Biomarkers for sporadic Creutzfeldt–Jakob disease

Soomro, Sanam; Mohan, Chandra

doi:10.1002/acn3.304

Cited by 9 publications

(9 citation statements)

References 55 publications

(69 reference statements)

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“…Эпидемия ТГЭ была взята под контроль после массивного убоя скота и изменений в технологии производства, которые резко сократили загрязнение мяса компонентами нервной ткани. В Великобритании ежегодное число новых случаев вБКЯ, которое достигло пика в 2000 г., неуклонно снижается, и в 2013 г. был подтвержден только 1 случай заболевания [15,32].…”

Section: клинические формы пзunclassified

“…Большинство случаев вБКЯ диагностировано в Великобритании, их число составило около 200 в 2015 г., по сравнению с примерно 60 случаями в других странах [15,32]. У всех пациентов вБКЯ развился после употребления в пищу мяса, полученного от крупного рогатого скота, заболевшего ТГЭ.…”

Section: клинические формы пзunclassified

“…инкубационный период составил 11-12 лет. В последних диагностированных случаях инкубационный период достигал от 12 до более 20 лет [32,33].…”

Section: клинические формы пзunclassified

“…Довольно быстро он теряет способность самообслуживания. Для вБКЯ типичны не только начало в более молодом возрасте, но и средняя выживаемость, превышающая 14 мес [15,32,33]. Вероятно, что различия в выживаемости отчасти связаны с возрастом пациентов.…”

Section: клинические формы пзunclassified

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Human prion diseases: current issues

Zavadenko

Khondkaryan

Bembeeva

et al. 2018

Z. nevrol. psikhiatr. im. S.S. Korsakova

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Prion diseases, or transmissible spongiform encephalopathies, are a group of neurodegenerative diseases with progressive dementia and movement disorders. There are three variants of prion diseases pathogenesis: direct contamination, genetic and sporadic forms. The following clinical forms are known: Creutzfeldt-Jakob disease (common type), variant Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker disease, variably protease-sensitive prionopathy, fatal insomnia and fatal familial insomnia, kuru, prion disease associated with diarrhea and autonomic neuropathy. Clinical characteristic of prion diseases, molecular-genetic aspects of their pathogenesis and current diagnostic approaches are discussed. Because of the lack of effective treatment, prevention of both alimentary prion infections (consumption of contaminated meat products) and transmissible iatrogenic infections (the use of biopreparations from animal tissues) is important. The safety of such biopreparations should be ensured by modern manufacturing technologies and specially developed procedures that meet international requirements and standards.

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Section: клинические формы пзunclassified

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Human prion diseases: current issues

Zavadenko

Khondkaryan

Bembeeva

et al. 2018

Z. nevrol. psikhiatr. im. S.S. Korsakova

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“…Indeed, diagnosis using CSF biomarkers has shown several benefits of reducing the diagnostic turn-around time with reproducible data without any risk of damage to the patient's brain. One of these candidate biomarkers for sCJD is the 14-3-3 protein, showing diagnostic sensitivities ranging from 61% to 96% and specificities ranging from 67% to 95% [11][12][13][14][15][16]. Moreover, quantification of the CSF protein α-synuclein (a-syn) using an in-house enhanced chemiluminesence-based enzyme-linked immunosorbent assay (ELISA) was recently reported to be an excellent approach for sCJD diagnosis [10,17].…”

Section: Introductionmentioning

confidence: 99%

Sensitivity and specificity evaluation of multiple neurodegenerative proteins for Creutzfeldt-Jakob disease diagnosis using a deep-learning approach

Lee

Hyeon

Kim

et al. 2019

Prion

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The diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) can only be confirmed by abnormal protease-resistant prion protein accumulation in post-mortem brain tissue. The relationships between sCJD and cerebrospinal fluid (CSF) proteins such as 14-3-3, tau, and α-synuclein (a-syn) have been investigated for their potential value in pre-mortem diagnosis. Recently, deeplearning (DL) methods have attracted attention in neurodegenerative disease research. We established DL-aided pre-mortem diagnostic methods for CJD using multiple CSF biomarkers to improve their discriminatory sensitivity and specificity. Enzyme-linked immunosorbent assays were performed on phospho-tau (p-tau), total-tau (t-tau), a-syn, and β-amyloid (1-42), and western blot analysis was performed for 14-3-3 protein from CSF samples of 49 sCJD and 256 non-CJD Korean patients, respectively. The deep neural network structure comprised one input, five hidden, and one output layers, with 20, 40, 30, 20 and 12 hidden unit numbers per hidden layer, respectively. The best performing DL model demonstrated 90.38% accuracy, 83.33% sensitivity, and 92.5% specificity for the three-protein combination of t-tau, p-tau, and a-syn, and all other patients in a separate CSF set (n = 15) with other neuronal diseases were correctly predicted to not have CJD. Thus, DL-aided pre-mortem diagnosis may provide a suitable tool for discriminating CJD patients from non-CJD patients.

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Rapidly Progressing Sporadic Creutzfeldt-Jakob Disease Presenting as a Stroke

et al. 2018

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Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, fatal human prion disease that is characterized by progressive dementia and neurologic degeneration. It can mimic multiple other neurological disorders, and a high index of clinical suspicion is necessary to make a diagnosis. A 74-year-old woman with a 3-month history of a stroke and progressive neurologic deterioration was found to have sCJD. She expired within a week of her diagnosis. Autopsy revealed spongiform encephalopathy consistent with prion disease, and genetic analysis revealed 129 polymorphism and no pathologic mutation, confirming the diagnosis of nonfamilial human prion disease. No pathologic evidence of a stroke was found. Awareness of the disease by clinicians is important not only at the time of initial presentation but also during the following months. Since there is no treatment, invasive medical procedures should be limited to only those that are required for either diagnosis or hospice care.

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Biomarkers for sporadic Creutzfeldt–Jakob disease

Cited by 9 publications

References 55 publications

Human prion diseases: current issues

Human prion diseases: current issues

Sensitivity and specificity evaluation of multiple neurodegenerative proteins for Creutzfeldt-Jakob disease diagnosis using a deep-learning approach

Rapidly Progressing Sporadic Creutzfeldt-Jakob Disease Presenting as a Stroke

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