Results 21 to 30 of about 5,518 (132)

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

open access: yesActa 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   +7 more
doaj   +1 more source

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

open access: yesDementia & 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   +9 more
doaj   +1 more source

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

open access: yesViruses, 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   +13 more
doaj   +1 more source

The human spongiform encephalopathies [PDF]

open access: yesRomanian 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   +2 more
doaj   +1 more source

Sporadic MM-1 Type Creutzfeldt-Jakob Disease With Hemiballic Presentation and No Cognitive Impairment Until Death: How New NCJDRSU Diagnostic Criteria May Allow Early Diagnosis

open access: yesFrontiers in Neurology, 2018
Sporadic Creutzfeldt-Jakob disease is the most common human prion disorder. Although associated with heterogeneous clinical phenotypes, its distinctive feature is the presence of a rapidly progressive multidomain cognitive impairment.
Lorenzo Saraceno   +8 more
doaj   +1 more source

High diagnostic performance of plasma and cerebrospinal fluid beta‐synuclein for sporadic Creutzfeldt–Jakob disease

open access: yesAnnals of Clinical and Translational Neurology, 2023
Beta‐synuclein is a promising cerebrospinal fluid and blood biomarker of synaptic damage. Here we analysed its accuracy in the discrimination between sporadic Creutzfeldt–Jakob disease (n = 150) and non‐prion rapidly progressive dementias (n = 106).
Samir Abu‐Rumeileh   +11 more
doaj   +1 more source

Interlaboratory validation of cerebrospinal fluid α‐synuclein quantification in the diagnosis of sporadic Creutzfeldt‐Jakob disease

open access: yesAlzheimer’s & Dementia: Diagnosis, Assessment & Disease Monitoring, 2018
Introduction Cerebrospinal fluid α‐synuclein level is increased in sporadic Creutzfeldt‐Jakob disease cases. However, the clinical value of this biomarker remains to be established.
Niels Kruse   +12 more
doaj   +1 more source

Ring trial of 2nd generation RT‐QuIC diagnostic tests for sporadic CJD

open access: yesAnnals of Clinical and Translational Neurology, 2020
Objective Real‐time quaking‐induced conversion (RT‐QuIC) assays detect prion‐seeding activity in a variety of human biospecimens, including cerebrospinal fluid and olfactory mucosa swabs.
Christina D. Orrú   +21 more
doaj   +1 more source

Creutzfeldt–Jakob disease: A case report and differential diagnoses

open access: yesClinical Case Reports, 2022
Although sporadic Creutzfeldt–Jakob disease is a rare neurodegenerative disease and often difficult to diagnose at the earliest onset, meticulous clinical examination, electroencephalography, and neuroimaging findings will help in diagnosis.
Akash Raut   +5 more
doaj   +1 more source

Public health concerns regarding sporadic Creutzfeldt–Jakob disease in China: a case series

open access: yesJournal of International Medical Research, 2019
In this study, we report three cases of sporadic Creutzfeldt–Jakob disease in China, two confirmed cases and one probable case. The aim of this study was to enrich the data regarding clinical and epidemiological features of this disease and to provide ...
Ru-Yi Zhou   +12 more
doaj   +1 more source

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