Sporadic Creutzfeldt–Jakob disease

Meissner, Bettina; Westner, Ingo M.; Kallenberg, Kai; Krasnianski, Anna; Bartl, Michael; Varges, Daniela; Bösenberg, C.; Kretzschmar, Hans A.; Knauth, Michael; Schulz‐Schaeffer, Walter; Zerr, Inga

doi:10.1212/01.wnl.0000184674.32924.c9

Cited by 76 publications

(41 citation statements)

References 25 publications

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“…Further- more, a more sensitive method for early diagnosis of sCJD is needed because clinical diagnosis is sometimes diffi cult, particularly in atypical sCJD cases, such as MM2, MV2, VV1, or VV2 types (20)(21)(22)(23), according to 6 phenotypes of sCJD divided by codon 129 polymorphisms of PrP (methionine/valine) and type of infectious PrP by Western blotting (24). Even neurologists may misdiagnose the initial stage of the atypical sCJD cases as being another neurodegenrative disease such as Alzheimer disease and progressive supranuclear palsy (20).…”

Section: Discussionmentioning

confidence: 99%

Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999–2008

Hamaguchi¹,

Noguchi‐Shinohara²,

Nozaki³

et al. 2009

Emerg. Infect. Dis.

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Section: Discussionmentioning

confidence: 99%

Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999–2008

Hamaguchi¹,

Noguchi‐Shinohara²,

Nozaki³

et al. 2009

Emerg. Infect. Dis.

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“…A prominent, slowly progressive frontotemporal dementia with psychiatric changes is described; MRI shows hyperintensity in the cortex without much basal ganglia involvement, EEG does not show PSWCs, but the test for 14-3-3 protein is almost always positive. 36,42 clinical signs…”

Section: Molecular Strainsmentioning

confidence: 99%

Creutzfeldt-Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy

Manix

Kalakoti

Henry

et al. 2015

FOC

157

160

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Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative condition with a rapid disease course and a mortality rate of 100%. Several forms of the disease have been described, and the most common is the sporadic type. The most challenging aspect of this disease is its diagnosis—the gold standard for definitive diagnosis is considered to be histopatho-logical confirmation—but newer tests are providing means for an antemortem diagnosis in ways less invasive than brain biopsy. Imaging studies, electroencephalography, and biomarkers are used in conjunction with the clinical picture to try to make the diagnosis of CJD without brain tissue samples, and all of these are reviewed in this article. The current diagnostic criteria are limited; test sensitivity and specificity varies with the genetics of the disease as well as the clinical stage. Physicians may be unsure of all diagnostic testing available, and may order outdated tests or prematurely request a brain biopsy when the diagnostic workup is incomplete. The authors review CJD, discuss the role of brain biopsy in this patient population, provide a diagnostic pathway for the patient presenting with rapidly progressive dementia, and propose newer diagnostic criteria.

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“…For VV1, basal ganglia hyperintensities are rare, 15 and in MM2-cortical, isolated cerebral cortex involvement with limited basal ganglia involvement is characteristic, although normal MRI scans have been reported. 12,13 In our study, 10 patients were classified as MM2-cortical, and widespread cortical signal increase represented the main characteristic.…”

Section: Crude Analysis and Cluster Analysis Of The Raw Datamentioning

confidence: 99%