The Revised Self-Monitoring Scale detects early impairment of social cognition in genetic frontotemporal dementia within the GENFI cohort

on behalf of the Genetic FTD Initiative, GENFI., Hannah D. Franklin, Lucy L. Russell, Georgia Peakman, Caroline V. Greaves, Martina Bocchetta, Jennifer Nicholas, Jackie Poos, Rhian S. Convery, David M. Cash, John van Swieten, Lize Jiskoot, Fermin Moreno, Raquel Sanchez-Valle, Barbara Borroni, Robert Laforce, Mario Masellis, Maria Carmela Tartaglia, Caroline Graff, Daniela GalimbertiJames B. Rowe, Elizabeth Finger, Matthis Synofzik, Rik Vandenberghe, Alexandre de Mendonça, Fabrizio Tagliavini, Isabel Santana, Simon Ducharme, Chris Butler, Alex Gerhard, Johannes Levin, Adrian Danek, Markus Otto, Sandro Sorbi, Isabelle Le Ber, Florence Pasquier, Jonathan D. Rohrer, Sónia Afonso, Maria Rosario Almeida, Andrea Arighi, Paola Caroppo, Giuseppe Di Fede, Chiara Fenoglio, Giorgio Fumagalli, Giorgio Giaccone, Benedetta Nacmias, Sara Prioni, Veronica Redaelli, Giacomina Rossi, Elio Scarpini, Pietro Tiraboschi

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Although social cognitive dysfunction is a major feature of frontotemporal dementia (FTD), it has been poorly studied in familial forms. A key goal of studies is to detect early cognitive impairment using validated measures in large patient cohorts. Methods: We used the Revised Self-Monitoring Scale (RSMS) as a measure of socioemotional sensitivity in 730 participants from the genetic FTD initiative (GENFI) observational study: 269 mutation-negative healthy controls, 193 C9orf72 expansion carriers, 193 GRN mutation carriers and 75 MAPT mutation carriers. All participants underwent the standardised GENFI clinical assessment including the ‘CDR® plus NACC FTLD’ scale and RSMS. The RSMS total score and its two subscores, socioemotional expressiveness (EX score) and modification of self-presentation (SP score) were measured. Volumetric T1-weighted magnetic resonance imaging was available from 377 mutation carriers for voxel-based morphometry (VBM) analysis. Results: The RSMS was decreased in symptomatic mutation carriers in all genetic groups but at a prodromal stage only in the C9orf72 (for the total score and both subscores) and GRN (for the modification of self-presentation subscore) groups. RSMS score correlated with disease severity in all groups. The VBM analysis implicated an overlapping network of regions including the orbitofrontal cortex, insula, temporal pole, medial temporal lobe and striatum. Conclusions: The RSMS indexes socioemotional impairment at an early stage of genetic FTD and may be a suitable outcome measure in forthcoming trials.

Original languageEnglish
Article number127
JournalAlzheimer's Research and Therapy
Volume13
Issue number1
DOIs
Publication statusPublished - Dec 2021

Keywords

  • C9orf72
  • CDR® plus NACC FTLD
  • Familial
  • Frontotemporal dementia
  • GRN
  • MAPT
  • RSMS
  • VBM

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology
  • Cognitive Neuroscience

Fingerprint

Dive into the research topics of 'The Revised Self-Monitoring Scale detects early impairment of social cognition in genetic frontotemporal dementia within the GENFI cohort'. Together they form a unique fingerprint.

Cite this