Psychiatric diagnoses underlying the phenocopy syndrome of behavioural variant frontotemporal dementia.

INTRODUCTION: The frontotemporal dementia (FTD) consortium criteria (2011) emphasise the importance of distinguishing possible and probable behavioural variant FTD (bvFTD). A significant number of possible patients with bvFTD do not show functional decline and remain with normal neuroimaging over time, thus exhibiting the bvFTD phenocopy syndrome. A neurodegenerative nature is unlikely but an alternative explanation is missing. Our aim was to detect psychiatric conditions underlying the bvFTD phenocopy syndrome after extensive evaluation. METHODS: We included patients with the bvFTD phenocopy syndrome whereby patients with probable bvFTD served as a control group. Patients had to have undergone both neurological and psychiatric evaluation. Their charts were reviewed retrospectively. Using both qualitative and quantitative methods, psychiatric and psychological conditions associated with the clinical syndrome were determined in both groups and their relative frequencies were compared. RESULTS: Of 181 suspected bvFTD cases, 33 patients with bvFTD phenocopy syndrome and 19 with probable bvFTD were included. Recent life events, relationship problems and cluster C personality traits were the most prevalent psychiatric/psychological conditions. The frequency of these conditions was higher in the group of patients with the bvFTD phenocopy syndrome (n=28) compared to the probable bvFTD group (n=9) (χ(2) p<0.05). CONCLUSIONS: This is the first study thoroughly exploring psychiatric causes of the bvFTD phenocopy syndrome, revealing that in most cases multiple factors played a contributory role. Our study gives arguments for neurological and psychiatric collaboration when diagnosing bvFTD. Prompt diagnosis of treatable psychiatric conditions is to be gained.

The added value of 18-fluorodeoxyglucose-positron emission tomography in the diagnosis of the behavioral variant of frontotemporal dementia.

UNLABELLED: Characteristic frontotemporal abnormalities on structural or functional neuroimaging are mandatory for a diagnosis of probable behavioral variant of frontotemporal dementia (bvFTD) according to the new criteria. 18-Fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) imaging is commonly reserved for patients with suspected bvFTD without characteristic structural neuroimaging results. We studied the diagnostic value of 18F-FDG-PET in these patients. METHODS: The 18F-FDG-PET was performed in 52 patients with suspected bvFTD but lacking characteristic structural neuroimaging results. The clinical diagnosis of bvFTD in the presence of functional decline (bvFTD/fd+) after a follow-up period of 2 years was used as a golden standard. RESULTS: The sensitivity of 18F-FDG-PET for bvFTD/fd+ was 47% at a specificity of 92%. The differential diagnosis comprised alternative neurodegenerative and psychiatric disorders and a benign phenocopy of bvFTD. CONCLUSIONS: The 18F-FDG-PET is able to identify nearly half of the patients with bvFTD who remain undetected by magnetic resonance imaging. In our selected group, high specificity enables exclusion of psychiatric and other neurodegenerative disorders.