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Creutzfeldt-Jakob Disease (and Other Prion Diseases)
Published in Alexander R. Toftness, Incredible Consequences of Brain Injury, 2023
There's also a type of CJD that comes from an entirely different source but causes similar symptoms, which is aptly named variant Creutzfeldt-Jakob disease (vCJD). vCJD is transmitted to humans through the consumption of products contaminated with bovine spongiform encephalopathy, which mostly includes beef products such as hamburgers. The disease is better known as mad cow disease. This makes vCJD a zoonotic disease, meaning that it can jump the species barrier to infect a different animal's brain (Comoy et al., 2015). The resulting disease does not occur immediately. Instead, the person develops vCJD years after they were exposed to the infectious prion. The amount of time that it takes to develop vCJD after being infected depends upon the person's genetics (Scheckel & Aguzzi, 2018).
Hyperkinetic Movement Disorders
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Morales-Briceno Hugo, Victor S.C. Fung, Annu Aggarwal, Philip Thompson
Infective: HIV.Variant Creutzfeldt–Jakob disease (vCJD).Tuberculosis.Toxoplasmosis.SSPE.
Transfusion products
Published in Jennifer Duguid, Lawrence Tim Goodnough, Michael J. Desmond, Transfusion Medicine in Practice, 2020
Several countries, including Canada, France, Ireland, Portugal, and the UK, have implemented universal leukocyte depletion of blood components, and it is likely that others will follow. The impetus in the UK and Ireland was the theoretical risk that variant Creutzfeldt–Jakob disease (vCJD) might be transmissible by blood, and in particular by the leukocytes.19 However, the benefit of leukocyte depletion in reducing the infectivity of blood from individuals with vCJD is unknown, since the distribution of PrPsc (the insoluble form of cellular prion protein) remains uncertain. PrPc (the normal cellular prion protein) is also known to be expressed in higher quantities on platelets than on white cells or red cells.20
In vitro-derived platelets: the challenges we will have to face to assess quality and safety
Published in Platelets, 2020
S. Mookerjee, H. R. Foster, A. K Waller, C. J. Ghevaert
The use of blood donor-derived products has also been marred in recent years by incidents of infected components, particularly for components where several donations are pooled such as clotting factor concentrates. The pooling of 4 donations to make one unit of platelets remains one of the main production methods although the use of apheresis to provide single-donor platelets has markedly increased in the last two decades following the UK Bovine Spongiform Encephalopathy outbreak and the fear of transmission of variant Creutzfeldt-Jakob disease through blood transfusion (now 60–80% of all units).
An interesting case of functional visual loss presenting as a left homonymous hemianopia
Published in Clinical and Experimental Optometry, 2021
The differential diagnosis for a homonymous hemianopia with a normal magnetic resonance imaging and electroencephalogram is quite limited.7 Organic conditions to consider include hypoxic brain injury, Heidenhain variant Creutzfeldt–Jakob disease, posterior reversible encephalopathy, and posterior cortical atrophy.7 In all of these cases, the magnetic resonance imaging is abnormal but may be misinterpreted. Post-migraine and post-ictal (Todd’s paralysis) cases are usually transient. The other possibility is non-organic or functional visual loss.
3-Nitrotyrosine: a versatile oxidative stress biomarker for major neurodegenerative diseases
Published in International Journal of Neuroscience, 2020
Maria Bandookwala, Pinaki Sengupta
Human prion diseases or transmissible spongiform encephalopathies (TSEs) is a rare condition originating either spontaneously, due to genetically altered proteome, cellular stress response or by infection [69]. It is a group of fatal neurodegenerative diseases with approximately 1 death in a million people annually according to worldwide epidemiological data published by WHO. It includes transmissible and progressive degenerative disorders of the CNS like Kuru, fatal familial insomnia, variably protease-sensitive prionopathy (VPSPr), Gerstmann–Sträussler–Scheinker syndrome and sporadic and variant Creutzfeldt-Jakob disease among which sporadic CJD is the most common form [70]. Rapidly developing dementia, confusion, hallucinations, muscle stiffness, difficulty in speaking and shuffling gait are the symptoms affecting the quality of life of people. A clinical study by Chen et al. [71] in China revealed that patient usually dies on an average of 7.1 months after disease which coincided with the reports from epidemiological studies in the west. Damage to the nerve cells is central to the manifestation of prion disease typically characterized by the accumulation of mutated prion protein PrPSc. OS has long been recognized as a molecular and cellular mechanism as a driving force in the pathophysiology of prion disease. In vivo immunohistochemical studies by D. R. Brown in scrapie-infected mice model backed with supporting evidence validate the role of OS via confirmation of the presence of OS biomarkers [72]. Brown also conducted in vitro studies, and similar results were obtained from scrapie-infected cells where reduced levels of antioxidants were observed making the cells more vulnerable to the damage by free radicals [72]. Protein profiling experiments have revealed expressional alteration of DJ-1, an antioxidant protein and its nuclear translocalization due to OS in sCJD. Tahir et al. [73] observed a significantly higher amount of DJ-1 in the CSF of clinical sCJD patients. The same research group also detected DJ-1 upregulation in humanized PrP transgenic mice model at the pre-symptomatic and symptomatic stages indicative of OS [73]. Global metabolic profiling conducted in the hippocampus and cortex of prion protein-infected mice by Bourgognon et al. [74]showed alteration in glucose metabolism, upregulation of NO signalling, downregulation of SOD1 and SOD2 antioxidant genes which contribute to oxidative and nitrosative stress. Figure 5 shows the OS mechanism in TSE.