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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Available from: Michael Hornberger
    • "Previous studies including data on language functions in bvFTD678910111213141516171819202122 are summarized inTable 1. Deficits in confrontation naming [7–9, 11, 19, 22], comprehension of single words [7, 18] [6, 10, 16], and more generalized semantic and language impairment [12, 14, 15, 17, 20] have been described in bvFTD. Regional frontotemporal atrophy in bvFTD often overlaps brain networks canonically concerned with language [2, 3] and available neuroanatomical evidence has implicated distributed frontal, temporal, and parietal circuitry in the genesis of language deficits in this syndrome [6, 8, 9, 19]. Taken together, this evidence suggests that bvFTD may lead to language dysfunction, particularly where there is a requirement for processing verbal associations, searching the verbal lexicon, or planning propositional utterances. "
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    ABSTRACT: Background: The language profile of behavioral variant frontotemporal dementia (bvFTD) remains to be fully defined. Objective: We aimed to quantify the extent of language deficits in this patient group. Methods: We assessed a cohort of patients with bvFTD (n = 24) in relation to patIents with semantic variant primary progressive aphasia (svPPA; n = 14), nonfluent variant primary progressive aphasia (nfvPPA; n = 18), and healthy age-matched individuals (n = 24) cross-sectionally and longitudinally using a comprehensive battery of language and general neuropsychological tests. Neuroanatomical associations of language performance were assessed using voxel-based morphometry of patients' brain magnetic resonance images. Results: Relative to healthy controls, and after accounting for nonverbal executive performance, patients with bvFTD showed deficits of noun and verb naming and single word comprehension, diminished spontaneous propositional speech, and deterioration in naming performance over time. Within the bvFTD group, patients with MAPT mutations had more severe impairments of noun naming and single word comprehension than patients with C9orf72 mutations. Overall the bvFTD group had less severe language deficits than patients with PPA, but showed a language profile that was qualitatively similar to svPPA. Neuroanatomical correlates of naming and word comprehension performance in bvFTD were identified predominantly in inferior frontal and antero-inferior temporal cortices within the dominant hemispheric language network. Conclusions: bvFTD is associated with a language profile including verbal semantic impairment that warrants further evaluation as a novel biomarker.
    No preview · Article · Dec 2015 · Journal of Alzheimer's disease: JAD
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    • "We did not employ language or chronological filters in the search. Titles and abstracts of the papers retrieved in the initial search were screened according to the following eligibility criteria:[1]original research,[2]case series, cohort or cross-sectional design, and[3]imaging methods (MRI, PET and/or SPECT). Abstracts with insufficient information, individual case reports and review articles were not included in the final selection. "

    Full-text · Article · Dec 2015 · Dementia e Neuropsychologia
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    • "© 2016 – IOS Press and the authors. All rights reserved chological gold standard to distinguish bvFTD from Alzheimer's disease at presentation [1] [2]. However, increasing evidence suggest that bvFTD patients can show episodic memory deficits [3], with a subgroup of bvFTD patients being impaired to a similar level as Alzheimer's disease, even in biologically and pathologically confirmed cases [4] [5]. "
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    ABSTRACT: Relative sparing of episodic memory is a diagnostic criterion of behavioral variant frontotemporal dementia (bvFTD). However, increasing evidence suggests that bvFTD patients can show episodic memory deficits at a similar level as Alzheimer’s disease (AD). Social cognition tasks have been proposed to distinguish bvFTD, but no study to date has explored the utility of such tasks for the diagnosis of amnestic bvFTD. Here, we contrasted social cognition performance of amnestic and non-amnestic bvFTD from AD, with a subgroup having confirmed in vivo pathology markers. Ninety-six participants (38 bvFTD and 28 AD patients as well as 30 controls) performed the short Social-cognition and Emotional Assessment (mini-SEA). BvFTD patients were divided into amnestic versus non-amnestic presentation using the validated Free and Cued Selective Reminding Test (FCSRT) assessing episodic memory. As expected, the accuracy of the FCSRT to distinguish the overall bvFTD group from AD was low (69.7%) with ∼50% of bvFTD patients being amnestic. By contrast, the diagnostic accuracy of the mini-SEA was high (87.9%). When bvFTD patients were split on the level of amnesia, mini-SEA diagnostic accuracy remained high (85.1%) for amnestic bvFTD versus AD and increased to very high (93.9%) for non-amnestic bvFTD versus AD. Social cognition deficits can distinguish bvFTD and AD regardless of amnesia to a high degree and provide a simple way to distinguish both diseases at presentation. These findings have clear implications for the diagnostic criteria of bvFTD. They suggest that the emphasis should be on social cognition deficits with episodic memory deficits not being a helpful diagnostic criterion in bvFTD.
    Full-text · Article · Nov 2015 · Journal of Alzheimer's disease: JAD
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