The bvFTD phenocopy syndrome: a case study supported by repeated MRI, [18F]FDG-PET and pathological assessment
Publication date
2021-04
Authors
van Engelen, Marie-Paule E
Rozemuller, Annemieke J M
Ulugut Erkoyun, Hülya
Groot, Colin
Fieldhouse, Jay L P
Koene, Ted
Ossenkoppele, Rik
Gossink, Flora T
Krudop, Welmoed A
Vijverberg, Everard G B
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Advisors
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Document Type
Article
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cc_by_nc_nd
Abstract
A clinical syndrome with neuropsychiatric features of bvFTD without neuroimaging abnormalities and a lack of decline is a phenocopy of bvFTD (phFTD). Growing evidence suggests that psychological, psychiatric and environmental factors underlie phFTD. We describe a patient diagnosed with bvFTD prior to the revision of the diagnostic guidelines of FTD. Repeated neuroimaging was normal and there was no FTD pathology at autopsy, rejecting the diagnosis. We hypothesize on etiological factors that on hindsight might have played a role. This case report contributes to the understanding of phFTD and adds to the sparse literature of the postmortem assessment of phFTD.
Keywords
Fluorodeoxyglucose F18, Frontotemporal Dementia, Humans, Magnetic Resonance Imaging, Neuroimaging, Phenotype, Case Reports, Journal Article, Research Support, Non-U.S. Gov't
Citation
van Engelen, M-P E, Rozemuller, A J M, Ulugut Erkoyun, H, Groot, C, Fieldhouse, J L P, Koene, T, Ossenkoppele, R, Gossink, F T, Krudop, W A, Vijverberg, E G B, Dols, A, Barkhof, F, Berckel, B N M V, Scheltens, P, Brain Bank, N & Pijnenburg, Y A L 2021, 'The bvFTD phenocopy syndrome : a case study supported by repeated MRI, [18F]FDG-PET and pathological assessment', Neurocase, vol. 27, no. 2, pp. 181-189. https://doi.org/10.1080/13554794.2021.1905855