Diffusion-Weighted Magnetic Resonance Imaging in Probable Creutzfeldt-Jakob Disease

Na, Duk L.; Suh, Chung Kyu; Choi, Seong Hye; Moon, Heui Soo; Seo, Dae Won; Kim, Sang Eun; Na, Dong Gyu; Adair, John C.

doi:10.1001/archneur.56.8.951

Cited by 80 publications

(51 citation statements)

References 31 publications

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“…In contrast, DWI abnormalities are most probably caused by spongiform degeneration leading to decreased water diffusion due to the small size of the vacuoles. 4,5 We assume that vacuoles are the predominant neuropathologic feature in the early disease stage, correlating with higher DWI sensitivity. Reactive gliotic changes may become more prominent during the disease course, causing high signals on FLAIR.…”

Section: Discussionmentioning

confidence: 99%

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Isolated Cortical Signal Increase on MR Imaging as a Frequent Lesion Pattern in Sporadic Creutzfeldt-Jakob Disease

Meissner¹,

Kallenberg²,

Sánchez‐Juan³

et al. 2008

AJNR Am J Neuroradiol

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Section: Discussionmentioning

confidence: 99%

“…[4][5][6] DWI was shown to be the most sensitive sequence in the detection of brain lesions, particularly in the neocortex. [7][8][9][10] Isolated cortex involvement was also found.…”

mentioning

confidence: 99%

Isolated Cortical Signal Increase on MR Imaging as a Frequent Lesion Pattern in Sporadic Creutzfeldt-Jakob Disease

Meissner¹,

Kallenberg²,

Sánchez‐Juan³

et al. 2008

AJNR Am J Neuroradiol

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“…2 The imaging findings in sCJD typically consist of cortical atrophy and hyperintensities in the basal ganglia, thalamus, and cortex on fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI). [3][4][5][6][7][8][9][10][11][12][13] fCJD has imaging findings and neuropathology that are, in general, similar to the most common forms of sCJD; however, most imaging studies on fCJD consist primarily of case reports or studies focused on sCJD that combine a few fCJD patients into a single CJD patient sample. 8,[14][15][16][17][18][19][20][21][22] fCJD studies are limited not only by small sample sizes but also by nonstandardized imaging protocols and clinical and pathophysiologic heterogeneity.…”

mentioning

confidence: 99%

MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study

Fulbright

Hoffmann²,

Lee³

et al. 2008

AJNR Am J Neuroradiol

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“…[6][7][8] Although the CSF composition is not formally part of the diagnostic criteria, the CSF in CJD is characteristically acellular with normal glucose. [9][10][11][12][13][14] Cases with mild pleocytosis (5 to 11 cells per mm 3 ) have been infrequently reported. 15 The CSF protein may be normal or slightly elevated, but rarely greater than 500 mg per deciliter.…”

Section: Discussionmentioning

confidence: 99%

Inflammatory Cerebrospinal Fluid in Sporadic Creutzfeldt-Jakob Disease

Bui

Ehrensperger²,

Sahlas³

et al. 2008

Can. j. neurol. sci.

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Transmissible spongiform encephalopathies (TSE) are associated with the accumulation of abnormal protease-resistant proteins (prions) which are isoforms of protease-sensitive normal host cellular proteins. 1 The protein-only hypothesis, initially promulgated by Prusiner and supplanting an earlier slow-virus etiology, posits a conformational change of the alphahelical component of the normal host cellular protein to a betapleated sheet structure resulting in prion accumulation within, and eventual degeneration of, affected neuronal cells.2 Human TSE occurs in both sporadic (non-familial) and, less commonly, inherited forms. Mutant prion proteins have been implicated in Creutzfeldt-Jakob disease (CJD), new variant CJD, GerstmannStraussler-Scheinker disease, familial and sporadic fatal insomnia, and kuru. In these conditions, immune/inflammatory reactions are generally absent in blood, brain parenchyma and cerebrospinal fluid (CSF), perhaps because their pathogeneses do not entail exposure to foreign antigens. In patients with progressive central nervous system (CNS) dysfunction, the presence of a significant CSF pleocytosis generally excludes TSE as a viable diagnostic consideration. In this article, we report two patients with neuropathologically-confirmed sporadic CJD presenting with an inflammatory CSF profile. ABSTRACT:Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a fatal, transmissible spongiform encephalopathy characterized by rapidly progressive dementia, myoclonus, ataxia and akinetic mutism. The underlying mechanism is believed to be a conformational change of a native prion protein which characteristically fails to provoke an immune response. Commensurate with the latter, cerebrospinal fluid (CSF) classically exhibits a non-inflammatory profile. Cases: We report two patients with pathologically-proven sporadic CJD presenting with a significant CSF pleocytosis. Conclusion: Although uncommon, the presence of an inflammatory CSF profile should not exclude the diagnosis of sporadic CJD.RÉSUMÉ: Liquide céphalorachidien inflammatoire dans la maladie de Creutzfeldt-Jakob sporadique. Contexte : La maladie de Creutzfeldt-Jakob (MCJ) est une encéphalopathie spongiforme transmissible qui est fatale et qui se caractérise par une démence rapidement progressive, du myoclonus, de l'ataxie et un mutisme akinétique. Le mécanisme sous-jacent est, croit-on, un changement de conformation d'une protéine prion native qui ne provoque donc pas de réponse immunitaire. Le liquide céphalorachidien (LCR) a un profil non inflammatoire chez ces patients. Observations : Nous rapportons les observations faites chez deux patients, dont la MCJ a été prouvée en anatomopathologie, qui présentaient une pleiocytose importante du LCR. Conclusion : Bien que rare, la présence d'un profil inflammatoire du LCR ne devrait pas exclure le diagnostic de MCJ sporadique.

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Diffusion-Weighted Magnetic Resonance Imaging in Probable Creutzfeldt-Jakob Disease

Abstract: These findings extend previous observations that suggested a diagnostic and localizing utility of diffusion-weighted imaging in CJD.

Cited by 80 publications

References 31 publications

Isolated Cortical Signal Increase on MR Imaging as a Frequent Lesion Pattern in Sporadic Creutzfeldt-Jakob Disease

Isolated Cortical Signal Increase on MR Imaging as a Frequent Lesion Pattern in Sporadic Creutzfeldt-Jakob Disease

MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study

Inflammatory Cerebrospinal Fluid in Sporadic Creutzfeldt-Jakob Disease

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