TY  - JOUR
AB  - 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.
AU  - Obergassel, Julius
AU  - Lohmann, Lisa
AU  - Meuth, Sven
AU  - Meuth, Sven G.
AU  - Wiendl, Heinz
AU  - Wiendl, H.
AU  - Grauer, Oliver Martin
AU  - Nelke, Christopher
DA  - 2019-12-27
DO  - 10.1080/19336896.2019.1706703
KW  - Creutzfeld-Jakob disease
KW  - HeidenhainM
KW  - MM1
KW  - stroke
KW  - FDG-PET
KW  - RT-QuIC
LA  - eng
N1  - PRION 14 (2020) 1, 24–28
N1  - Finanziert durch den Open-Access-Publikationsfonds der Westfälischen Wilhelms-Universität Münster (WWU Münster).
PY  - 2019-12-27
TI  - An enigmatic case of cortical anopsia: Antemortem diagnosis of a 14-3-3 negative Heidenhain-variant MM1-sCJD
UR  - https://nbn-resolving.org/urn:nbn:de:hbz:6-52109425399
Y2  - 2024-11-21T18:27:17
ER  -