Degradation of cognitive timing mechanisms in behavioural variant frontotemporal dementia
Susie M.D. HenleyLaura DowneyJennifer M. NicholasKirsi M. KinnunenHannah L. GoldenAisling BuckleyColin MahoneySebastian J. Crutch
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Abstract:
The current study examined motor timing in frontotemporal dementia (FTD), which manifests as progressive deterioration in social, behavioural and cognitive functions. Twenty-patients fulfilling consensus clinical criteria for behavioural variant FTD (bvFTD), 11 patients fulfilling consensus clinical criteria for semantic-variant primary progressive aphasia (svPPA), four patients fulfilling criteria for nonfluent/agrammatic primary progressive aphasia (naPPA), eight patients fulfilling criteria for Alzheimer׳s disease (AD), and 31 controls were assessed on both an externally- and self-paced finger-tapping task requiring maintenance of a regular, 1500 ms beat over 50 taps. Grey and white matter correlates of deficits in motor timing were examined using voxel-based morphometry (VBM) and diffusion tensor imaging (DTI). bvFTD patients exhibited significant deficits in aspects of both externally- and self-paced tapping. Increased mean inter-response interval (faster than target tap time) in the self-paced task was associated with reduced grey matter volume in the cerebellum bilaterally, right middle temporal gyrus, and with increased axial diffusivity in the right superior longitudinal fasciculus, regions and tracts which have been suggested to be involved in a subcortical-cortical network of structures underlying timing abilities. This suggests that such structures can be affected in bvFTD, and that impaired motor timing may underlie some characteristics of the bvFTD phenotype.Keywords:
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The term frontotemporal dementia (FTD) describes a diverse group of clinical syndromes, including behavioral-variant FTD (bvFTD), nonfluent/agrammatic-variant primary progressive aphasia (nfvPPA), semantic-variant primary progressive aphasia (svPPA), FTD motor neuron disease (FTD-MND), progressive supranuclear palsy syndrome (PSP-S), and corticobasal syndrome (CBS). Although each of these syndromes may be distinguished by their respective disturbances in behavior, language, or motor function and characteristic imaging findings, they may present a diagnostic dilemma when encountered clinically. In this article, we review the clinical features, diagnostic criteria, pathology, genetics, and therapeutic interventions for FTD spectrum disorders.
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Behavioral variant frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA) are related dementias with different presenting symptoms, but with increasing symptom-overlap as they progress.Loss of insight is associated with early bvFTD, but not PPA.This study used the Frontal Behavioral Inventory (FBI) to compare patient and caregiver concepts of symptom presence and severity.BvFTD patients were found to have worse insight overall than PPA patients.However, the PPA group showed reduced insight into behavioral symptoms, and the bvFTD groups had intact insight into some language symptoms.Theoretical and clinical implications are discussed.
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Frontotemporal dementia (FTD) is a progressive neurodegenerative disease that can present with three different clinical syndromes: behavioural-variant frontotemporal dementia (bvFTD), associated with behavioural and executive deficits; non-fluent variant primary progressive aphasia (nfPPA), with progressive deficits in speech, grammar, and word output; and semantic variant primary progressive aphasia (svPPA), which is a progressive disorder of semantic knowledge and naming. The disease can mimic various psychiatric disorders and sometimes can be difficult to discriminate against other forms of dementia. Advances in clinical, imaging, and molecular characterisation have increased the accuracy of the diagnosis of frontotemporal dementia. Updated diagnostic criteria have been developed and are now widely used. Recognition and accurate diagnoses of FTD subtypes will aid the neurologist in the management of patients.
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