Neuropsychiatric Aspects of Frontotemporal Dementia
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Keywords:
Primary progressive aphasia
Semantic dementia
Corticobasal degeneration
Pedigrees from 269 patients with frontotemporal lobar degeneration (FTLD), including frontotemporal dementia (FTD), FTD with ALS (FTD/ALS), progressive nonfluent aphasia, semantic dementia (SD), corticobasal degeneration, and progressive supranuclear palsy were analyzed to determine the degree of heritability of these disorders. FTD/ALS was the most and SD the least heritable subtype. FTLD syndromes appear to have different etiologies and recurrence risks.
Corticobasal degeneration
Semantic dementia
Frontotemporal lobar degeneration
Primary progressive aphasia
Etiology
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Abstract The syndromes caused by frontotemporal lobar degeneration have highly heterogeneous and overlapping clinical features. There has been great progress in the refinement of clinical diagnostic criteria in the past decade, but we propose that a better understanding of aetiology, pathophysiology and symptomatic treatments can arise from a transdiagnostic approach to clinical phenotype and brain morphometry. In a cross-sectional epidemiological study, we examined 310 patients with a syndrome likely to be caused by frontotemporal lobar degeneration, including behavioural variant frontotemporal dementia, non-fluent, and semantic variants of primary progressive aphasia (PPA), progressive supranuclear palsy and corticobasal syndrome. We included patients with logopenic PPA and those who met criteria for PPA but not a specific subtype. To date, 49 patients have a neuropathological diagnosis. A principal component analysis identified symptom dimensions that broadly recapitulated the core features of the main clinical syndromes. However, the subject-specific scores on these dimensions showed considerable overlap across the diagnostic groups. Sixty-two per cent of participants had phenotypic features that met the diagnostic criteria for more than one syndrome. Behavioural disturbance was prevalent in all groups. Forty-four per cent of patients with corticobasal syndrome had progressive supranuclear palsy-like features and 30% of patients with progressive supranuclear palsy had corticobasal syndrome-like features. Many patients with progressive supranuclear palsy and corticobasal syndrome had language impairments consistent with non-fluent variant PPA while patients with behavioural variant frontotemporal dementia often had semantic impairments. Using multivariate source-based morphometry on a subset of patients (n = 133), we identified patterns of covarying brain atrophy that were represented across the diagnostic groups. Canonical correlation analysis of clinical and imaging components found three key brain-behaviour relationships, with a continuous spectrum across the cohort rather than discrete diagnostic entities. In the 46 patients with follow-up (mean 3.6 years) syndromic overlap increased with time. Together, these results show that syndromes associated with frontotemporal lobar degeneration do not form discrete mutually exclusive categories from their clinical features or structural brain changes, but instead exist in a multidimensional spectrum. Patients often manifest diagnostic features of multiple disorders while deficits in behaviour, movement and language domains are not confined to specific diagnostic groups. It is important to recognize individual differences in clinical phenotype, both for clinical management and to understand pathogenic mechanisms. We suggest that a transdiagnostic approach to the spectrum of frontotemporal lobar degeneration syndromes provides a useful framework with which to understand disease aetiology, progression, and heterogeneity and to target future treatments to a higher proportion of patients.
Corticobasal degeneration
Semantic dementia
Frontotemporal lobar degeneration
Primary progressive aphasia
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Objective: To compare the behavioral profiles in different variants of primary progressive aphasia (PPA). Methods: We classified 67 patients with PPA into three clinical variants: semantic dementia (SEMD), progressive nonfluent aphasia (PNFA), and logopenic progressive aphasia (LPA), and we compared the severity of behavioral dysfunction, as measured by the Neuropsychiatric Inventory, in these groups and patients with frontotemporal dementia (FTD) and Alzheimer disease (AD). Results: SEMD was associated with significantly more socioemotional behavioral dysfunction than the other two variants of PPA and than AD, specifically more disinhibition, aberrant motor behavior, and eating disorders—behaviors that are typical of FTD. In contrast, PNFA and LPA did not differ from each other or from AD in the type or severity of behavioral dysfunction. Behavioral abnormalities increased in severity with disease duration in SEMD, but this association was not detected in PNFA or LPA. Conclusions: Semantic dementia is associated with significantly more behavioral dysfunction than other variants of primary progressive aphasia, specifically behavioral features typical of frontotemporal dementia.
Semantic dementia
Primary progressive aphasia
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Frontotemporal lobar degeneration
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Not so long ago many neurologists were trained to believe that Pick's disease was rare and usually impossible to differentiate accurately from Alzheimer's disease. Through the persistent efforts of several research groups, especially in Lund (Sweden) and Manchester (UK), it became clear that this ‘disease’ is not rare and can frequently be distinguished from Alzheimer's disease (Brun et al. , 1994). Now, this entity is better described as frontotemporal dementia (FTD) or frontotemporal lobar degeneration based on the frequent occurrence of lobar atrophy involving frontal and/or temporal lobes (Snowden et al. , 1996). And rather than constituting a single disease, FTD represents a group of overlapping disorders having different pathogenic mechanisms, some, but not all, of which have been identified.
There are several different ways to subdivide the various types of FTD. One is to use clinical manifestations such as the behavioural or language variants (such as semantic dementia and many cases of primary progressive aphasia) and FTD associated with motor neuron disease. The second type of subdivision is made on the basis of neuropathological features. This …
Primary progressive aphasia
Semantic dementia
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Pick's disease
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This article provides an overview of the historical entity hitherto, the pivotal clinical symptoms of FTLD (frontotemporal lobar degeneration), and an introduction for the revised criteria for bvFTD (behavioral variant frontotemporal dementia): FTDC (International Behavioral Variant FTD Criteria Consortium) presented by Rascovsky et al(2011), and the classification criteria for PPA(primary progressive aphasia) heralded by Gorno-Tempini et al (2011). According to the former criteria, bvFTD can be diagnosed by the clinical symptoms as having possible bvFTD or probable bvFTD, and the pathological findings could lead definite bvFTD. In the latter classification criteria of PPA, two types are implicated in FTLD: one is the non-fluent/agrammatic variant PPA(PNFA/nfvPPA/naPPA), and the other is semantic variant PPA(SD/svPPA).
Primary progressive aphasia
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Semantic dementia
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Primary progressive aphasia
Semantic dementia
Frontotemporal lobar degeneration
Tauopathy
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Primary progressive aphasia
Semantic dementia
Frontotemporal lobar degeneration
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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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ABSTRACT “Frontotemporal dementia” (FTD) is a clinical syndrome characterized by the focal involvement of the frontal and/or temporal lobes. FTD has three clinical phenotypes: the behavioral variant and two linguistic subtypes, namely, non-fluent/agrammatic primary progressive aphasia (PPA-NF/A) and semantic PPA (PPA-S). FTD is the second most common cause of dementia in individuals under the age of 65 years. This article presents recommendations for the diagnosis of FTD in the Brazilian scenario, considering the three levels of complexity of the health system: primary health care, secondary and tertiary levels. Diagnostic guidelines are proposed, including cognitive testing, behavioral and language assessments, laboratory tests, and neuroimaging.
Primary progressive aphasia
Semantic dementia
Behavioral neurology
Cognitive Decline
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Frontotemporal dementia (FTD) syndromes comprise a heterogeneous group of neurodegenerative conditions characterized by atrophy in the frontal and temporal lobes. Three main clinical variants are recognized: Behavioral variant (bv-FTD), Semantic dementia (SD), and Progressive nonfluent aphasia (PNFA). However, logopenic/phonological (LPA) variant has been recently described, showing a distinctive pattern of brain atrophy and often associated to Alzheimer's disease pathology. The diagnosis of FTD is challenging, since there is clinical, pathological, and genetic overlap between the variants and other neurodegenerative diseases, such as motoneuron disease (MND) and corticobasal degeneration (CBD). In addition, patients with gene mutations (tau and progranulin) display an inconsistent clinical phenotype and the correspondence between the clinical variant and its pathology is unpredictable. New cognitive tests based on social cognition and emotional recognition together with advances in molecular pathology and genetics have contributed to an improved understanding. There is now a real possibility of accurate biomarkers for early diagnosis. The present review concentrates on new insights and debates in FTD.
Corticobasal degeneration
Semantic dementia
Primary progressive aphasia
Frontotemporal lobar degeneration
Molecular Pathology
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