Results 61 to 70 of about 6,976 (171)

Anti-N-methyl-D-Aspartate Receptor Encephalitis Mimicking Sporadic Creutzfeldt–Jakob Disease

Frontiers in Neurology, 2020
Objectives: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and sporadic Creutzfeldt–Jakob disease (sCJD) share similar clinical features. Here, we present two unusual cases of anti-NMDAR encephalitis who were misdiagnosed as sCJD at first ...
Jiao Liu, Liyan Chen, Jing Yang, Lan Wang, Huifang Shang, Xueping Chen   +5 more
doaj   +1 more source

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

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

The Molecular Pathology of Prion Diseases [PDF]

, 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, Herms, Jochen, Kretzschmar, Hans A.   +2 more
core  

Sporadic Creutzfeldt-Jakob Disease.

, 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., PARCHI, PIERO, Ironside J. W., Budka H., Gambetti P., Tagliavini F.   +5 more
core   +1 more source

A case of probable sporadic Creutzfeldt–Jakob disease

Family Medicine and Community Health, 2017
Creutzfeldt-Jakob Disease (CJD) is a fatal neurodegenerative brain disease. The author describes a case presented to primary care clinic whereby neuropsychiatric symptoms were the patient’s initial presentation which later manifested with declining ...
Norita Hussein
doaj   +1 more source

Selective vulnerability to atrophy in sporadic Creutzfeldt‐Jakob disease

Annals of Clinical and Translational Neurology, 2021
Objective Identification of brain regions susceptible to quantifiable atrophy in sporadic Creutzfeldt‐Jakob disease (sCJD) should allow for improved understanding of disease pathophysiology and development of structural biomarkers that might be useful in
Kyan Younes, Julio C. Rojas, Amy Wolf, Goh M. Sheng‐Yang, Matteo Paoletti, Gianina Toller, Eduardo Caverzasi, Maria Luisa Mandelli, Ignacio Illán‐Gala, Joel H. Kramer, Yann Cobigo, Bruce L. Miller, Howard J. Rosen, Michael D. Geschwind   +13 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

A novel form of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report

BMC Neurology, 2010
Background Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder in humans included in the group of Transmissible Spongiform Encephalopathies or prion diseases. The vast majority of sCJD cases are molecularly classified according
Bilbao Miren J, Atarés Begoña, de Pancorbo Marian M, Arteagoitia Jose M, Zarranz Juan J, Garrido Joseba M, Rodríguez-Martínez Ana B, Ferrer Isidro, Juste Ramón A   +8 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