The bvFTD phenocopy syndrome: a case study supported by repeated MRI, [18F]FDG-PET and pathological assessment. Issue 2 (4th March 2021)

• Record Type: Journal Article
• 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.; Dols, Annemieke; Barkhof, Frederik; Berckel, Bart N.M.Van; Scheltens, Philip; Brain Bank, Netherlands; Pijnenburg, Yolande A.L.
• Is Part Of: Neurocase. Volume 27:Issue 2(2021)
• Page Start: 181
• Page End: 189
• Publication Date: 2021-03-04
• Subjects: Behavioral variant frontotemporal dementia -- misdiagnosis -- neuropsychiatry -- phenocopy syndrome -- diagnostic distinction of bvFTD and primary psychiatric diseases; Neurology -- Periodicals; Neuropsychology -- Case Report; Mental Disorders -- Case Report; Nervous System Diseases -- Case Report; 616.8
• DOI: 10.1080/13554794.2021.1905855
• Languages: English
• ISSNs: 1355-4794

Abstract

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.