In this article we review the concept and terminology of prions, their replication and some current hypothesis on the nature of these infectious agents causing neurodegenerative diseases. This revision also summarizes the etiopathogenic, epidemiological, clinical and neuropathological features of the prion diseases or human transmissible spongiform encephalopathies, and some methods for their early diagnosis. Finally, we discuss the possible link between the bovine spongiform encephalopathy and the new cases of Creutzfeldt-Jakob disease identified in the United Kingdom.
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Mood Alterations in the Prodromal Phase of Sporadic Creutzfeldt-Jakob Disease.
JAMA Neurol
December 2024
Department of Neurology, Medical University of Vienna, Vienna, Austria.
Importance: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, rapidly progressive and fatal neurodegenerative disease. Definite sCJD diagnosis can only be made post mortem, and little is known about the prodromal phase of the disease.
Objective: To compare drug prescription patterns before the clinical onset of sCJD between patients and matched controls for exploration of potential risk factors and to assess correlations between drug exposure and sCJD survival.
Interpretable deep learning survival predictions in sporadic Creutzfeldt-Jakob disease.
J Neurol
December 2024
The UK National CJD Research and Surveillance Unit, Centre for Clinical Brain Sciences, Chancellor's Building, University of Edinburgh, Edinburgh, EH16 4TG, UK.
Background: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive and fatal prion disease with significant public health implications. Survival is heterogenous, posing challenges for prognostication and care planning. We developed a survival model using diagnostic data from comprehensive UK sCJD surveillance.
View Article and Find Full Text PDFPathological spectrum of sporadic Creutzfeldt-Jakob disease.
Pathology
November 2024
National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, Chancellor's Building, University of Edinburgh, Edinburgh BioQuarter, Edinburgh, United Kingdom; Academic Department of Neuropathology, Centre for Clinical Brain Sciences, Chancellor's Building, University of Edinburgh, Edinburgh BioQuarter, Edinburgh, United Kingdom.
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. While advances have been made in the development of clinical tools and biomarkers in the diagnosis of prion disease, allowing greater diagnostic certainty for surveillance purposes, definitive diagnosis requires neuropathological examination of the brain at postmortem.
View Article and Find Full Text PDFBiological activities of astaxanthin in the treatment of neurodegenerative diseases.
Neurodegener Dis Manag
January 2025
Fertility and Infertility Research Center, Health Technology Research Institute, Kermanshah University of Medical Sciences, Kermanshah, Iran.
Introduction: Neurodegenerative diseases (NDs) develop with the gradual advancement of neuronal damage and dysfunction in the central nervous system (CNS). These disorders are mostly the outcomes of the improper sedimentation and accumulation of proteins, such as amyloid-β (Aβ), α-synuclein, and prions. Astaxanthin (AST) exists in different types of living organisms and displays antioxidant and anti-inflammatory functions.
View Article and Find Full Text PDFA case report of an individual with Creutzfeldt-Jakob disease characterized by prolonged isolated thalamic lesions and rare MM2-cortical-type pathology.
BMC Neurol
November 2024
Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Kanazawa-ku, Yokohama, 236-0004, Japan.
Background: Diffusion-weighted magnetic resonance imaging (DWI) is essential for diagnosing Creutzfeldt-Jakob disease (CJD). Thalamic lesions are rarely detected by DWI in sporadic CJD (sCJD) cases with methionine homozygosity at polymorphic codon 129 (129MM) of the prion protein (PrP) gene. Here, we describe an unusual sCJD case, characterized by prolonged isolated thalamic diffusion hyperintensities and atypical brain pathology, in combination with the 129MM genotype.
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