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Creutzfeldt–Jakob disease: A case report [PDF]

Radiology Case Reports
Creutzfeldt–Jakob Disease (CJD) is a rare, fatal neurodegenerative disorder that is caused by prion proteins. Patients often present with rapidly progressive dementia, ataxia, myoclonus, memory impairment, visual problems, and changes in personality.
Fahad Rasool Butt, HBSc, Thanansayan Dhivagaran, HBSc, Syed Naqvi, MD, FRCPC, DABR   +2 more
doaj   +2 more sources

Subtype and Regional-Specific Neuroinflammation in Sporadic Creutzfeldt–Jakob Disease [PDF]

Frontiers in Aging Neuroscience, 2014
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 VV2 subtypes compared to age-matched controls. Deregulated genes include pro- and anti-inflammatory cytokines, toll-like receptors, colony stimulating factors, cathepsins ...
Llorens, Franc, López-González, Irene, Thüne, Katrin, Carmona, Margarita, Zafar, Saima, Andréoletti, Olivier, Zerr, Inga, Ferrer, Isidre   +7 more
openaire   +7 more sources

Developing neuropalliative care for sporadic Creutzfeldt-Jakob Disease

Prion, 2022
We aimed to identify targets for neuropalliative care interventions in sporadic Creutzfeldt-Jakob disease by examining characteristics of patients and sources of distress and support among former caregivers.
Krista L. Harrison, Sarah B. Garrett, Joni Gilissen, Michael J. Terranova, Alissa Bernstein Sideman, Christine S. Ritchie, Michael D. Geschwind   +6 more
doaj   +1 more source

Mitochondrial respiratory chain deficiency correlates with the severity of neuropathology in sporadic Creutzfeldt-Jakob disease

Acta Neuropathologica Communications, 2020
Mitochondrial dysfunction has been implicated in multiple neurodegenerative diseases but remains largely unexplored in Creutzfeldt-Jakob disease. Here, we characterize the mitochondrial respiratory chain at the individual neuron level in the MM1 and VV2 ...
Irene H. Flønes, Gerda Ricken, Sigrid Klotz, Alexandra Lang, Thomas Ströbel, Christian Dölle, Gabor G. Kovacs, Charalampos Tzoulis   +7 more
doaj   +1 more source

The First Sporadic Creutzfeldt–Jakob Disease Case with a Rare Molecular Subtype VV1 and 1-Octapeptide Repeat Deletion in PRNP

Viruses, 2021
In the present manuscript, we report the clinical presentation and challenging diagnostic work-up of a sporadic Creutzfeldt–Jakob disease patient with confirmed VV1 subtype and heterozygous 1-octapeptide repeat deletion in the prion protein gene.
Aušrinė Areškevičiūtė, Eva Løbner Lund, Sabina Capellari, Piero Parchi, Christian Tersbøl Pinkowsky   +4 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

Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt-Jakob disease [PDF]

, 2013
Blood transfusion has been identified as a source of human-to-human transmission of variant Creutzfeldt–Jakob disease. Three cases of variant Creutzfeldt–Jakob disease have been identified following red cell transfusions from donors who subsequently ...
Abigail B. Diack, Bishop, Brown, Bruce, Bruce, Bruce, Clarke, Diack, Diane L. Ritchie, Fraser, Garske, Head, Hill, Hilton, HPA, James W. Ironside, Jean C. Manson, Llewelyn, Matthew T. Bishop, Peden, Pocchiari, Prusiner, Ritchie, Robert G. Will, Wadsworth, Will, Wroe   +26 more
core   +1 more source

Creutzfeldt-Jakob Disease Mimicking Wernicke Encephalopathy [PDF]

Journal of Neurocritical Care, 2015
Background: Creutzfeldt-Jakob disease (CJD) is the prototype of a family of rare and fatal human degenerative conditions characterized by rapidly progressive dementia, myoclonus and akinetic mutism.
Min Suck Kim, Sung Je Kim, Youngrok Do
doaj   +1 more source

Proteomic analysis of the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease [PDF]

, 2007
So far, only the detection of 14-3-3 proteins in cerebrospinal fluid (CSF) has been accepted as diagnostic criterion for Creutzfeldt-Jakob disease (CJD). However, this assay cannot be used for screening because of the high rate of false-positive results,
Brit Mollenhauer, Enrico Klingebiel, Hans A. Kretzschmar, Harrington MG, Harrington MG, Jens Wiltfang, Jiang L, Kretzschmar HA, Lukas Cepek, Markus Otto, McKhann G, Mirko Bibl, Otto M, Peter Brechlin, Petra Steinacker, Rabilloud T, Steinhoff BJ, Will RG, Zerr I, Zheng PP   +19 more
core   +1 more source

Follow-up investigations of tau protein and S-100B levels in cerebrospinal fluid of patients with Creutzfeldt-Jakob disease [PDF]

, 2005
Background: S-100B and tau protein have a high differential diagnostic potential for the diagnosis of Creutzfeldt-Jakob disease (CJD). So far there has been only limited information available about the dynamics of these parameters in the cerebrospinal ...
Barbara Ciesielczyk, Birgitt Wiese, Brit Mollenhauer, Green AJ, Hans A. Kretzschmar, Hulstaert F, Inga Zerr, Jens Wiltfang, Kretzschmar HA, Lukas Cepek, Markus Otto, Mirko Bibl, Otto M, Otto M, Petra Steinacker, Sigrid Poser, Steinhoff BJ, Walter Schulz-Schaeffer, Will RG   +18 more
core   +1 more source