Aurélie Funkiewiez

Social Cognition and Emotional Assessment (SEA) is a Marker of Medial and Orbital Frontal Functions: A Voxel-Based Morphometry Study in Behavioral Variant of Frontotemporal Degeneration

The aim of this study was to explore the cerebral correlates of functional deficits that occur in behavioral variant frontotemporal dementia (bvFTD). A specific neuropsychological battery, the Social cognition & Emotional Assessment (SEA; Funkiewiez et al., 2012), was used to assess impaired social and emotional functions in 20 bvFTD patients who also underwent structural MRI scanning. The SEA subscores of theory of mind, reversal-learning tests, facial emotion identification, and apathy evaluation were entered as covariates in a voxel-based morphometry analysis. The results revealed that the gray matter volume in the rostral part of the medial prefrontal cortex [mPFC, Brodmann area (BA) 10] was associated with scores on the theory of mind subtest, while gray matter volume within the orbitofrontal (OFC) and ventral mPFC (BA 11 and 47) was related to the scores observed in the reversal-learning subtest. Gray matter volume within BA 9 in the mPFC was correlated with scores on the emotion recognition subtest, and the severity of apathetic symptoms in the Apathy scale covaried with gray matter volume in the lateral PFC (BA 44/45). Among these regions, the mPFC and OFC cortices have been shown to be atrophied in the early stages of bvFTD. In addition, SEA and its abbreviated version (mini-SEA) have been demonstrated to be sensitive to early impairments in bvFTD (Bertoux et al., 2012). Taken together, these results suggest a differential involvement of orbital and medial prefrontal subregions in SEA subscores and support the use of the SEA to evaluate the integrity of these regions in the early stages of bvFTD.

How preserved is emotion recognition in Alzheimer disease compared with behavioral variant frontotemporal dementia

Background: Emotion deficits are a recognised biomarker for behavioural variant frontotemporal dementia (bvFTD), but recent studies have reported emotion deficits also in Alzheimer’s disease (AD). Methods: A hundred and twenty-three participants (33 AD, 60 bvFTD, 30 controls) were administered a facial emotion recognition test, to investigate the clinical factors influencing the diagnostic distinction on this measure. Binomial regression analysis revealed that facial emotion recognition in AD was influenced by disease duration and MMSE, whereas the same was not true for bvFTD. Based on this information, we median-split the AD group on disease duration (3 years) or MMSE (24) and compared the facial emotion recognition performance of mild-AD, moderate-AD, bvFTD patients and controls. Results: Results showed that very mild-AD performed consistently at control levels for all emotions. By contrast, mild/moderate-AD and bvFTD were impaired compared to controls on most emotions. Interestingly, mild/moderate-AD were significantly impaired compared to very mild-AD on total score, anger and sadness subscores. Logistic regression analyses corroborated these findings with ~94% of very mild-AD being successfully distinguished from bvFTD at presentation, while this distinction was reduced to ~78% for mild/moderate-AD. Conclusions: Facial emotion recognition in AD is influenced by disease progression, with very mild-AD being virtually intact for emotion performance. Mild/moderate-AD and bvFTD show consistent impairment in emotion recognition, with bvFTD being worse. A disease progression of over 3 years or a MMSE lower than 24 should warrant caution to put too much emphasis on emotion recognition performance in the diagnostic distinction of AD and bvFTD.

Neural correlates of the mini-SEA (Social cognition and Emotional Assessment) in behavioral variant frontotemporal dementia

Although Frontotemporal Dementia (FTD) is the second most common form of dementia after Alzheimer’s disease, its diagnosis remains particularly challenging today. This is particularly true for the behavioral variant (bvFTD), the most common phenotype of FTD, which is characterised by dramatic changes in personal and social conduct. Novel clinical cognitive tests have been recently proposed to diagnose and assess these patients. Among them, the mini-SEA (Social cognition & Emotional Assessment) has shown promising results. This quick clinical tool evaluates emotion recognition and theory of mind deficits, both recognized as hallmark features of bvFTD. In this study, we investigated the neural correlates of the mini-SEA in twenty bvFTD patients, using single photon emission computed tomography (SPECT) and focusing on the mPFC. Results showed that detection of faux pas during a theory of mind evaluation was related to rostral mPFC perfusion (BA 10) while recognition of emotion involved more dorsal regions within the mPFC (BA 9). As significant and early dysfunction of the mPFC has been extensively described in bvFTD, this study supports the use of the mini-SEA in evaluation and diagnosis purposes in bvFTD.

A mosquito bites and a butterfly flies: A specific response type of frontal patients in a similarity task

Background: Patients with neurodegenerative diseases affecting the frontal lobes have difficulties in categorization tasks, such as the similarity tasks. They give two types of unusual response to the question: “In what way are an orange and a banana alike?”, either a differentiation (“one is yellow, the other is orange”) or a concrete similarity (“they are sweet”). Objective: To characterize the categorization deficit of frontal patients and develop a short diagnostic tool to assess the nature of these difficulties. Method: We analyzed the responses provided by frontal and non‐frontal neurodegenerative patients in a novel verbal similarity task (SimiCat). We included 40 frontal patients with behavioral variant fronto‐temporal dementia (bvFTD) and progressive supranuclear palsy (PSP), 23 patients with Alzheimers disease (AD) and 41 healthy matched controls. Responses that did not correspond to the expected taxonomic category (e.g.: fruits) were considered as errors. Results: All patients groups were impaired at the SimiCat test compared to controls. Differentiation errors were specific to frontal patients. Receiver operating characteristic analyses showed that a cut‐off of two differentiation errors or more achieved 85% sensitivity of 100% specificity to discriminate bvFTD from AD. A short version of the test (<5 min) showed similar discriminative validity as the full version. Conclusion: Differentiation responses were specific to frontal patients. The SimiCat demonstrates good discriminative validity to differentiate between bvFTD and AD. The short version of the test is a promising diagnostic tool that will need validation in future studies. HighlightsNeurodegenerative patients provide unexpected responses at the similarities task.Both frontal and non‐frontal patients provide concrete similarities.Only frontal patients provide differences when asked for similarities.The SimiCat Task accurately discriminates AD from bvFTD patients.

THE MINI EATING BEHAVIOR INVENTORY (MINI EBI): A 10-ITEM CLINICAL TOOL FOR THE EARLY DIAGNOSIS OF FRONTOTEMPORAL DEMENTIA

Background: In order to be helpful for the diagnosis and prediction of the shunting surgery outcome in Chinese idiopathic normal pressure hydrocephalus patients, we herein were to explore the correlation between the clinical, neuroimaging characteristics and the cerebrospinal fluid tap test response. Methods: 43 iNPH patients in PUMCH from 2013-2015 were included. All patients were evaluated using MMSE, MOCA, ADL, iNPH scale, underwent 1.5T head MRI scan and a cerebrospinal fluid tap with removal of 30 ml of CSF .The evaluations prior to and posterior to CSF tap test included the 10m walking time and steps, TMT-A, digital symbol and STROOP test. Two experienced neurologists were responsible for the adjustment of the CSF tap test response. The clinical and neuroimaging parameters of iNPH patients were analyzed between the CSF tap test responders and non-responders. Results:Compared with non-responders, the patients of CSF tap test responders had more male patients (male: female 20:4 vs. 9:10, p1⁄4 0.021), longer walking disturbance history ((1040.136708.33) days vs. (597.066527.7) days, p1⁄4 0.035), lower baseline cognitive tests z score (-15.731616.537 vs. -4.23668.280, p1⁄4 0.041) and more periventricular white matter lesions (p1⁄4 0.033). Conclusions: We report some clinical and neuroimaging characters of iNPH patients which were a little different from those have been reported. Our limitation is that the shunting result hadn’t been analyzed because of the limited number of patients. However the results indicated that the patients with the longer duration, the lower cognitive tests score and the prominent white matter lesions should not be excluded for the shunting surgery.

THE MORAL EMOTIONS ASSESSMENT: A NEW TOOL FOR THE EARLY DIAGNOSIS OF FRONTOTEMPORAL DEMENTIA

TOOL FOR THE EARLY DIAGNOSIS OF FRONTOTEMPORAL DEMENTIA Carole Azuar, Chlo e Daigmorte, Maeva Camus, Thomas Mauras, Aurelie Funkiewiez, Isabelle Le Ber, Bruno Dubois, Richard Levy, Marc Teichmann, APHPGroupe Hospitalier Pitie Salpetriere, Paris, France; 2 INSERMUniversite Pierre et Marie Curie, Paris 6, IHU-ICM, Paris, France; Centre Hospitalier Sainte Anne, Paris, France; APHP-Groupe Hospitalier Pitie Salpetriere, Paris, France; INSERMUniversit e Pierre et Marie Curie, Paris 6, IHU-ICM, Paris, France. Contact e-mail: carole.azuar@gmail.com

P4-8 Présentation du centre de référence sur les démences rares

Introduction Le plan national maladies rares a permis la labellisation de centres de reference dedies aux patients atteints de maladies rares. Ces centres ont pour objectifs d’ameliorer la prise en charge des patients, de favoriser leur acces a des consultations specialisees, de diffuser l’information medicale et de developper la recherche sur ces maladies. Nous presentons le centre de reference sur les demences rares. Methodes Le centre de reference sur les demences rares a ete labellise en juillet 2007. Il prend en charge les pathologies suivantes : demences frontotemporales, aphasies progressives non fluentes, demence semantique, paralysie supra-nucleaire progressive et degenerescence corticobasale. Le centre de reference agit en collaboration avec 12 centres de competences regionaux repartis dans toute la France et labellises depuis decembre 2008. Nous presentons les actions du centre de reference et des centres de competences regionaux. Resultats Differentes actions sont menees pour ameliorer le diagnostic de la maladie et la prise en charge des patients : 1/ consultations multidisciplinaires (incluant neurologue specialise, psychologue clinicien, neuropsychologue, orthophoniste et assistant social) dediees a chacune des pathologies prises en charge par le CR ; 2/ amelioration des procedures de diagnostic clinique (elaboration de nouveaux outils d’evaluation neuropsychologique), de diagnostic moleculaire et evaluation de nouveaux biomarqueurs (progranuline plasmatique) ; 3/ reflexion autour d’une consultation d’annonce de diagnostic ; 4/ Animation de groupes de psycho-education ; 5/ renforcements des liens avec les associations de patients ; 6/ creation d’un site Internet ; 7/ creation d’un registre de patients. La labellisation de 12 centres de competence a permis de creer un reseau national d’experts partageant les memes procedures de diagnostic, de prise en charge et de developper des projets de recherche communs portant sur ces pathologies. Conclusions La structuration recente du centre de reference permet de proposer une prise en charge specifique des patients et de leurs familles. La collaboration avec des centres de competence devrait permettre d’initier des groupes de travail et de recherche a l’echelle nationale. Des procedures d’autoevaluation seront elaborees pour evaluer le fonctionnement du centre et le benefice que cette structure apporte aux patients.