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Thread: hvCJD, sporadic spontaneous CJD and the TSE Prion December 14, 2019

  1. #1
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    Default hvCJD, sporadic spontaneous CJD and the TSE Prion December 14, 2019

    THURSDAY, DECEMBER 12, 2019

    Heidenhain Variant Creutzfeldt Jakob Disease hvCJD, sporadic spontaneous CJD and the TSE Prion December 14, 2019

    22 years, rip mom dod 12/14/97 confirmed hvcjd, just made a promise to mom, and you don't break those promises, never forget, and never let them forget, before we all do...this pearl's for you! love terry

    https://creutzfeldt-jakob-disease.bl...ldt-jakob.html

    kind regards, terry

  2. #2
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    Case Report

    An enigmatic case of cortical anopsia: Antemortem diagnosis of a 14-3-3 negative Heidenhain-variant MM1-sCJD

    Julius Obergassel,Lisa Lohmann,Sven G. Meuth,Heinz Wiendl,Oliver Grauer &Christopher Nelke

    Pages 24-28 | Received 13 Nov 2019, Accepted 13 Dec 2019, Published online: 27 Dec 2019

    Download citation https://doi.org/10.1080/19336896.2019.1706703 CrossMark

    ABSTRACT

    Sporadic Creutzfeldt-Jakob disease is the predominant type of human prion disease. While routine diagnostic in phenotypic cases has advanced considerably, the clinical heterogeneity and rarity of subtypes continue to constitute a major clinical and diagnostic challenge. Here, we report a peculiar case of the Heidenhain-variant of MM1 sporadic Creutzfeldt-Jakob disease presenting as a stroke mimic in an 81-year-old patient with a rapid and clinically distinct course of disease as compared to previously reported cases. While 14-3-3 protein was negative, clinical findings substantiated by 18F-FDG-PET imaging and RT-QuIC-Assay were able to establish the diagnosis. We conclude that in cases presenting with rapid progressive dementia secondary to sudden cortical anopsia the Heidenhain-variant of CJD should be considered.

    snip...

    In conclusion, we established the probable antemortem diagnosis of Heidenhain-variant sCJD with MM1 molecular subtype. Key clinical findings were an advanced age at onset combined with an accelerated course of disease mimicking an ischaemic stroke. We propose that an unusual presentation of the Heidenhain-variant might be considered as a ‘stroke-mimic’ in cases of isolated visual symptoms followed by unexplained neurological deterioration. FDG-PET imaging and CSF RT-QuIC prion protein aggregation assay have proven their diagnostic value, while we were unable to detect 14-3-3 protein. A limitation of our case is the lack of a histopathology. The clinical heterogeneity of sCJD sustains diagnostic difficulties.

    KEYWORDS: Creutzfeld-Jakob disease, Heidenhain, MM1, stroke, FDG-PET, RT-QuIC

    https://www.tandfonline.com/doi/full...6.2019.1706703

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