Results 21 to 30 of about 16,859 (216)

Long survival sporadic Creutzfeldt-Jakob disease [PDF]

Neurology, 2020
A 63-year-old woman had slowly progressive dementia. EEG displayed periodic sharp wave complexes. MRI showed cortical ribbon pattern in cortex and hyperintensity in basal ganglia (figure, A–H). Genetics testing confirmed homozygosity for methionine at the polymorphic codon 129 of the PRNP gene.
Xiao-Ying Liu, Qi Shi, Gang Wang
openaire   +2 more sources

Total and Phosphorylated Cerebrospinal Fluid Tau in the Differential Diagnosis of Sporadic Creutzfeldt-Jakob Disease and Rapidly Progressive Alzheimer’s Disease

Viruses, 2022
Background: CSF total-tau (t-tau) became a standard cerebrospinal fluid biomarker in Alzheimer’s disease (AD). In parallel, extremely elevated levels were observed in Creutzfeldt-Jakob disease (CJD).
Peter Hermann, Philip Haller, S. Goebel, T. Bunck, C. Schmidt, J. Wiltfang, I. Zerr   +6 more
semanticscholar   +1 more source

Diagnostic approach in a patient with Creutzfeldt-Jakob disease [PDF]

Dementia & Neuropsychologia, 2022
Prion diseases are an important cause of rapidly progressive dementias. Among them, the most common is sporadic Creutzfeldt-Jakob disease (CJD). It is a rare and incurable disease, with rapid progression to death.
José Wagner Leonel Tavares-Júnior, Renata de Oliveira Carvalho, Raul Raposo Pereira Feitosa, Flávia de Paiva Santos Rolim, Felipe Araújo Rocha, Milena Sales Pitombeira, George Linard Silva Malveira, João José Freitas de Carvalho, Norberto Anizio Ferreira Frota, Daniel Aguiar Dias   +9 more
doaj   +1 more source

Characterization of Prion Disease Associated with a Two-Octapeptide Repeat Insertion

Viruses, 2021
Genetic prion disease accounts for 10–15% of prion disease. While insertion of four or more octapeptide repeats are clearly pathogenic, smaller repeat insertions have an unclear pathogenicity.
Nicholas Brennecke, Ignazio Cali, Tze How Mok, Helen Speedy, Genomics England Research Consortium, Laszlo L. P. Hosszu, Christiane Stehmann, Laura Cracco, Gianfranco Puoti, Thomas W. Prior, Mark L. Cohen, Steven J. Collins, Simon Mead, Brian S. Appleby   +13 more
doaj   +1 more source

A discriminative event‐based model for subtype diagnosis of sporadic Creutzfeldt‐Jakob disease using brain MRI

Alzheimer's & Dementia, 2023
Sporadic Creutzfeldt‐Jakob disease (sCJD) comprises multiple subtypes (MM1, MM2, MV1, MV2C, MV2K, VV1, and VV2) with distinct disease durations and spatiotemporal cascades of brain lesions.
V. Venkatraghavan, Riccardo Pascuzzo, E. Bron, M. Moscatelli, M. Grisoli, A. Pickens, M. Cohen, L. Schonberger, P. Gambetti, B. Appleby, S. Klein, A. Bizzi   +11 more
semanticscholar   +1 more source

Diagnostic and prognostic value of plasma neurofilament light and total-tau in sporadic Creutzfeldt-Jakob disease

Alzheimer's Research & Therapy, 2021
Blood neurofilament light (Nfl) and total-tau (t-tau) have been described to be increased in several neurological conditions, including prion diseases and other neurodegenerative dementias.
I. Zerr, Anna Villar-Piqué, Peter Hermann, M. Schmitz, D. Varges, I. Ferrer, J. Riggert, H. Zetterberg, K. Blennow, F. Llorens   +9 more
semanticscholar   +1 more source

The human spongiform encephalopathies [PDF]

Romanian Journal of Neurology, 2018
The human spongiform encephalopathies are a group of heterogenous, usually fatal diseases, characterized by a unique pathogenetic mechanism and distinct clinical presentation. They are classified into sporadic, familial and acquired forms.
Mavroudis Ioannis, Petrides Foivos, Kazis Dimitrios   +2 more
doaj   +1 more source

Sporadic Creutzfeldt–Jakob disease

Neurology, 2012
Sporadic Creutzfeldt-Jakob disease (sCJD) comprises approximately 85% of human prion diseases and is considered due to a post-translational modification of the native prion protein. Definitive diagnosis is only possible through neuropathologic examination at postmortem analysis or via an antemortem brain biopsy. Accurate and early clinical diagnosis of
Appleby, Brian S., Zerr, Inga
openaire   +4 more sources

Biomarkers for sporadic Creutzfeldt–Jakob disease [PDF]

Annals of Clinical and Translational Neurology, 2016
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare but fatal type of spongiform encephalopathy with unknown cause. Unfortunately, definitive diagnosis of this disease can only be done by examination of postmortem brain tissue. Presumptive diagnosis is done through a combination of clinical manifestations, radiology results, and cerebrospinal fluid ...
Soomro, Sanam, Mohan, Chandra
openaire   +2 more sources

Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease

Brain : a journal of neurology, 2009
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.
I. Zerr, K. Kallenberg, D. Summers, C. Romero, A. Taratuto, U. Heinemann, M. Breithaupt, D. Varges, B. Meissner, A. Ladogana, M. Schuur, S. Haik, S. Collins, G. Jansen, G. Stokin, J. Pimentel, E. Hewer, D. Collie, P. Smith, H. Roberts, J. Brandel, C. V. van Duijn, M. Pocchiari, C. Begué, P. Cras, R. Will, P. Sánchez-Juan   +26 more
semanticscholar   +1 more source