The Revised Self-Monitoring Scale detects early impairment of social cognition in genetic frontotemporal dementia within the GENFI cohort
Authors: Franklin Hannah D. , Russell Lucy L. , Peakman Georgia , Greaves Caroline V. , Bocchetta Martina , Nicholas Jennifer , Poos Jackie , Convery Rhian S. , Cash David M. , van Swieten John , Jiskoot Lize , Moreno Fermin , Sanchez-Valle Raquel , Borroni Barbara , Laforce Robert , Masellis Mario , Tartaglia Maria Carmela , Graff Caroline , Galimberti Daniela , Rowe James B. , Finger Elizabeth , Synofzik Matthis , Vandenberghe Rik , de Mendonça Alexandre , Tagliavini Fabrizio , Santana Isabel , Ducharme Simon , Butler Chris , Gerhard Alex , Levin Johannes , Danek Adrian , Otto Markus , Sorbi Sandro , Le Ber Isabelle , Pasquier Florence , Rohrer Jonathan D. ,   on behalf of the Genetic FTD Initiative, GENFI, Afonso Sónia , Almeida Maria Rosario , Anderl-Straub Sarah , Andersson Christin , Antonell Anna , Archetti Silvana , Arighi Andrea , Balasa Mircea , Barandiaran Myriam , Bargalló Nuria , Bartha Robart , Bender Benjamin , Benussi Alberto , Bertoux Maxime , Bertrand Anne , Bessi Valentina , Black Sandra , Borrego-Ecija Sergi , Bras Jose , Brice Alexis , Bruffaerts Rose , Camuzat Agnès , Cañada Marta , Cantoni Valentina , Caroppo Paola , Castelo-Branco Miguel , Colliot Olivier , Cope Thomas , Deramecourt Vincent , de Arriba María , Di Fede Giuseppe , Díez Alina , Duro Diana , Fenoglio Chiara , Ferrari Camilla , Ferreira Catarina B. , Fox Nick , Freedman Morris , Fumagalli Giorgio , Funkiewiez Aurélie , Gabilondo Alazne , Gasparotti Roberto , Gauthier Serge , Gazzina Stefano , Giaccone Giorgio , Gorostidi Ana , Greaves Caroline , Guerreiro Rita , Heller Carolin , Hoegen Tobias , Indakoetxea Begoña , Jelic Vesna , Karnath Hans-Otto , Keren Ron , Kuchcinski Gregory , Langheinrich Tobias , Lebouvier Thibaud , Leitão Maria João , Lladó Albert , Lombardi Gemma , Loosli Sandra , Maruta Carolina , Mead Simon , Meeter Lieke , Miltenberger Gabriel , van Minkelen Rick , Mitchell Sara , Moore Katrina , Nacmias Benedetta , Nelson Annabel , Öijerstedt Linn , Olives Jaume , Ourselin Sebastien , Padovani Alessandro , Panman Jessica , Papma Janne M. , Pijnenburg Yolande , Polito Cristina , Premi Enrico , Prioni Sara , Prix Catharina , Rademakers Rosa , Redaelli Veronica , Rinaldi Daisy , Rittman Tim , Rogaeva Ekaterina , Rollin Adeline , Rosa-Neto Pedro , Rossi Giacomina , Rossor Martin , Santiago Beatriz , Saracino Dario , Sayah Sabrina , Scarpini Elio , Schönecker Sonja , Seelaar Harro , Semler Elisa , Shafei Rachelle , Shoesmith Christen , Swift Imogen , Tábuas-Pereira Miguel , Tainta Mikel , Taipa Ricardo , Tang-Wai David , Thomas David L. , Thompson Paul , Thonberg Hakan , Timberlake Carolyn , Tiraboschi Pietro , Todd Emily , Van Damme Philip , Vandenbulcke Mathieu , Veldsman Michele , Verdelho Ana , Villanua Jorge , Warren Jason , Wilke Carlo , Woollacott Ione , Wlasich Elisabeth , Zetterberg Henrik , Zulaica Miren 
Published: July 2021

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.