Results 31 to 40 of about 5,376 (165)

Long survival sporadic Creutzfeldt-Jakob disease [PDF]

Neurology, 2020
A 63-year-old woman had slowly progressive dementia. EEG displayed periodic sharp wave complexes. MRI showed cortical ribbon pattern in cortex and hyperintensity in basal ganglia (figure, A–H). Genetics testing confirmed homozygosity for methionine at the polymorphic codon 129 of the PRNP gene.
Xiao-Ying Liu, Qi Shi, Gang Wang
openaire   +2 more sources

Sporadic Creutzfeldt-Jakob disease

Neurology, 2012
Sporadic Creutzfeldt-Jakob disease (sCJD) comprises approximately 85% of human prion diseases and is considered due to a post-translational modification of the native prion protein. Definitive diagnosis is only possible through neuropathologic examination at postmortem analysis or via an antemortem brain biopsy. Accurate and early clinical diagnosis of
Appleby, Brian S., Zerr, Inga
openaire   +4 more sources

Creutzfeldt–Jakob disease: A case report and differential diagnoses

Clinical 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, Anjila Thapa, Ashish Shrestha, Kamal Saud, Reema Rajbhandari, Shailendra Katwal   +5 more
doaj   +1 more source

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

Annals 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, Bradley R. Groveman, Aaron Foutz, Matilde Bongianni, Franco Cardone, Neil McKenzie, Audrey Culeux, Anna Poleggi, Katarina Grznarova, Daniela Perra, Michele Fiorini, Xiaoqin Liu, Anna Ladogana, Marco Sbriccoli, Andrew G. Hughson, Stéphane Haïk, Alison J. Green, Michael D. Geschwind, Maurizio Pocchiari, Jiri G. Safar, Gianluigi Zanusso, Byron Caughey   +21 more
doaj   +1 more source

Pathological spectrum of sporadic Creutzfeldt–Jakob disease [PDF]

Pathology
Human prion diseases are a rare group of transmissible neurodegenerative conditions which are classified according to their aetiology as sporadic, genetic or acquired forms. Creutzfeldt-Jakob disease (CJD) is the most common form of human prion disease, with the sporadic form accounting for ∼85% of all reported cases.
Ritchie, Diane L, Smith, Colin; id_orcid 0000-0002-4507-5132   +1 more
openaire   +2 more sources

Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease

Emerging Infectious Diseases, 2014
We report the presence of infectivity in erythrocytes, leukocytes, and plasma of 1 person with variant Creutzfeldt-Jakob disease and in the plasma of 2 in 4 persons whose tests were positive for sporadic Creutzfeldt-Jakob disease.
Jean Yves Douet, Saima Zafar, Armand Perret-Liaudet, Caroline Lacroux, Séverine Lugan, Naima Aron, Herve Cassard, Claudia Ponto, Fabien Corbière, Juan Maria Torres, Inga Zerr, Olivier Andreoletti   +11 more
doaj   +1 more source

Sporadic Creutzfeldt–Jakob disease

, 2018
Sporadic Creutzfeldt-Jakob disease (CJD), the most common human prion disease, is generally regarded as a spontaneous neurodegenerative illness, arising either from a spontaneous PRNP somatic mutation or a stochastic PrP structural change. Alternatively, the possibility of an infection from animals or other source remains to be completely ruled out ...
Zerr, Inga, Parchi, Piero
openaire   +4 more sources

Sporadic Creutzfeldt-Jakob disease in Northern Tasmania

Journal of the Royal College of Physicians of Edinburgh, 2023
Creutzfeldt-Jakob disease is a rare and incurable form of rapidly progressive neurodegenerative disease. The disease is fatal, and most patients die within 1 year of diagnosis. Clinical features include progressive cognitive dysfunction, delusions or hallucinations, cerebellar ataxia, myoclonus, visual disturbances, extrapyramidal signs and eventually ...
Priyanka Rajalingam, Aaron de Souza, Matthew Lee-Archer, Mahesh Dhakal   +3 more
openaire   +2 more sources

Young-onset sporadic Creutzfeldt–Jakob disease with atypical phenotypic features: a case report

Journal of Medical Case Reports, 2019
Background Sporadic Creutzfeldt–Jakob disease, with a mean survival of 6 months, is duly considered among the most fatal neurological disorders. Rapidly progressive dementia with multi-axial involvement of the nervous system is the known presentation ...
Durjoy Lahiri, Srimant Pattnaik, Ashwani Bhat, Souvik Dubey, Atanu Biswas, Biman Kanti Roy   +5 more
doaj   +1 more source

Applicability of long-term electroencephalography in pre-mortem diagnosis of Creutzfeldt–Jakob disease: A case report

SAGE Open Medical Case Reports, 2017
Creutzfeldt–Jakob disease accounts for more than 90% of all sporadic prion disease cases. The molecular MM2 genotype has been divided into cortical and thalamic subtypes based on structures involved and is characterized clinically by progressive dementia
Sanaz Attaripour Isfahani, Michelle Dougherty, Gediminas Peter Gliebus   +2 more
doaj   +1 more source