Impact of the clinical context on the 14-3-3 test for the diagnosis of sporadic CJD

Cuadrado-Corrales, Natividad; Jiménez‐Huete, Adolfo; Albó, Carmen; Hortigüela, Rafael; Vega, Luz de la Sierra-de la; Cerrato, Laura; Sierra-Moros, Maríajosé; Rábano, Alberto; Pedro‐Cuesta, Jesús de; Calero, Miguel

doi:10.1186/1471-2377-6-25

Cited by 30 publications

(23 citation statements)

References 14 publications

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“…The disease related family history is notified in five cases, including the recently reported four German cases belonging to two different families 12 and the first French case whose mother and brother died with dementia and motor impairment without definite diagnosis. 9 Other six cases seem lacking of definite family history. [13][14][15] Onset ages of E196K gCJD range from 64-80 year-old (median: 71 year-old), which are obviously later than that of most other gCJD cases.…”

Section: Discussionmentioning

confidence: 99%

“…CSF 14-3-3 protein was detected with western blot according to the protocol described elsewhere with minor modifications. 9 Briefly, 20 μl CSF sample was separated by 12% SDS-PAGE and electronically transformed onto nitrocellulose membrane. Blots were incubated in 1:1,000 diluted 14-3-3 polyclonal antibodies (Santa Cruz, CA) and further incubated in 1:5,000 diluted HRP-conjugated goat antiRabbit IgG (PerkinElmer, Germany).…”

Section: Discussionmentioning

confidence: 99%

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A Chinese Creutzfeldt-Jakob disease patient with E196K mutation inPRNP

Shi

Chen

Song

et al. 2011

Prion

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A Chinese Creutzfeldt-Jakob disease patient with E196K mutation inPRNP

Shi

Chen

Song

et al. 2011

Prion

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“…However, the amounts of tau isoforms with exon-2 and exon-10 segments in CSF correlate well with positive CSF protein 14-3-3 in the patients of sCJD. 14-3-3 positive in CSF is usually believed as a result of neuronal damage or death [29]. Protein 14-3-3 positive in CSF can be observed in a series of acute brain damage, such as acute cerebral infarction, viral encephalitis, etc.…”

Section: Discussionmentioning

confidence: 99%

The Levels of Tau Isoforms Containing Exon-2 and Exon-10 Segments Increased in the Cerebrospinal Fluids of the Patients with Sporadic Creutzfeldt-Jakob Disease

Chen

Zhou

et al. 2015

Mol Neurobiol

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The alteration of protein tau in the cerebrospinal fluid (CSF) of Creutzfeldt-Jakob disease (CJD) has been widely evaluated, possessing a significant diagnostic value for CJD. With the biotin-labeled tau-exon-specific mAbs, direct ELISA methods were established and the levels of tau isoforms containing exon-2 and exon-10 segments in CSF of the patients with various human prion diseases and in brain tissues of scrapie-infected animals were evaluated. The results showed that the levels of tau, especially containing four repeats in microtubule binding domain, were increased in the CSF samples of the patients with sporadic CJD (sCJD). Using the unlabeled (cold) mixed exon-specific mAbs, a competitive tau ELISA was conducted based on a commercial tau kit. It revealed that the majority of the increased tau in the CSF of sCJD cases was derived from the tau isoforms with exon-2 and exon-10 segments. Increases of CSF tau isoforms with exon-2 and exon-10 segments were also observed in the patients of E200K and T188K genetic CJD (gCJD), but not in the cases of fatal familiar insomnia (FFI). The increasing levels of tau isoforms with exon-2 and exon-10 segments in the group of sCJD correlated well with the positive 14-3-3 in CSF. Additionally, the similar alterative profiles of tau isoforms with exon-2 and exon-10 segments were also observed in the brain tissues of scrapie-infected rodents and a sCJD patient. Our data here propose the tau isoforms with exon-2 and exon-10 segments increase in CSF of sCJD and some types of gCJD, which may help to understand the physiological metabolism and pathological significance of various tau isoforms in the pathogenesis of prion diseases.

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“…He developed diffuse spontaneous myoclonus and became increasingly drowsy, then comatose and then died. Autopsy was refused; however, in this setting, the diagnostic certainty of CJD is greater than 90% 9 …”

Section: Casementioning

confidence: 99%

“…Useful clinical tests include electroencephalographic (EEG) indication of periodic sharp‐wave complexes and increased cerebrospinal fluid (CSF) levels of 14‐3‐3 protein, but neither test is specific for CJD 8 . Increased 14‐3‐3 protein levels have a high positive predictive value when clinical suspicion is strong, but are less accurate with decreased pretest diagnostic certainty 9 . Brain biopsy involves risks to both patient and neurosurgeon with the added risk of false‐negative results due to sampling error 10 …”

Section: Introductionmentioning

confidence: 99%

MRI of sporadic Creutzfeldt–Jakob disease

et al. 2008

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The key MRI findings in five cases of sporadic Creutzfeldt-Jakob disease (CJD) are illustrated with four 'definite' and one 'probable' according to World Health Organization criteria. Close attention to fluid-attenuation inversion recovery and diffusion-weighted imaging sequences are important for diagnosis, noting especially restricted diffusion in cortical and deep grey matter. Our study and those of others show predominant cortical, caudate and thalamic involvement. This pattern is highly sensitive and specific for the diagnosis. Fluid-attenuation inversion recovery and diffusion-weighted imaging signal abnormality becomes progressively more extensive and bilateral as disease progresses, but may become less pronounced in end-stage disease because of atrophy.

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Impact of the clinical context on the 14-3-3 test for the diagnosis of sporadic CJD

Cited by 30 publications

References 14 publications

A Chinese Creutzfeldt-Jakob disease patient with E196K mutation inPRNP

A Chinese Creutzfeldt-Jakob disease patient with E196K mutation inPRNP

The Levels of Tau Isoforms Containing Exon-2 and Exon-10 Segments Increased in the Cerebrospinal Fluids of the Patients with Sporadic Creutzfeldt-Jakob Disease

MRI of sporadic Creutzfeldt–Jakob disease

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