Affiliations: [a] Department of Clinical Research in Neurology, Center for Neurodegenerative Diseases and the Aging Brain, University of Bari ‘Aldo Moro’, “Pia Fondazione Cardinale G.Panico”, Tricase, Italy
| [b] Department of Medicine, Surgery and Health Sciences, Clinical Unit of Neurology, University Hospital of Trieste, University of Trieste, Trieste, Italy
| [c] Department of Neurosciences, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, London, UK
| [d]
Department of Diagnostic Imaging, Pia Fondazione di Culto e Religione “Card. G.Panico”, Tricase, Italy
Correspondence:
[*]
Correspondence to: Dr. Daniele Urso, Department of Clinical Research in Neurology, Center for Neurodegenerative Diseases and the Aging Brain, University of Bari ‘Aldo Moro’, “Pia Fondazione Cardinale G. Panico”, Tricase, Lecce, Italy. E-mail: daniele.urso@kcl.ac.uk.
Note: [1] These authors contributed equally to this work.
Abstract: Background:Behavioral variant frontotemporal dementia (bvFTD) typically involves subtle changes in personality that can delay a timely diagnosis. Objective:Here, we report the case of a patient diagnosed of GRN-positive bvFTD at the age of 52 presenting with a 7-year history of narcissistic personality disorder, accordingly to DSM-5 criteria. Methods:The patient was referred to neurological and neuropsychological examination. She underwent 3 Tesla magnetic resonance imaging (MRI) and genetic studies. Results:The neuropsychological examination revealed profound deficits in all cognitive domains and 3T brain MRI showed marked fronto-temporal atrophy. A mutation in the GRN gene further confirmed the diagnosis. Conclusions:The present case documents an unusual onset of bvFTD and highlights the problematic nature of the differential diagnosis between prodromal psychiatric features of the disease and primary psychiatric disorders. Early recognition and diagnosis of bvFTD can lead to appropriate management and support for patients and their families. This case highlights the importance of considering neurodegenerative diseases, such as bvFTD, in the differential diagnosis of psychiatric disorders, especially when exacerbations of behavioral traits manifest in adults.
