Results 71 to 80 of about 5,084,169 (167)

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

open access: yesAnnals of Neurology, Volume 99, Issue 5, Page 1303-1314, May 2026.
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   +13 more
wiley   +1 more source

The Molecular Pathology of Prion Diseases [PDF]

open access: yes, 2004
Prion diseases, or transmissible spongiform encephalopathies (TSEs), are a group of invariably fatal neurodegenerative disorders. Uniquely, they may present as sporadic, inherited, or infectious forms, all of which involve conversion of the normal ...
Vassallo, Neville   +2 more
core  

Sporadic Creutzfeldt–Jakob Disease Appears to Be Sporadic Fatal Insomnia: A Case Report and Review of the Literature

open access: yesNature and Science of Sleep, 2021
Qingqing Sun, Pingping Shen, Jiayi Tang, Hongmei Meng, Jiachun Feng, Zan Wang, Li Cui Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, People’s Republic of ChinaCorrespondence: Zan Wang; Li Cui ...
Sun Q   +6 more
doaj  

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

open access: yesBrain 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   +13 more
wiley   +1 more source

Sporadic Creutzfeldt-Jakob Disease.

open access: yes, 2011
The book chapter describes the epidemiology, genetics, clinical features, imaging and laboratory findings, histopathology (including immunohistochemistry and ultrastructure), molecular pathology, subtype classification and differential diagnosis of ...
Head M. W.   +5 more
core   +1 more source

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

open access: yesAnnals 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   +10 more
wiley   +1 more source

Epilepsia partialis continua as the presenting manifestation of Creutzfeldt–Jakob disease: A video‐polygraphic clinical vignette

open access: yes
Epileptic Disorders, Volume 28, Issue 3, Page 920-924, June 2026.
Roberta Cutellè   +8 more
wiley   +1 more source

Neuroprotection trial design in progressive supranuclear palsy: challenges and solutions

open access: yesAlzheimer'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

The worst is yet to come: probable sporadic Creutzfeldt–Jakob disease in a well-controlled HIV patient

open access: yesPrion, 2019
We describe a case of probable sporadic Creutzfeldt–Jakob disease in the setting of well-controlled HIV and discuss whether exist, in fact, HIV-related factors that may predispose to the development of prion disease. To the best of our knowledge, this is
Euripedes Gomes De Carvalho Neto   +8 more
doaj   +1 more source

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

open access: yesEFSA 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)   +25 more
wiley   +1 more source

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