scholarly journals Apathy in Frontotemporal Dementia: Behavioral and Neuroimaging Correlates

2012 ◽  
Vol 25 (2) ◽  
pp. 127-136 ◽  
Author(s):  
Paul J. Eslinger ◽  
Peachie Moore ◽  
Shweta Antani ◽  
Chivon Anderson ◽  
Murray Grossman

We investigated the occurrence of goal-directed motivational change in the form of apathy in patients with frontotemporal dementia (FTD), particularly those with behavioral variant social and executive deficits (bvFTD). Standardized behavioral inventory was employed to survey and compare apathy ratings from patients and caregivers. In cases of bvFTD, apathy ratings were further related to measures of social cognition, executive function, and atrophy on brain MRI. Results indicated that caregivers rated bvFTD patients as having significantly elevated apathy scores though patient self-ratings were normal. Caregiver and self-ratings of FTD samples with progressive nonfluent aphasia and semantic dementia did not differ from healthy controls and their informants. In the bvFTD sample, caregiver apathy scores were not correlated with general cognitive screening or depression scores, but were significantly correlated with social cognition and executive function measures. Voxel-based morphometry revealed that apathy ratings in bvFTD were related to prominent atrophy in the right caudate (including the ventral striatum), the right temporo-parietal junction, right posterior inferior and middle temporal gyri, and left frontal operculum- anterior insula region. Findings suggest that bvFTD is associated with a significant breakdown in goal-directed motivated behavior involving disruption of cortical-basal ganglia circuits that is also related to social and executive function deficits.

2021 ◽  
Author(s):  
Vitor Arca ◽  
Pedro Albuquerque ◽  
Victor Correia ◽  
Amanda Pires ◽  
Hugo Araújo ◽  
...  

Background: Case 1: a 59-year old man presented to our service with 4 years of progressive cognitive and behavioral symptoms. He became forgetful and experienced difficulties managing his payments. After 4 years he could no longer recognise his relatives. Cognitive assessment showed a mini-mental status examination of 17/30. MRI and SPECT revealed respectively focal atrophy and hipoperfusion of the frontal regions and anterior right temporal lobe. Case 2: a 72-year-old woman was brought to evaluation with a 5-years history of progressive language and behavioral deterioration. Her family reported early speech errors and behavioral changes, with a marked aggressiveness, ritualistic behaviors and hyperorality. Cognitive evaluation revealed a MMSE of 6/30 mainly due to a relatively fluent afasia. Brain MRI showed asymmetric cerebral atrophy, more prominent in the anterior left temporal lobe. Objective: N/H Methods: N/H Results: N/H Conclusion: We describe two cases of suspected frontotemporal dementia (FTD) syndromes. The left ATL may receive proportionately more input from the lexical and phonological centers subserving word processing. The right ATL may receive more input from right-lateralized emotion processing hubs. Focal atrophy of the left anterior temporal lobe has been associated with the semantic type of primary progressive aphasia evolving to semantic dementia. In contrast, focal atrophy of the right temporal lobe has recently been described as a controversial entity reported as the right temporal variant of FTD.


2018 ◽  
Vol 25 (2) ◽  
pp. 184-194 ◽  
Author(s):  
Gayathri Cheran ◽  
Liwen Wu ◽  
Seonjoo Lee ◽  
Masood Manoochehri ◽  
Sarah Cines ◽  
...  

AbstractObjectives: The cognitive indicators of preclinical behavioral variant Frontotemporal Dementia (bvFTD) have not been identified. To investigate these indicators, we compared cross-sectional performance on a range of cognitive measures in 12 carriers of pathogenic MAPT mutations not meeting diagnostic criteria for bvFTD (i.e., preclinical) versus 32 demographically-matched familial non-carriers (n = 44). Studying preclinical carriers offers a rare glimpse into emergent disease, environmentally and genetically contextualized through comparison to familial controls. Methods: Evaluating personnel blinded to carrier status administered a standardized neuropsychological battery assessing attention, speed, executive function, language, memory, spatial ability, and social cognition. Results from mixed effect modeling were corrected for multiplicity of comparison by the false discovery rate method, and results were considered significant at p < .05. To control for potential interfamilial variation arising from enrollment of six families, family was treated as a random effect, while carrier status, age, gender, and education were treated as fixed effects. Results: Group differences were detected in 17 of 31 cognitive scores and spanned all domains except spatial ability. As hypothesized, carriers performed worse on specific measures of executive function, and social cognition, but also on measures of attention, speed, semantic processing, and memory storage and retrieval. Conclusions: Most notably, group differences arose on measures of memory storage, challenging long-standing ideas about the absence of amnestic features on neuropsychological testing in early bvFTD. Current findings provide important and clinically relevant information about specific measures that may be sensitive to early bvFTD, and advance understanding of neurocognitive changes that occur early in the disease. (JINS, 2019, 25, 184–194)


2021 ◽  
Vol 2 (1) ◽  
pp. e00153735
Author(s):  
Lucia Crivelli ◽  
Ismael-Luis Calandri ◽  
Belén Helou ◽  
Greta Keller ◽  
Marcela-Paula Fiol ◽  
...  

Cognitive impairment and deficits in social cognition (SC) are frequent in patients with multiple sclerosis (MS). The aim of the present work is to study SC in patients with early MS and to analyze its neuroanatomical correlation. Thirty-four patients with relapsing remitting MS, with ≤ 2 years of disease progression and EDSS and ≤2, and 30 healthy control subjects matched for age, sex, and educational level were recruited. Subjects performed a comprehensive neuropsychological assessment (Rao BRB). SC was assessed using the International Affective Picture System IAPS, The Eyes in the Mind Test, the Empathy Quotient, and the Faux Pas Test. The anatomical correlation of patients with deficits in social cognition was studied through brain MRI and voxel-based morphometric for which cortical reconstruction and volumetric segmentation were performed using Freesurfer processing software. Patients showed significant deficits in executive functions, verbal memory and language tests. SC assessment showed that patients presented greater difficulties in the Faux Pas Test (p = 0.023), The Mind in the Eyes Test (p = 0.014), and presented a positive bias in the interpretation of neutral images of the IAPS (P = 0.023). Furthermore, patients with CS deficits presented less cortical thickness in areas of the right supramarginal gyrus, pars opercularis, and anterior cingulum.


2021 ◽  
Vol 12 ◽  
Author(s):  
Lina Velilla ◽  
Jonathan Hernández ◽  
Margarita Giraldo-Chica ◽  
Edmarie Guzmán-Vélez ◽  
Yakeel Quiroz ◽  
...  

The differential diagnosis among the behavioral variant of frontotemporal dementia FTD (bvFTD) and the linguist one primary progressive aphasia (PPA) is challenging. Presentations of dementia type or variants dominated by personality change or aphasia are frequently misinterpreted as psychiatric illness, stroke, or other conditions. Therefore, it is important to identify cognitive tests that can distinguish the distinct FTD variants to reduce misdiagnosis and best tailor interventions. We aim to examine the discriminative capacity of the most frequently used cognitive tests in their Spanish version for the context of dementia evaluation as well as the qualitative aspects of the neuropsychological performance such as the frequency and type of errors, perseverations, and false positives that can best discriminate between bvFTD and PPA. We also described mood and behavioral profiles of participants with mild to moderate probable bvFTD and PPA. A total of 55 subjects were included in this cross-sectional study: 20 with PPA and 35 with bvFTD. All participants underwent standard dementia screening that included a medical history and physical examination, brain MRI, a semistructured caregiver interview, and neuropsychological testing. We found that bvFTD patients had worse performance in executive function tests, and the PPA presented with the lower performance in language tests and the global score of Mini-Mental State Examination (MMSE). After running the linear discriminant model, we found three functions of cognitive test and subtests combination and three functions made by the Montreal Cognitive Assessment (MoCA) language subtest and performance errors that predicted group belonging. Those functions were more capable to classify bvFTD cases rather than PPA. In conclusion, our study supports that the combination of an individual test of executive function and language, MoCA's subtest, and performance errors as well have good accuracy to discriminate between bvFTD and PPA.


2010 ◽  
Vol 16 (4) ◽  
pp. 687-694 ◽  
Author(s):  
EZEQUIEL GLEICHGERRCHT ◽  
TERESA TORRALVA ◽  
MARÍA ROCA ◽  
FACUNDO MANES

AbstractThe detection of executive deficits in early behavioral variant frontotemporal dementia (bvFTD) is crucial, as impairments in the executive domain constitute an important diagnostic feature of the newly proposed diagnostic criteria for bvFTD. Our group has recently demonstrated that classical executive tests fail to detect the executive deficits of a subgroup of early bvFTD patients. When administered an executive and social cognition battery (ESCB) that includes tasks that mimic everyday scenarios (e.g., affective decision-making, planning and organization, theory of mind), however, the performance of those bvFTD patients differed significantly from that of controls. One limitation of the ESCB is its lengthy nature (approximately 90 min). For this reason, the present study analyzes the usefulness of alternative shorter versions of this battery. We propose one particular two-task combination that demands approximately 30 min for its administration and scoring, and which presents similar discriminatory accuracy as that of the complete ESCB, while maintaining its significantly superior capacity to detect subtle executive deficits in bvFTD patients relative to classical executive tests. We suggest that, in clinical settings where tools, time, or human resources are scarce, this abbreviated ESCB may be useful in the detection of subtle yet impairing executive impairments of patients with bvFTD. (JINS, 2010,16, 687–694.)


Neurocase ◽  
2001 ◽  
Vol 7 (2) ◽  
pp. 123-130 ◽  
Author(s):  
Sinclair Lough ◽  
Carol Gregory ◽  
John R. Hodges

Neurology ◽  
2017 ◽  
Vol 89 (6) ◽  
pp. 570-577 ◽  
Author(s):  
Claire M. O'Connor ◽  
Ramon Landin-Romero ◽  
Lindy Clemson ◽  
Cassandra Kaizik ◽  
Naomi Daveson ◽  
...  

Objective:To identify distinct behavioral phenotypes of behavioral-variant frontotemporal dementia (bvFTD) and to elucidate differences in functional, neuroimaging, and progression to residential care placement.Methods:Eighty-eight patients with bvFTD were included in a cluster analysis applying levels of disinhibition and apathy (Cambridge Behavioural Inventory-Revised) to identify phenotypic subgroups. Between-group (Kruskal-Wallis, Mann-Whitney U) functional differences (Disability Assessment for Dementia) and time to residential care placement (survival analyses) were examined. Cortical thickness differences (whole-brain MRI) were analyzed in patients with bvFTD vs healthy controls (n = 30) and between phenotypic subgroups.Results:Four phenotypic subgroups were identified: primary severe apathy (n = 26), severe apathy and disinhibition (n = 26), mild apathy and disinhibition (n = 27), and primary severe disinhibition (n = 9). Patients with severely apathetic phenotypes were more functionally impaired and had more extensive brain atrophy than those with mild apathy or severe disinhibition alone. Further imaging analyses indicated that the right middle temporal region is critical for the development of disinhibition, an association that remains with disease progression and in the context of severe apathy. Finally, no difference in time to residential care admission was found between phenotypes.Conclusions:This study reveals that different clinical behavioral phenotypes of bvFTD have differing profiles of functional decline and distinct patterns of associated cortical changes. These findings emphasize the importance of apathy in functional impairment, highlight the role of the right temporal region in disinhibition, and suggest that disability may be a sensitive outcome measure for treatments targeting reduction of apathy. These phenotypes could also support understanding of prognosis and clinical management.


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