Abstract
BackgroundAlthough 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.MethodsWe 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.ResultsThe 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.ConclusionsThe RSMS indexes socioemotional impairment at an early stage of genetic FTD and may be a suitable outcome measure in forthcoming trials.
Highlights
Social cognitive dysfunction is a major feature of frontotemporal dementia (FTD), it has been poorly studied in familial forms
Of the 849 participants enrolled in the second phase of the study, cross-sectional data on the Revised Self-Monitoring Scale (RSMS) was available from 730 participants, consisting of 269 healthy controls, 193 chromosome 9 open reading frame 72 (C9orf72) expansion carriers, 193 GRN mutation carriers and 75 microtubuleassociated protein tau (MAPT) mutation carriers (Table 1)
In summary, this study describes the ability of the RSMS to detect early changes in socioemotional behaviour in distinct genetic cohorts of FTD and illustrates the neural correlates of self-monitoring in these populations
Summary
Social cognitive dysfunction is a major feature of frontotemporal dementia (FTD), it has been poorly studied in familial forms. Despite differences in presentation, many people with FTD develop impaired social cognition [1], a set of psychological processes which includes the ability to evaluate social and emotional cues from others and select an appropriate behavioural response, a phenomenon often referred to as ‘socioemotional sensitivity’ or ‘self-monitoring’. In both healthy and clinical populations, the Revised Self-Monitoring Scale (RSMS) [2] has often been used to study socioemotional sensitivity and responsiveness as well as the neural networks that underlie them [3, 4]. Whilst social cognitive dysfunction has been studied extensively in sporadic FTD, few investigations have looked at genetic cohorts exclusively
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