Results 21 to 30 of about 13,613 (255)

Enhanced Sensitivity of a Modified Quaking-Induced Conversion Diagnostic Test for the Broad Detection of Sporadic and Inherited Prion Diseases: A Retrospective Study. [PDF]

Ann Neurol
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 ...
Myskiw J, Fox R, Kielich DMS, Lamoureux L, Leonhardt M, Nykvist O, Slota JA, Avery K, Phillipson C, Frost K, Simon S, Appleby BS, Bailey-Elkin BA, Booth SA.   +13 more
europepmc   +2 more sources

Systematic review of pharmacological management in Creutzfeldt-Jakob disease: no options so far?

Arquivos de Neuro-Psiquiatria, 2022
Background The Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy that manifests as a rapidly progressive dementia syndrome. Currently, CJD has no cure, and many patients die within the first year, but some drugs are being studied ...
Luiz Henrique Lélis Miranda, André Felipe Pastick de Holanda Oliveira, Davi Mariano de Carvalho, Gustavo Marques Figueredo Souza, João Guilherme Monteiro Magalhães, Joselmo Alberto Cabral Júnior, Pedro Thadeu Mertens Brainer de Queiroz Lima, Renato Melo Aguiar Júnior, Sérgio Pereira Lins Filho, Hugo Moura de Albuquerque Melo   +9 more
doaj   +1 more source

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

Dementia & 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, Renata de Oliveira Carvalho, Raul Raposo Pereira Feitosa, Flávia de Paiva Santos Rolim, Felipe Araújo Rocha, Milena Sales Pitombeira, George Linard Silva Malveira, João José Freitas de Carvalho, Norberto Anizio Ferreira Frota, Daniel Aguiar Dias   +9 more
doaj   +1 more source

Do Severe Acute Respiratory Syndrome Coronavirus 2 Vaccines Change Creutzfeldt-Jakob Disease Prognosis?

Balkan Medical Journal, 2022
Yüce Doğru, Sezgin Kehaya
doaj   +2 more sources

Bilateral hearing loss as an initial presentation of Creutzfeldt-Jakob disease

Dementia & Neuropsychologia, 2021
. We reported a case of a 61-year-old male patient with anacusis, cerebellar syndrome, myoclonus, and frontal signs. The brain magnetic resonance imaging showed bilateral striated hyperintensity of the fluid-attenuated inversion recovery and restricted ...
Janaina Mariana de Araujo Miranda Brito-Marques, Eduardo Sousa de Melo, Fabíola Lys de Medeiros, Cristiano Sobral de Carvalho, Paulo Roberto de Brito-Marques   +4 more
doaj   +1 more source

The Phenotypic Spectrum of Sporadic Creutzfeldt-Jakob Disease Cortical Subtype. [PDF]

Ann Neurol
Objective The objective of this study was to characterize the phenotypic spectrum of the rare sporadic Creutzfeldt‐Jakob disease cortical subtype (sCJDMM/MV2C) in a large multicentric autopsy cohort. Methods We evaluated clinical histories, biofluid markers, brain diffusion‐weighted (DW)‐magnetic resonance imaging (MRI), and electroencephalogram (EEG ...
Baiardi S, Vargiu CM, Appleby BS, Barria M, Bentivenga GM, Calì I, Carlà B, Cohen M, Giese A, Herms J, Kortelainen AM, Ladogana A, Mammana A, Ritchie D, Windl O, Capellari S, Parchi P.   +16 more
europepmc   +2 more sources

Epidemiology of progressive intellectual and neurological deterioration in UK children. [PDF]

Dev Med Child Neurol
This study of PIND in UK children was carried out via the British Paediatric Surveillance Unit from 1997 to 2024. It identified six cases of vCJD. 2367 children had other diagnoses explain their deterioration. There were 259 other diseases in the diagnosed group.
Verity CM, Maunder PJ, Winstone AM, Pal S.   +3 more
europepmc   +2 more sources

Creutzfeldt-Jakob disease: literature review based on three case reports

Dementia & Neuropsychologia, 2022
. Creutzfeldt-Jakob disease (CJD) is one of the transmissible spongiform encephalopathies that lead to rapidly progressive dementia. CJD has a low prevalence, and the average survival is only 1 year after the onset of symptoms.
Amandha Alencar Maia Carneiro, Mateus Aragão Esmeraldo, David Elison de Lima e Silva, Espártaco Moraes Lima Ribeiro   +3 more
doaj   +1 more source

Application value of real ⁃ time quaking ⁃ induced conversion technology in clinical diagnosis of Creutzfeldt⁃Jakob disease

Chinese Journal of Contemporary Neurology and Neurosurgery, 2023
Creutzfeldt⁃Jakob disease (CJD) is a group of clinically rare neurodegenerative diseases caused by misfolding of prion proteins, and is the most common type of human prion diseases.
Dian HE
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

Frontiers 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, Vito A. G. Ricigliano, Michele Cavalli, Alessandro Cagol, Giovanna Bosco, Fabio Moda, Paola Caroppo, Giovanni Meola, Giovanni Meola   +8 more
doaj   +1 more source