The Revised Self-Monitoring Scale detects early impairment of social cognition in genetic frontotemporal dementia within the GENFI cohort.
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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, Genetic FTD Initiative, GENFI
Аннотация
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.