8 more
wiley   +1 more source

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

From Meat to Plant‐Based Products? The Enduring Impact of BSE on Beef Consumption

Agricultural Economics, Volume 57, Issue 4, July 2026.
ABSTRACT This study reassesses the impact of the historical BSE outbreak on EU diets, showing that consumption patterns shifted persistently even after policy actions eliminated the food safety risk. Utilizing advanced difference‐in‐differences techniques on 1980–2020 data, we demonstrate that while beef consumption exhibited a transient U‐shaped ...
Jader Velásquez, Shon Ferguson
wiley   +1 more source

HaloTag Fusion Enables Dynamic Analysis of Prion Protein Biosynthesis, Turnover, and Misfolding

ChemBioChem, Volume 27, Issue 11, 15 June 2026.
Prion protein (PrP) misfolding underlies fatal neurodegenerative diseases. We developed a HaloTag‐based PrP fusion enabling spatiotemporal labeling of distinct PrP populations in living cells. This system recapitulates native PrP biology, reveals early misfolding events in disease‐associated mutants, and allows mechanistic interrogation of PrP‐lowering
Antonio Masone, Eleonora Busani, Roberto Chiesa   +2 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

Probable sporadic Creutzfeldt–Jakob disease mimicking focal epilepsy

Epilepsy and Behavior Case Reports, 2019
Creutzfeldt–Jakob disease (CJD) presents with seizures as an early symptom in only approximately 3% of cases. These seizures often present as nonconvulsive status epilepticus (NCSE) or epilepsia partialis continua (EPC).
Pei-Shan Hsiao, Yuan-Ming Lee, Fu-Sin Chu, Chao-Lin Lee, Fang-Chun Liu, Ping-Huang Tsai   +5 more
doaj   +1 more source