The Role of PET Imaging in Patients with Prion Disease: A Literature Review
AbstractPrion diseases are rare, rapidly progressive, and fatal incurable degenerative brain disorders caused by the misfolding of a normal protein called PrPC into an abnormal protein called PrPSc. Their highly variable clinical presentation mimics various degenerative and non-degenerative brain disorders, making diagnosis a significant challenge for neurologists. Currently, definitive diagnosis relies on post-mortem examination of nervous tissue to detect the pathogenic prion protein. The current diagnostic criteria are limited. While structural magnetic resonance imaging (MRI) remains the gold standard imaging modality ...
Source: Molecular Imaging and Biology - February 1, 2024 Category: Molecular Biology Source Type: research
Alzheimer ’s disease may have been transmitted in now-banned hormone treatments
Alzheimer’s disease can spread via brain tissue from one person to another, according to a new study of several people in the United Kingdom who, as children, were prescribed a now-abandoned hormone therapy treatment. All received years of injections of human growth hormone extracted from pituitary glands in the brains of cadavers. The approach was halted in the mid-1980s after alarming evidence that it could transmit another fatal brain disease.
The researchers couldn’t prove the treatment caused Alzheimer’s, although mouse studies have shown that transplanting Alzheimer’s-affected brain tissue can recreate ...
Source: Science of Aging Knowledge Environment - January 29, 2024 Category: Geriatrics Source Type: research
Large-scale validation of skin prion seeding activity as a biomarker for diagnosis of prion diseases
AbstractDefinitive diagnosis of sporadic Creutzfeldt –Jakob disease (sCJD) relies on the examination of brain tissues for the pathological prion protein (PrPSc). Our previous study revealed that PrPSc-seeding activity (PrPSc-SA) is detectable in skin of sCJD patients by an ultrasensitive PrPSc seed amplification assay (PrPSc-SAA) known as real-time quaking-induced conversion (RT-QuIC). A total of 875 skin samples were collected from 2 cohorts (1 and 2) at autopsy from 2 –3 body areas of 339 cases with neuropathologically confirmed prion diseases and non-sCJD controls. The skin samples were analyzed for PrPSc-SA by RT-Q...
Source: Acta Neuropathologica - January 17, 2024 Category: Neurology Source Type: research
Behavioral sciences applied to acute care teams: a research agenda for the years ahead by a European research network
Multi-disciplinary behavioral research on acute care teams has focused on understanding how teams work and on identifying behaviors characteristic of efficient and effective team performance. We aimed to defin... (Source: BMC Health Services Research)
Source: BMC Health Services Research - January 13, 2024 Category: General Medicine Authors: Sandra Keller, Judith G. M. Jelsma, Franziska Tschan, Nick Sevdalis, Ruth M. L öllgen, Johan Creutzfeldt, Lauren R. Kennedy-Metz, Walter Eppich, Norbert K. Semmer, Isabelle Van Herzeele, Karin Pukk Härenstam and Martine C. de Bruijne Tags: Research Source Type: research
Outcomes and experiences of patients and their caregivers after severe stroke requiring tube feeding in Peru
Evaluate clinical outcomes of stroke survivors in Peru discharged with artificial nutrition via a feeding tube (FT), and explore perspectives and experiences of these patients and their caregivers. (Source: Journal of Pain and Symptom Management)
Source: Journal of Pain and Symptom Management - January 10, 2024 Category: Palliative Care Authors: Nauzley C. Abedini, Orli M. Shulein, Sandra Berr ú-Villalobos, Jorge Ramírez-Quiñones, Claire J. Creutzfeldt, Jill Portocarrero, Joseph R. Zunt, Carlos Abanto-Argomedo Tags: Original Article Source Type: research
A review of the enhanced CJD surveillance feasibility study in the elderly in Scotland, UK
Variant Creutzfeldt - Jakob disease (vCJD) arose from dietary contamination with bovine-spongiform-encephalopathy (BSE). Because of concerns that vCJD-cases might be missed in the elderly, a feasibility study ... (Source: BMC Geriatrics)
Source: BMC Geriatrics - January 3, 2024 Category: Geriatrics Authors: Lovney Kanguru, Sarah Cudmore, Gemma Logan, Briony Waddell, Colin Smith, Anna Molesworth and Richard Knight Tags: Research Source Type: research
Punctate Diffuse Cortex Signals in Creutzfeldt-Jakob Disease
This case report describes 2 patients with genetic Creutzfeldt-Jakob disease with atypical changes on diffusion-weighted imaging. (Source: JAMA Neurology)
Source: JAMA Neurology - January 2, 2024 Category: Neurology Source Type: research
Pathological findings in autoimmune encephalitis autopsy specimens from cases of suspected prion disease
DiscussionSeronegative AE may have consistent pathology with diffuse or multifocal perivascular inflammation and microglial activation. Half the patients do not have neuronal loss suggesting a potential for neurological recovery. These findings are preliminary and require further confirmation. (Source: Journal of Neurology)
Source: Journal of Neurology - January 1, 2024 Category: Neurology Source Type: research
Pathology of neurodegenerative disease for the general neurologist
Pract Neurol. 2023 Dec 16:pn-2023-003988. doi: 10.1136/pn-2023-003988. Online ahead of print.ABSTRACTNeurodegeneration refers to progressive dysfunction or loss of selectively vulnerable neurones from brain and spinal cord regions. Despite important advances in fluid and imaging biomarkers, the definitive diagnosis of most neurodegenerative diseases still relies on neuropathological examination. Not only has careful clinicopathological correlation shaped current clinical diagnostic criteria and informed our understanding of the natural history of neurodegenerative diseases, but it has also identified conditions with import...
Source: Practical Neurology - December 21, 2023 Category: Neurology Authors: Patrick W Cullinane Sarah Wrigley Jacy Bezerra Parmera Fernanda Valerio Thomas O Millner Karen Shaw Eduardo De Pablo-Fernandez Thomas T Warner Zane Jaunmuktane Source Type: research
Pathology of neurodegenerative disease for the general neurologist
Pract Neurol. 2023 Dec 16:pn-2023-003988. doi: 10.1136/pn-2023-003988. Online ahead of print.ABSTRACTNeurodegeneration refers to progressive dysfunction or loss of selectively vulnerable neurones from brain and spinal cord regions. Despite important advances in fluid and imaging biomarkers, the definitive diagnosis of most neurodegenerative diseases still relies on neuropathological examination. Not only has careful clinicopathological correlation shaped current clinical diagnostic criteria and informed our understanding of the natural history of neurodegenerative diseases, but it has also identified conditions with import...
Source: Practical Neurology - December 21, 2023 Category: Neurology Authors: Patrick W Cullinane Sarah Wrigley Jacy Bezerra Parmera Fernanda Valerio Thomas O Millner Karen Shaw Eduardo De Pablo-Fernandez Thomas T Warner Zane Jaunmuktane Source Type: research
Pathology of neurodegenerative disease for the general neurologist
Pract Neurol. 2023 Dec 16:pn-2023-003988. doi: 10.1136/pn-2023-003988. Online ahead of print.ABSTRACTNeurodegeneration refers to progressive dysfunction or loss of selectively vulnerable neurones from brain and spinal cord regions. Despite important advances in fluid and imaging biomarkers, the definitive diagnosis of most neurodegenerative diseases still relies on neuropathological examination. Not only has careful clinicopathological correlation shaped current clinical diagnostic criteria and informed our understanding of the natural history of neurodegenerative diseases, but it has also identified conditions with import...
Source: Practical Neurology - December 21, 2023 Category: Neurology Authors: Patrick W Cullinane Sarah Wrigley Jacy Bezerra Parmera Fernanda Valerio Thomas O Millner Karen Shaw Eduardo De Pablo-Fernandez Thomas T Warner Zane Jaunmuktane Source Type: research
Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms
ConclusionWe observed a decrease of CJD infectious forms, demonstrating the effectiveness of measures to limit human exposure to exogenous prions. However, active surveillance is needed regarding uncertainties about future occurrences of vCJD, possible zoonotic potential of chronic wasting diseases in cervids and increasing trends of sCJD observed in France and other countries.PMID:38099349 | DOI:10.2807/1560-7917.ES.2023.28.50.2300101 (Source: Euro Surveill)
Source: Euro Surveill - December 15, 2023 Category: Infectious Diseases Authors: Ang éline Denouel Jean-Philippe Brandel Laur ène Peckeu-Abboud Danielle Seilhean Elodie Bouaziz-Amar Isabelle Quadrio Jean-Baptiste Oudart Sylvain Lehmann Pantxika Bellecave Jean-Louis Laplanche St éphane Haik Source Type: research
Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms
ConclusionWe observed a decrease of CJD infectious forms, demonstrating the effectiveness of measures to limit human exposure to exogenous prions. However, active surveillance is needed regarding uncertainties about future occurrences of vCJD, possible zoonotic potential of chronic wasting diseases in cervids and increasing trends of sCJD observed in France and other countries.PMID:38099349 | DOI:10.2807/1560-7917.ES.2023.28.50.2300101 (Source: Euro Surveill)
Source: Euro Surveill - December 15, 2023 Category: Infectious Diseases Authors: Ang éline Denouel Jean-Philippe Brandel Laur ène Peckeu-Abboud Danielle Seilhean Elodie Bouaziz-Amar Isabelle Quadrio Jean-Baptiste Oudart Sylvain Lehmann Pantxika Bellecave Jean-Louis Laplanche St éphane Haik Source Type: research
Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms
ConclusionWe observed a decrease of CJD infectious forms, demonstrating the effectiveness of measures to limit human exposure to exogenous prions. However, active surveillance is needed regarding uncertainties about future occurrences of vCJD, possible zoonotic potential of chronic wasting diseases in cervids and increasing trends of sCJD observed in France and other countries.PMID:38099349 | DOI:10.2807/1560-7917.ES.2023.28.50.2300101 (Source: Euro Surveill)
Source: Euro Surveill - December 15, 2023 Category: Infectious Diseases Authors: Ang éline Denouel Jean-Philippe Brandel Laur ène Peckeu-Abboud Danielle Seilhean Elodie Bouaziz-Amar Isabelle Quadrio Jean-Baptiste Oudart Sylvain Lehmann Pantxika Bellecave Jean-Louis Laplanche St éphane Haik Source Type: research
Prospective 25-year surveillance of prion diseases in France, 1992 to 2016: a slow waning of epidemics and an increase in observed sporadic forms
ConclusionWe observed a decrease of CJD infectious forms, demonstrating the effectiveness of measures to limit human exposure to exogenous prions. However, active surveillance is needed regarding uncertainties about future occurrences of vCJD, possible zoonotic potential of chronic wasting diseases in cervids and increasing trends of sCJD observed in France and other countries.PMID:38099349 | DOI:10.2807/1560-7917.ES.2023.28.50.2300101 (Source: Euro Surveill)
Source: Euro Surveill - December 15, 2023 Category: Infectious Diseases Authors: Ang éline Denouel Jean-Philippe Brandel Laur ène Peckeu-Abboud Danielle Seilhean Elodie Bouaziz-Amar Isabelle Quadrio Jean-Baptiste Oudart Sylvain Lehmann Pantxika Bellecave Jean-Louis Laplanche St éphane Haik Source Type: research
