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Journal ArticleDOI

Analysis of EEG and CSF 14-3-3 proteins as aids to the diagnosis of Creutzfeldt-Jakob disease.

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TLDR
In this paper, a comparison of clinical and neuropathologic diagnoses and evaluation of the sensitivity and specificity of EEG and 14-3-3 CSF immunoassay were conducted.
Abstract
Objective: To improve diagnostic criteria for sporadic Creutzfeldt–Jakob disease (CJD). Methods: Pooled data on initial and final diagnostic classification of suspected CJD patients were accumulated, including results of investigations derived from a coordinated multinational study of CJD. Prospective analysis for a comparison of clinical and neuropathologic diagnoses and evaluation of the sensitivity and specificity of EEG and 14-3-3 CSF immunoassay were conducted. Results: Data on 1,003 patients with suspected CJD were collected using a standard questionnaire. After follow-up was carried out, complete clinical data and neuropathologic diagnoses were available in 805 cases. In these patients, the sensitivity of the detection of periodic sharp wave complexes in the EEG was 66%, with a specificity of 74%. The detection of 14-3-3 proteins in the CSF correlated with the clinical diagnosis in 94% (sensitivity). The specificity (84%) was higher than that of EEG. A combination of both investigations further increased the sensitivity but decreased the specificity. Conclusions: Incorporation of CSF 14-3-3 analysis in the diagnostic criteria for CJD significantly increases the sensitivity of case definition. Amended diagnostic criteria for CJD are proposed.

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Recommendations for the diagnosis and management of Alzheimer's disease and other disorders associated with dementia: EFNS guideline.

TL;DR: This international guideline on dementia covers major aspects of diagnostic evaluation and treatment, with particular emphasis on the type of patient often referred to the specialist physician, and revised the recommendations for clinical diagnosis, blood tests, neuroimaging, electroencephalography, and cerebrospinal fluid analysis.
Journal ArticleDOI

Sporadic and familial CJD: classification and characterisation

TL;DR: This review attempts to classify and characterise sporadic and familial Creutzfeldt-Jakob disease (CJD) as a function of these two disease determinants: PrP genotype and PrP(Sc) type.
Journal ArticleDOI

Iatrogenic Creutzfeldt-Jakob disease at the millennium.

TL;DR: Knowledge about potential high-risk sources of contamination gained during the last quarter century, and the implementation of methods to circumvent them, should minimize the potential for iatrogenic contributions to the current spectrum of CJD.
References
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Journal ArticleDOI

Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease

TL;DR: Incubation periods as well as the durations and character of illness showed great variability, even in animals receiving the same inoculum, mirroring the spectrum of clinical profiles seen in human disease.
Journal ArticleDOI

Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples

TL;DR: If, in the appropriate clinical context, a tonsil biopsy sample was positive for PrPSc, variant CJD could be diagnosed, which obviates the need for a brainBiopsy sample to be taken, and the results show that variants CJD has a different pathogenesis to sporadic CJD.
Journal ArticleDOI

The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies.

TL;DR: In patients with dementia, a positive immunoassay for the 14-3-3 brain protein in cerebrospinal fluid strongly supports a diagnosis of Creutzfeldt-Jakob disease, but this finding does not support the use of the test in patients without clinically evident dementia.
Journal ArticleDOI

Creutzfeldt‐Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering

TL;DR: The worldwide epidemiology of Creutzfeldt‐Jakob disease (CJD) is presented from an analysis of 1,435 patients, and fifteen percent of the cases were of the familial type, suggesting a genetic susceptibility to infection.
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