Results 31 to 40 of about 11,396 (179)

Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments [PDF]

, 2019
Despite their phenotypic heterogeneity, most human prion diseases belong to two broadly defined groups: Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker disease (GSS).
Cali, Ignazio, Cracco, Laura, Gambetti, Pierluigi, Ghetti, Bernardino, Lavrich, Jody, Nemani, Satish K., Notari, Silvio, Surewicz, Witold K., Xiao, Xiangzhu   +8 more
core   +1 more source

Applicability of long-term electroencephalography in pre-mortem diagnosis of Creutzfeldt–Jakob disease: A case report

SAGE Open Medical Case Reports, 2017
Creutzfeldt–Jakob disease accounts for more than 90% of all sporadic prion disease cases. The molecular MM2 genotype has been divided into cortical and thalamic subtypes based on structures involved and is characterized clinically by progressive dementia
Sanaz Attaripour Isfahani, Michelle Dougherty, Gediminas Peter Gliebus   +2 more
doaj   +1 more source

Imaging and CSF analyses effectively distinguish CJD from its mimics [PDF]

, 2017
OBJECTIVE: To review clinical and investigation findings in patients referred to a specialist prion clinic who were suspected to have sporadic Creutzfeldt-Jakob disease (sCJD) and yet were found to have an alternative final diagnosis. METHODS: Review the
Collinge, J, Green, A, Hyare, H, Mead, S, Rudge, P   +4 more
core   +1 more source

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

Human stem cell-derived astrocytes replicate human prions in a PRNP genotype-dependent manner. [PDF]

, 2017
Prions are infectious agents that cause neurodegenerative diseases such as Creutzfeldt-Jakob disease (CJD). The absence of a human cell culture model that replicates human prions has hampered prion disease research for decades.
Alibhai, James, Chandran, Siddharthan, Head, Mark W, Ironside, James W, Krejciova, Zuzana, Krencik, Robert, Manson, Jean, Rzechorzek, Nina M, Ullian, Erik M, Zhao, Chen   +9 more
core   +1 more source

Neurofilament light chain and tau concentrations are markedly increased in the serum of patients with sporadic Creutzfeldt-Jakob disease, and tau correlates with rate of disease progression [PDF]

, 2018
OBJECTIVES: A blood-based biomarker of neuronal damage in sporadic Creutzfeldt-Jakob disease (sCJD) will be extremely valuable for both clinical practice and research aiming to develop effective therapies.
Collinge, J, Heslegrave, AJ, Jackson, GS, Luk, C, Mead, SH, Thompson, AGB, Zetterberg, H   +6 more
core   +1 more source

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  

Enhanced Sensitivity of a Modified Quaking‐Induced Conversion Diagnostic Test for the Broad Detection of Sporadic and Inherited Prion Diseases: A Retrospective Study

Annals of Neurology, EarlyView.
Objective Quaking‐induced conversion (QuIC) tests, which detect prion‐seeding activity in cerebrospinal fluid (CSF), have markedly advanced the antemortem diagnosis of prion diseases such as Creutzfeldt‐Jakob disease (CJD). These tests provide high diagnostic accuracy and enable timely differentiation from other rapidly progressive neurodegenerative ...
Jennifer Myskiw, Rebecca Fox, Dominic M.S. Kielich, Lise Lamoureux, Melanie Leonhardt, Olivia Nykvist, Jessy A. Slota, Kristen Avery, Clark Phillipson, Kathy Frost, Sharon Simon, Brian S. Appleby, Ben A. Bailey‐Elkin, Stephanie A. Booth   +13 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