Raaphorst, Joost, Beeldman, Emma, Schmand, Ben, Berkhout, Joris, Linssen, Wim H. J. P., van den Berg, Leonard H., Pijnenburg, Yolande A., Grupstra, Hepke F., Weikamp, Janneke G., Schelhaas, H. Jurgen, Papma, Janne M., van Swieten, John C., de Visser, Marianne, de Haan, Rob J.

Objective: The assessment of behavioral disturbances in amyotrophic lateral sclerosis (ALS) is important because of the overlap with the behavioral variant of frontotemporal dementia (ALS-bvFTD). Motor symptoms and dysarthria are not taken into account in currently used behavioral questionnaires. We examined the clinimetric properties of a new behavioral questionnaire for patients with ALS (Amyotrophic Lateral […]

Pardini, M., Emberti Gialloreti, L., Mascolo, M., Benassi, F., Abate, L., Guida, S., Viani, E., Dal Monte, O., Schintu, S., Krueger, F., Cocito, L.

Objective: Recent data suggest that theory of mind (ToM) deficits represent an early symptom of the behavioural variant of frontotemporal dementia (bvFTD). However, longitudinal data on the natural history of subjects presenting with isolated ToM deficits are lacking. The aim of the study was to verify if isolated ToM deficits represent an at-risk state for […]

Ossenkoppele, Rik, Prins, Niels D., Pijnenburg, Yolande A.L., Lemstra, Afina W., van der Flier, Wiesje M., Adriaanse, Sofie F., Windhorst, Albert D., Handels, Ron L.H., Wolfs, Claire A.G., Aalten, Pauline, Verhey, Frans R.J., Verbeek, Marcel M., van Buchem, Mark A., Hoekstra, Otto S., Lammertsma, Adriaan A., Scheltens, Philip, van Berckel, Bart N.M.

Background: [(11)C]Pittsburgh compound B ([(11)C]PIB) and [(18)F]-2-fluoro-2-deoxy-D-glucose ([(18)F]FDG) PET measure fibrillar amyloid-β load and glucose metabolism, respectively. We evaluated the impact of these tracers on the diagnostic process in a memory clinic population.; Methods: One hundred fifty-four patients underwent paired dynamic [(11)C]PIB and static [(18)F]FDG PET scans shortly after completing a standard dementia screening. Two-year […]

Jesso, S., Morlog, D., Ross, S., Pell, M. D., Pasternak, S. H., Mitchell, D. G. V., Kertesz, A., Finger, E. C.

Patients with behavioural variant frontotemporal dementia demonstrate abnormalities in behaviour and social cognition, including deficits in emotion recognition. Recent studies suggest that the neuropeptide oxytocin is an important mediator of social behaviour, enhancing prosocial behaviours and some aspects of emotion recognition across species. The objective of this study was to assess the effects of a […]

Freedman, Morris, Binns, Malcolm A., Black, Sandra E., Levine, Brian, Miller, Bruce L., Ramirez, Joel, Szilagyi, Gregory M., Scott, Christopher J. M., McNeely, Alicia A., Stuss, Donald T.

We studied behavioral variant frontotemporal dementia (bvFTD) using object alternation (OA) as a novel probe of cognition. This task was adopted from animal models and is sensitive to ventrolateral-orbitofrontal and medial frontal function in humans. OA was administered to bvFTD patients, normal controls, and a dementia control group with Alzheimer disease (AD). Two other frontal […]

Finger, E. C., MacKinley, J., Blair, M., Oliver, L. D., Jesso, S., Tartaglia, M. C., Borrie, M., Wells, J., Dziobek, I., Pasternak, S., Mitchell, D. G. V., Rankin, K., Kertesz, A., Boxer, A.

Objective: To determine the safety and tolerability of 3 doses of intranasal oxytocin (Syntocinon; Novartis, Bern, Switzerland) administered to patients with frontotemporal dementia (FTD). Methods: We conducted a randomized, parallel-group, double-blind, placebo-controlled study using a dose-escalation design to test 3 clinically feasible doses of intranasal oxytocin (24, 48, or 72 IU) administered twice daily for […]

Ehrlé, Nathalie, Henry, Audrey, Pesa, Audrey, Bakchine, Serge

This paper presents a French battery designed to assess emotional and sociocognitive abilities in neurological patients in clinical practice. The first part of this battery includes subtests assessing emotions: a recognition task of primary facial emotions, a discrimination task of facial emotions, a task of expressive intensity judgment, a task of gender identification, a recognition […]

Borroni, Barbara, Malinverno, Matteo, Gardoni, Fabrizio, Grassi, Mario, Parnetti, Lucilla, Agosti, Chiara, Alberici, Antonella, Premi, Enrico, Bonuccelli, Ubaldo, Gasparotti, Roberto, Calabresi, Paolo, Di Luca, Monica, Padovani, Alessandro

Cerebrospinal fluid (CSF) tau ratio decrease (33kDa/55kDa forms) and mid-saggital midbrain-to-pons (MP) atrophy have been suggested as diagnostic markers for progressive supranuclear palsy (PSP). The usefulness of their combined evaluation has never been tested. We evaluated the CSF tau ratio and the MP atrophy as a combined marker for early identification of PSP. A total […]

Alberici, A., Archetti, S., Pilotto, A., Premi, E., Cosseddu, M., Bianchetti, A., Semeraro, F., Salvetti, M., Muiesan, M. L., Padovani, A., Borroni, B.

Frontotemporal dementia (FTD) is one of the most important neurodegenerative conditions and Granulin (GRN) is one of the major genes associated to the disease. FTD-GRN patients are still orphan for any evidence-based target-therapy approach. Interestingly, it has been recently found that alkalizing agents rescued haploinsufficiency in cellular models expressing FTD-GRN mutations. We set up a […]