Objectives
Frontotemporal dementia (FTD) can present with changes in music appreciation. Research has suggested a relationship of altered music appreciation phenotypes with typical socio‐emotional ...changes. We aimed to determine the prevalence and severity of music appreciation phenotypes in FTD and study the relationship with emotion recognition capacities in order to examine whether they could serve as a proxy for changes in socio‐emotional functioning.
Methods/Design
Based on reported musical changes in the literature, we developed an informant‐based questionnaire to assess musical changes and a music test to assess music emotion recognition. Social cognition was assessed with the Ekman 60 faces test in a subgroup of patients (n = 23). Relationships between measures were assessed with linear regressions.
Results
We included 47 patients (44.7% female, mean age 65.0 ± 8.4, 31 behavioral variant FTD (bvFTD), 10 semantic dementia (SD), and six progressive nonfluent aphasia (PNFA)). Thirty‐six caregivers were included in the music emotion recognition test as controls. Altered music appreciation phenotypes were observed in 79% of the FTD patients. Musicophilia was present in a third of bvFTD patients, and only in up to 10% in language FTD variants. Changes in music appreciation were not associated with decreased music emotion recognition or visual emotion recognition. Compared to controls, bvFTD performed worse on the music emotion recognition task (p < 0.003), and no differences were found with SD (p = 0.06) and PNFA patients (p = 0.8).
Conclusions
Music appreciation phenotypes are highly prevalent in FTD patients. Future studies should further investigate the potential diagnostic value of changes in music processing.
Key points
Music appreciation phenotypes are highly prevalent in frontotemporal dementia.
Music appreciation phenotypes are not related to emotion recognition capacities.
bvFTD patients perform worse on a music emotion recognition task.
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FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
Abstract
The behavioural variant of Alzheimer’s disease (bvAD) is characterized by early predominant behavioural changes, mimicking the behavioural variant of frontotemporal dementia (bvFTD), which ...is characterized by social cognition deficits and altered biometric responses to socioemotional cues. These functions remain understudied in bvAD.
We investigated multiple social cognition components (i.e. emotion recognition, empathy, social norms and moral reasoning), using the Ekman 60 faces test, Interpersonal Reactivity Index, empathy eliciting videos, Social Norms Questionnaire and moral dilemmas, while measuring eye movements and galvanic skin response. We compared 12 patients with bvAD with patients with bvFTD (n = 14), typical Alzheimer’s disease (tAD, n = 13) and individuals with subjective cognitive decline (SCD, n = 13), using ANCOVAs and age- and sex-adjusted post hoc testing.
Patients with bvAD (40.1 ± 8.6) showed lower scores on the Ekman 60 faces test compared to individuals with SCD (49.7 ± 5.0, P < 0.001), and patients with tAD (46.2 ± 5.3, P = 0.05) and higher scores compared to patients with bvFTD (32.4 ± 7.3, P = 0.002). Eye-tracking during the Ekman 60 faces test revealed no differences in dwell time on the eyes (all P > 0.05), but patients with bvAD (18.7 ± 9.5%) and bvFTD (19.4 ± 14.3%) spent significantly less dwell time on the mouth than individuals with SCD (30.7 ± 11.6%, P < 0.01) and patients with tAD (32.7 ± 12.1%, P < 0.01). Patients with bvAD (11.3 ± 4.6) exhibited lower scores on the Interpersonal Reactivity Index compared with individuals with SCD (15.6 ± 3.1, P = 0.05) and similar scores to patients with bvFTD (8.7 ± 5.6, P = 0.19) and tAD (13.0 ± 3.2, P = 0.43). The galvanic skin response to empathy eliciting videos did not differ between groups (all P > 0.05). Patients with bvAD (16.0 ± 1.6) and bvFTD (15.2 ± 2.2) showed lower scores on the Social Norms Questionnaire than patients with tAD (17.8 ± 2.1, P < 0.05) and individuals with SCD (18.3 ± 1.4, P < 0.05). No group differences were observed in scores on moral dilemmas (all P > 0.05), while only patients with bvFTD (0.9 ± 1.1) showed a lower galvanic skin response during personal dilemmas compared with SCD (3.4 ± 3.3 peaks per min, P = 0.01).
Concluding, patients with bvAD showed a similar although milder social cognition profile and a similar eye-tracking signature to patients with bvFTD and greater social cognition impairments and divergent eye movement patterns compared with patients with tAD. Our results suggest reduced attention to salient facial features in these phenotypes, potentially contributing to their emotion recognition deficits.
Singleton et al. show that individuals with the behavioural variant of Alzheimer’s disease have similar but milder social cognition impairments versus individuals with behavioural variant frontotemporal dementia, as well as greater social cognition impairments and distinct eye-tracking patterns compared with individuals with typical Alzheimer’s disease.
The CRESST-II cryogenic Dark Matter search, aiming at detection of WIMPs via elastic scattering off nuclei in CaWO
4
crystals, completed 730 kg days of data taking in 2011. We present the data ...collected with eight detector modules, each with a two-channel readout; one for a phonon signal and the other for coincidently produced scintillation light. The former provides a precise measure of the energy deposited by an interaction, and the ratio of scintillation light to deposited energy can be used to discriminate different types of interacting particles and thus to distinguish possible signal events from the dominant backgrounds.
Sixty-seven events are found in the acceptance region where a WIMP signal in the form of low energy nuclear recoils would be expected. We estimate background contributions to this observation from four sources: (1) “leakage” from the
e
/
γ
-band (2) “leakage” from the
α
-particle band (3) neutrons and (4)
206
Pb recoils from
210
Po decay. Using a maximum likelihood analysis, we find, at a statistical significance of more than 4
σ
, that these sources alone are not sufficient to explain the data. The addition of a signal due to scattering of relatively light WIMPs could account for this discrepancy, and we determine the associated WIMP parameters.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
The CRESST experiment is a direct dark matter search which aims to measure interactions of potential dark matter particles in an Earth-bound detector. With the current stage, CRESST-III, we focus on ...a low energy threshold for increased sensitivity towards light dark matter particles. In this paper we describe the analysis of one detector operated in the first run of CRESST-III (05/2016–02/2018) achieving a nuclear recoil threshold of 30.1 eV. This result was obtained with a 23.6 g CaWO4 crystal operated as a cryogenic scintillating calorimeter in the CRESST setup at the Laboratori Nazionali del Gran Sasso (LNGS). Both the primary phonon (heat) signal and the simultaneously emitted scintillation light, which is absorbed in a separate silicon-on-sapphire light absorber, are measured with highly sensitive transition edge sensors operated at ∼15 mK. The unique combination of these sensors with the light element oxygen present in our target yields sensitivity to dark matter particle masses as low as 160 MeV/c2.
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CMK, CTK, FMFMET, IJS, NUK, PNG, UM
Hearing loss in older adults is associated with increased dementia risk. Underlying mechanisms that connect hearing loss with dementia remain largely unclear.
We studied the association of hearing ...loss and biomarkers for dementia risk in two age groups with normal cognition: 65 participants from the European Medical Information Framework (EMIF)-Alzheimer's disease (AD) 90+ study (oldest-old; mean age 92.7 years, 56.9% female) and 60 participants from the EMIF-AD PreclinAD study (younger-old; mean age 74.4, 43.3% female). Hearing function was tested by the 'digits-in-noise test' and cognition by repeated neuropsychological evaluation. Regressions and generalised estimating equations were used to test the association of hearing function and PET-derived amyloid burden, and linear mixed models were used to test the association of hearing function and cognitive decline. In the oldest-old group, mediation analyses were performed to study whether cognitive decline is mediated through regional brain atrophy.
In oldest-old individuals, hearing function was not associated with amyloid pathology (p=0.7), whereas in the younger-old individuals hearing loss was associated with higher amyloid burden (p=0.0034). In oldest-old individuals, poorer hearing was associated with a steeper decline in memory, global cognition and language, and in the younger-old with steeper decline in language only. The hippocampus and nucleus accumbens mediated the effects of hearing loss on memory and global cognition in the oldest-old individuals.
Hearing loss was associated with amyloid binding in younger-old individuals only, and with cognitive decline in both age groups. These results suggest that mechanisms linking hearing loss with risk for dementia depends on age.
Despite significant symptomatic overlap between behavioral variant frontotemporal dementia (bvFTD) and primary psychiatric disorders (PPDs), a potential overlap in their structural anatomical changes ...has not been studied systematically.
In this magnetic resonance imaging-based meta-analysis, we included studies on bvFTD, schizophrenia, bipolar disorder, and autism spectrum disorder that 1) used voxel-based morphometry analysis to assess regional gray matter volumes (GMVs) and 2) reported the coordinates of the regional GMV. Separate analyses were performed comparing clusters of coordinate-based changes in the GMVs (n = 24,183) between patients and control subjects, and overlapping brain regions between bvFTD and each PPD were examined.
We found that GMV alterations in the prefrontal and anterior cingulate cortices, temporal lobe, amygdala, and insula comprise the transdiagnostic brain alterations in bvFTD and PPD.
Our meta-analysis revealed significant anatomical overlap that paves the way for future investigations of shared pathophysiological pathways, and our cross-disorder approach would provide new insights to better understand the relationship between bvFTD and PPD.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
For above ground particle physics experiments, cosmic muons are common source of background, not only for direct detector hits, but also for secondary radiation created in neighboring materials. The ...ECHo experiment has been designed for the determination of the effective electron neutrino mass by the analysis of the endpoint region of the
163
Ho
electron capture spectrum. The fraction of events occurring in the region of interest of 10 eV below the
Q
EC
value of about 2.8 keV is only of the order of
10
-
9
. This means that the background in that region need to be studied, characterized and methods to suppress it need to be developed. We expect a major background contribution to be due to cosmic muons and radiation produced by muons traveling through material around the detectors. To determine the muon-related background in metallic magnetic calorimeters (MMCs) used in the ECHo experiment, we have performed an experiment in which a muon veto was installed around the cryostat used for the operation of the detectors. We analysed the acquired events to investigate the pulse shape of MMC events in coincidence with the muon veto and the rate of multiple coincidences among detector array pixels. With different methods used for identification of muon related events, we studied events generated by muons and secondary radiation depositing energy in the substrate close to the ECHo pixels. In addition, energy depositions of muons and secondary radiation in the detectors was studied via Monte Carlo simulation. At the present status of investigation, we conclude that muon related events will be a negligible background in the region of interest of the
163
Ho
spectrum.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Background and purpose
Early diagnosis of behavioral variant frontotemporal dementia (bvFTD) is challenging due to symptomatic overlap with primary psychiatric disorders (PPD). As emotion recognition ...deficits are early and key features of bvFTD, the aim was to explore processes driving social cognition deficits that may aid in the differentiation between bvFTD and PPD.
Methods
The total sample (N = 51) included 18 patients with bvFTD, 11 patients with PPD (mood, autism spectrum and psychotic disorders) and 22 controls from the Alzheimer Center Amsterdam of the Amsterdam UMC. Emotion recognition was assessed with the Ekman 60 Faces test, during which eye tracking metrics were collected in the first 5 s a face was presented. Group differences in dwell time on the total image as well as the circumscribed eyes area and mouth area were analysed using ANOVA, with post hoc comparisons.
Results
Patients with bvFTD scored lowest, patients with PPD scored intermediate and controls scored highest on emotion recognition. During facial processing, patients with bvFTD spent less dwell time on the total image than controls (mean difference 11.3%, F(2, 48) = 6.095, p = 0.004; bvFTD−controls p = 0.001, 95% confidence interval CI −892.64, −239.70). Dwell time on the eyes area did not differ between diagnostic groups, whilst patients with bvFTD spent less dwell time on the mouth area than PPD patients (mean difference 10.7%; F(2, 48) = 3.423, p = 0.041; bvFTD−PPD p = 0.022, 95% CI −986.38, −79.47) and controls (mean difference 7.8%; bvFTD−controls p = 0.043, 95% CI −765.91, −12.76).
Conclusions
In bvFTD, decreased emotion recognition may be related to reduced focus on facial hallmarks. These findings suggest a valuable role for biometrics in social cognition assessment and the differentiation between bvFTD and PPD.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SAZU, SBCE, SBMB, UL, UM, UPUK
The CRESST-II experiment uses cryogenic detectors to search for nuclear recoil events induced by the elastic scattering of dark matter particles in CaWO
4
crystals. Given the low energy threshold of ...our detectors in combination with light target nuclei, low mass dark matter particles can be probed with high sensitivity. In this letter we present the results from data of a single detector module corresponding to 52 kg live days. A blind analysis is carried out. With an energy threshold for nuclear recoils of 307 eV we substantially enhance the sensitivity for light dark matter. Thereby, we extend the reach of direct dark matter experiments to the sub- GeV/
c
2
region and demonstrate that the energy threshold is the key parameter in the search for low mass dark matter particles.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
The determination of the effective electron neutrino mass by analyzing the end point region of the
163
Ho electron capture (EC) spectrum relies on the precise description of the expected
163
Ho ...events and background events. In the ECHo experiment, arrays of metallic magnetic calorimeters, implanted with
163
Ho, are operated to measure the
163
Ho EC spectrum. In an energy range of 10 eV below
Q
EC
, the maximum available energy for the EC decay of about 2.8 keV, a
163
Ho event rate of the order of
10
-
4
day
-
1
pixel
-
1
is expected for an activity of 1 Bq of
163
Ho per pixel. This means, a control of the background level in the order of
10
-
5
day
-
1
pixel
-
1
is extremely important. We discuss the results of a Monte Carlo study based on simulations, which use the GEANT4 framework to understand the impact of natural radioactive isotopes close to the active detector volume in the case of the ECHo-1k set-up, which is used for the first phase of the ECHo experiment. For this, the ECHo-1k set-up was modeled in GEANT4 using the proper geometry and materials, including the information of screening measurements of some materials used in the ECHo-1k set-up and reasonable assumptions. Based on the simulation and on assumptions, we derive the expected background around
Q
EC
and give upper limits of tolerable concentrations of natural radionuclides in the set-up materials. In addition, we compare our results to background spectra acquired in detector pixels with and without implanted
163
Ho. We conclude that typical concentration of radioactive nuclides found in the used materials should not endanger the analysis of the endpoint region of the
163
Ho EC spectrum for an exposure time of half a year.
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DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK