Results 31 to 40 of about 5,281 (133)

Evidence for different seeding activities of misfolded tau in classical and rapidly progressive Alzheimer's disease

Brain Pathology, Volume 36, Issue 3, May 2026.
The study analyzed the seeding activity of misfolded tau protein in Alzheimer's disease and rapidly progressive Alzheimer's disease patients and provides evidence for the existence of different tau assemblies supported by differences in cellular toxicity and morphology of thioflavin T‐positive real‐time quaking‐induced conversion products.
Matthias Schmitz, Katrin Thüne, Anna‐Lisa Fischer, Peter Hermann, Maria Cramm, Leonie Sheila Kadel, Wiebke Möbius, Jakob Matschke, Tayyaba Saleem, Neelam Younas, Saima Zafar, Hermann Altmeppen, Markus Glatzel, Inga Zerr   +13 more
wiley   +1 more source

ENFERMEDAD DE JAKOB-CREUTZFELDT. A PROPÓSITO DE UN CASO

Revistas Argentina de Medicina, 2017
La enfermedad de Jakob-Creutzfeuldt esporádica es la más común de las patologías priónicas humanas. Se presenta el caso de una paciente de 59 años de edad internada por trastornos conductuales cognitivos, acompañados de mioclonías y mutismo acinético.
Lucía González, José Fernández, Vanesa Aguilar, Silvana Tobares, Melisa Contreras, Livio Lanciani, Jimena Boccardo, Juan Carlos Giugni   +7 more
doaj   +2 more sources

Variably Protease‐Sensitive Prionopathy: Two New Cases With Motor Neuron‐Dementia Syndrome

Annals of Clinical and Translational Neurology, Volume 13, Issue 4, Page 827-833, April 2026.
ABSTRACT We describe two patients with variably protease‐sensitive prionopathy (VPSPr) who developed progressive upper motor neuron symptoms, insomnia, behavioral and cognitive decline, compatible with primary lateral sclerosis associated with frontotemporal dementia (FTD).
María Elena Erro, María Victoria Zelaya, Hasier Eraña, Javier Sánchez Ruiz de Gordoa, Fermín García‐Amigot, Anika Simonovska‐Serra, María Cristina Caballero, Isidre Ferrer, Ellen Gelpi, Ivonne Jericó, Joaquín Castilla   +10 more
wiley   +1 more source

Sporadic Creutzfeldt-Jakob disease: clinical observation

Анналы клинической и экспериментальной неврологии, 2017
Creutzfeldt-Jakob disease (CJD) is a form of human prion diseases,fatal neurodegenerative conditions. They can be etiologicallydivided into sporadic, hereditary and acquired forms.Conformational change of the normal (cellular) form of prionprotein (PrPc)
A. V. Peresedova, N. I. Stoida, V. V. Gnezdizky, R. N. Konоvаlоv, O. S. Kоrеpina, I. A. Zavalishin   +5 more
doaj   +1 more source

Distribution of microRNA profiles in pre-clinical and clinical forms of murine and human prion disease

Communications Biology, 2021
Cheng et al. present differentially expressed extracellular vesicle miRNAs in human clinical samples from patients who suffer from sporadic Creutzfeldt-Jakob disease.
Lesley Cheng, Camelia Quek, Xia Li, Shayne A. Bellingham, Laura J. Ellett, Mitch Shambrook, Saima Zafar, Inga Zerr, Victoria A. Lawson, Andrew F. Hill   +9 more
doaj   +1 more source

Sporadic Creutzfeldt–Jakob Disease

Acta Medica Bulgarica
Sporadic Creutzfeldt–Jakob disease is a rare and fatal human prion disease characterized by a rapidly progressive dementia, myoclonus, cerebellar, pyramidal, extrapyramidal, visual, and psychiatric symptoms. These findings are all non-specific and making
Zhelyazkova S., Nachev S., Kalev O., Tournev I.   +3 more
doaj   +1 more source

A blood miRNA signature associates with sporadic Creutzfeldt-Jakob disease diagnosis

Nature Communications, 2020
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive dementia. No clinical blood tests are available for diagnosis. The authors identified three miRNAs in whole-blood that are downregulated in sCJD patients, and discriminate sCJD from ...
Penny J. Norsworthy, Andrew G. B. Thompson, Tze H. Mok, Fernando Guntoro, Luke C. Dabin, Akin Nihat, Ross W. Paterson, Jonathan M. Schott, John Collinge, Simon Mead, Emmanuelle A. Viré   +10 more
doaj   +1 more source

Neuroprotection trial design in progressive supranuclear palsy: challenges and solutions

Alzheimer's &Dementia: Translational Research &Clinical Interventions, Volume 12, Issue 2, April/June 2026.
Abstract INTRODUCTION We review the specific challenges posed to neuroprotective trial design by progressive supranuclear palsy (PSP), a rare disorder difficult to diagnose and to assess quantitatively. METHODS Focusing on reducing the size and cost of trials, we review elements of PSP neuroprotection trial methodology and formulate a new minimum ...
Lawrence I. Golbe, Ronald G. Thomas
wiley   +1 more source

Premortem diagnosis of pathologically confirmed sporadic Creutzfeldt‐Jakob disease

Clinical Case Reports, 2021
Sporadic Creutzfeldt‐Jakob disease should be considered in any case of rapid neuropsychiatric decline. While neuropathological examination of a brain biopsy specimen remains the only definitive diagnostic method and real‐time quaking‐induced conversion ...
Elina T Ziukelis, Vasu Keshav Sharma, James J Gome   +2 more
doaj   +1 more source

Possible alignment of the EU BSE surveillance with the new WOAH provisions

EFSA Journal, Volume 24, Issue 4, April 2026.
Abstract The European Commission requested the assessment of the capacity of the surveillance provisions of the World Organization for Animal Health (WOAH) to detect bovine spongiform encephalopathy (BSE) cases (C‐, H‐ and L‐type) in the European Union (EU) and to propose if any current EU surveillance provisions should be kept.
EFSA Panel on Animal Health and Welfare (AHAW), Søren Saxmose Nielsen, Julio Alvarez, Anette Boklund, Sabine Dippel, Fernanda Dórea, Jordi Figuerola, Mette S. Herskin, Virginie Michel, Miguel Angel Miranda Chueca, Eleonora Nannoni, Anja B. Riber, Karl Stahl, Jan Arend Stegeman, Hans‐Hermann Thulke, Frank Tuyttens, Christoph Winckler, Mark Arnold, Christian Ducrot, John Griffin, Giuseppe Ru, Marion Simmons, Sean Ashe, Angel Ortiz‐ Pelaez, Kamela Kryemadhi, Romolo Nonno   +25 more
wiley   +1 more source