Behavioural-variant frontotemporal dementia: Diagnosis, clinical staging, and management

Neuroscience Research Australia, Randwick, NSW, Australia.
The Lancet Neurology (Impact Factor: 21.9). 02/2011; 10(2):162-72. DOI: 10.1016/S1474-4422(10)70299-4
Source: PubMed


Patients with behavioural-variant frontotemporal dementia (bvFTD) present with insidious changes in personality and interpersonal conduct that indicate progressive disintegration of the neural circuits involved in social cognition, emotion regulation, motivation, and decision making. The underlying pathological changes are heterogeneous and are characterised by various intraneuronal inclusions. Biomarkers to detect these histopathological changes in life are becoming increasingly important with the development of disease-modifying drugs. Gene mutations have been found that collectively account for around 10-20% of cases. Recently, criteria proposed for bvFTD define three levels of diagnostic certainty: possible, probable, and definite. Detailed history taking from family members to elicit behavioural features underpins the diagnostic process, with support from neuropsychological testing designed to detect impairment in decision making, emotion processing, and social cognition. Brain imaging is important for increasing the level of diagnostic certainty. A recently developed staging instrument shows much promise for monitoring patients and evaluating therapies, which at present are aimed at symptom amelioration. Carer education and support remain of paramount importance.

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    • "Higher levels of burden are reported in caregivers of patients with frontotemporal dementia [2] [3]—a neurodegenerative disease resulting in an younger-onset dementia characterized clinically by the presence of striking changes in personality and behavior as well as marked loss of language comprehension and speech—than in comparison to Alzheimer's disease (AD), the most common type of dementia typically characterized in the early stages of the disease by loss of memory and disorientation [4]. Frontotemporal dementia (FTD) presents with clinical variants: behavioral-variant FTD (bvFTD) who show progressive and debilitating changes primarily in interpersonal skills and behavior [5], whereas one of the language presentations, semantic dementia (SD), is a coherent clinic-pathological entity, that present with an insidious decline in language skills which are initially circumscribed [6]. Within the FTD variants, caregivers of bvFTD patients experience higher levels of burden than the language-variants of FTD [7], with levels of burden in FTD associated with patient variables such as the severity of abnormal behaviors [4] [8] [9]. "
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    ABSTRACT: Background and aims: Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) represent a disease spectrum. Caregiver burden in subtypes of FTD has not yet been directly compared with those patients who have co-existent FTD and ALS (ALSFTD). Method: Perceived caregiver burden was evaluated using the short Zarit Burden Interview (ZBI) in patients with behavioral-variant FTD (bvFTD, n = 21), semantic dementia (SD, n = 18), and ALSFTD (n = 15) at the initial clinical presentation and follow-up assessments. The Mini-Addenbrooke's Cognitive Examination (M-ACE) and the Motor Neuron Disease Behaviour Scale (MiND-B) were also used. Linear mixed effects models examined longitudinal changes on the ZBI, M-ACE, and MiND-B across groups. Results: Burden at baseline was highest for the bvFTD group. Longitudinally, perceived burden increased for the SD and ALSFTD groups whereas in bvFTD, the level of burden which was high at baseline and remained high with disease progression. The severity of abnormal behaviors at baseline, as assessed by the MiND-B, correlated with baseline levels of caregiver burden and further accounted for 23% of the variance in caregiver burden at clinical follow-up. Conclusions: The trajectory of perceived burden differs across the FTD-ALS spectrum, with SD and ALSFTD caregivers demonstrating an increased burden that develops over time, compared to a persistently high level for bvFTD caregivers, evident throughout the disease course. The evolution of burden in these three syndromes likely reflects the initial presentation and clinical characterization that develops with time. Psycho-education programs for caregivers, which provide better coping strategies for challenging behaviors, may reduce levels of burden experienced with disease progression.
    Journal of Alzheimer's disease: JAD 10/2015; DOI:10.3233/JAD-150475 · 4.15 Impact Factor
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    • "The behavioural variant of frontotemporal dementia (FTD), a neurodegenerative condition with insidious, progressive change in personality and social interactions, represents the prototypical example of disordered social norm compliance. Patients commonly exhibit behavioural changes considered under this rubric, including loss of empathy and insight, disinhibited remarks or behaviour, egocentricity , impulsive spending or gambling, and gullibility (Piguet et al., 2011). Intriguingly, the earliest sites of pathology overlap with those regions implicated in social norm compliance, most notably in the ventromedial prefrontal cortex, anterior cingulate, insula, amygdalae and striatum (Broe et al., 2003; Seeley et al., 2008; O'Callaghan et al., 2014; Bertoux et al., 2015). "
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    ABSTRACT: Adherence to social norms is compromised in a variety of neuropsychiatric conditions. Functional neuroimaging studies have investigated social norm compliance in healthy individuals, leading to the identification of a network of fronto-subcortical regions that underpins this ability. However, there is a lack of corroborative evidence from human lesion models investigating the struc- tural anatomy of norm compliance across this fronto-subcortical network. To address this, we developed a neuroeconomic task to investigate social norm compliance in a neurodegenerative lesion model: behavioural variant frontotemporal dementia, a condition characterized by gross social dysfunction. The task assessed norm compliance across three behaviours that are well-studied in the neuroeconomics literature: fairness, prosocial and punishing behaviours. We administered our novel version of the Ultimatum Game in 22 patients with behavioural variant frontotemporal dementia and 22 age-matched controls, to assess how decision- making behaviour was modulated in response to (i) fairness of monetary offers; and (ii) social context of monetary offers designed to produce either prosocial or punishing behaviours. Voxel-based morphometry was used to characterize patterns of grey matter atrophy associated with task performance. Acceptance rates between patients and controls were equivalent when only fairness was manipulated. However, patients were impaired in modulating their decisions in response to social contextual information. Patients’ performance in the punishment condition was consistent with a reduced tendency to engage in punishment; this was associated with decreased grey matter volume in the anterior cingulate, orbitofrontal cortex, left dorsolateral prefrontal cortex and right inferior frontal gyrus. In the prosocial condition, patients’ performance suggested a reduced expression of prosocial behaviour, associated with decreased grey matter in the anterior insula, lateral orbitofrontal cortex, anterior cingulate and dorsal striatum. Acceptance rates in the Ultimatum Game were also significantly related to impairments in the everyday expression of empathic concern. In conclusion, we demonstrate that compliance to basic social norms (fairness) can be maintained in behavioural variant frontotemporal dementia; however, more complex normative behaviours (prosociality, punishment) that require integration of social contextual information are disrupted in association with atrophy in key fronto-striatal regions. These results suggest that the integration of social contextual information to guide normative behaviour is uniquely impaired in behavioural variant frontotem- poral dementia, and may explain other common features of the condition including gullibility and impaired empathy. Our findings also converge with previous functional neuroimaging investigations in healthy individuals and provide the first description of the structural anatomy of social norm compliance in a neurodegenerative lesion model.
    Brain 10/2015; DOI:10.1093/brain/awv315 · 9.20 Impact Factor
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    • "individuals with Alzheimer's disease ), and there is evidence that these services do not fit the needs of people with FTD and their families (Beattie et al. 2004, Nunnemann et al. 2012, Shnall et al. 2013). Currently, no specialised interventions are available for people with FTD (Massimo & Grossman 2008, Mendez 2009, Piguet et al. 2011). There is inconsistent evidence of the benefit of pharmacological interventions for this group, and psycho-social interventions , which are postulated to be the most effective intervention for dementia, have not yet been either developed systematically or studied using people with FTD (Piguet et al. 2011, Warren et al. 2013). "
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    ABSTRACT: Behavioural variant frontotemporal dementia (bvFTD) is an early-onset and progressive neurodegenerative disease associated with strong changes in judgement, behaviour, personality and emotions. These changes can cause significant problems in everyday life for people with bvFTD and their families, and have implications for health and society. Currently, there are no suitable evidence-based specific interventions for people with bvFTD. This scoping review aims to identify the self-expressed needs, demands and coping strategies of people with bvFTD. Identifying these issues is the first step towards the development of need-based psycho-social interventions for people with bvFTD. A comprehensive literature research was conducted of German and English scientific articles published between January 2000 and October 2014 using the databases MEDLINE, CINAHL, PsycINFO, PSYNDEX, SocINDEX, GeroLit, the Cochrane Library, ProQuest, the German National Library and additional search strategies in terms of a scoping review. Articles were identified by combining search terms related to 'frontotemporal dementia' with terms related to 'self-expressions', 'needs/demands' and 'coping'. After excluding duplicates, two independent reviewers screened the titles and abstracts of 2317 records for eligibility. Because eligibility could not be assessed from the titles or abstracts of 28 articles, those articles were assessed using the full text. One poster abstract met our research question and a few articles were related, but no article met all of the inclusion criteria. This lack of scientifically based knowledge concerning the perspective of people with bvFTD is discussed with reference to the search strategy and the research questions, disease-related aspects such as changes in behaviour or language and emotions and the difficulties in researching this topic. Recommendations are formulated for future research considering the perspective of people with bvFTD and that will involve the development of appropriate data collection methods. Subsequently, specialised interventions must be developed. © 2015 John Wiley & Sons Ltd.
    Health & Social Care in the Community 04/2015; DOI:10.1111/hsc.12225 · 1.15 Impact Factor
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