Abstract:Background and purpose
Predicting the course of behavioural variant frontotemporal dementia (bvFTD) remains a major clinical challenge. This study aimed to identify factors that predict survival and clinical progression in bvFTD.
Methods
Consecutive patients with clinically probable bvFTD were prospectively followed up over an 8‐year period. Baseline neuropsychological variables, presence of a known pathogenic frontotemporal dementia gene mutation and a systematic visual magnetic resonance imaging assessment a… Show more
“…With disease progression many patients with nfvPPA develop the phenotype of PSP or CBS, which is an adverse prognostic sign [44][45][46][47]. In keeping with these observations, previous survival analyses of frontotemporal dementia (bvFTD and PPA) have shown that reduced letter fluency, motor cortex atrophy and brainstem hypoperfusion were associated with reduced survival [5,48,49]. Our results go beyond these findings, suggesting that development of motor impairments, irrespective of diagnostic group, is an adverse prognostic sign.…”
Section: Discussionsupporting
confidence: 89%
“…Prognosis in syndromes associated with frontotemporal lobar degeneration (FTLD) is highly variable and difficult to predict. Disease duration is not fully explained by the diagnostic categorisation to behavioural variant frontotemporal dementia (bvFTD), non-fluent (nfvPPA) or semantic (svPPA) variants of primary progressive aphasia, progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS) [1][2][3][4][5]. Better prognostic models would aid both trial design and clinical management.…”
Section: Introductionmentioning
confidence: 99%
“…Mortality is a definite endpoint in FTD, PSP and CBS. However, these disorders also engender dependency and caregiver burden [5,[12][13][14][15]. Community-based studies suggest increased dependency, due to cognitive or physical impairment, predicts care home admission [16,17].…”
Objective
To test the hypothesis that in syndromes associated with frontotemporal lobar degeneration, behavioural impairment predicts loss of functional independence and motor clinical features predict mortality, irrespective of syndrome subtype.
Method
We used a transdiagnostic approach to survival in an epidemiological cohort, testing the association between clinical features, independence and survival in patients with clinical diagnoses of behavioural variant frontotemporal dementia (n=64), non-fluent variant primary progressive aphasia (n=36), semantic variant primary progressive aphasia (n=25), progressive supranuclear palsy (n=101) and corticobasal syndrome (n=68). A principal components analysis identified six dimensions of clinical features. Using Cox proportional hazards and logistic regression we identified the association between each of these dimensions and functionally independent survival (time from clinical assessment to care home admission), and absolute survival (time to death). Analyses adjusted for the covariates of age, gender and diagnostic group. Secondary analysis excluded specific diagnostic groups.
Results
Behavioural disturbance, including impulsivity and apathy, was associated with reduced functionally independent survival (OR 2.46, p<0.001), even if patients with bvFTD were removed from the analysis. Motor impairments were associated with reduced absolute survival, even if patients with PSP and CBS were removed from the analysis.
Conclusion
Our results may help individualised prognostication and planning of disease-modifying trials and support a transdiagnostic approach to symptomatic treatment trials in patients with clinical syndromes associated with frontotemporal lobar degeneration.
“…With disease progression many patients with nfvPPA develop the phenotype of PSP or CBS, which is an adverse prognostic sign [44][45][46][47]. In keeping with these observations, previous survival analyses of frontotemporal dementia (bvFTD and PPA) have shown that reduced letter fluency, motor cortex atrophy and brainstem hypoperfusion were associated with reduced survival [5,48,49]. Our results go beyond these findings, suggesting that development of motor impairments, irrespective of diagnostic group, is an adverse prognostic sign.…”
Section: Discussionsupporting
confidence: 89%
“…Prognosis in syndromes associated with frontotemporal lobar degeneration (FTLD) is highly variable and difficult to predict. Disease duration is not fully explained by the diagnostic categorisation to behavioural variant frontotemporal dementia (bvFTD), non-fluent (nfvPPA) or semantic (svPPA) variants of primary progressive aphasia, progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS) [1][2][3][4][5]. Better prognostic models would aid both trial design and clinical management.…”
Section: Introductionmentioning
confidence: 99%
“…Mortality is a definite endpoint in FTD, PSP and CBS. However, these disorders also engender dependency and caregiver burden [5,[12][13][14][15]. Community-based studies suggest increased dependency, due to cognitive or physical impairment, predicts care home admission [16,17].…”
Objective
To test the hypothesis that in syndromes associated with frontotemporal lobar degeneration, behavioural impairment predicts loss of functional independence and motor clinical features predict mortality, irrespective of syndrome subtype.
Method
We used a transdiagnostic approach to survival in an epidemiological cohort, testing the association between clinical features, independence and survival in patients with clinical diagnoses of behavioural variant frontotemporal dementia (n=64), non-fluent variant primary progressive aphasia (n=36), semantic variant primary progressive aphasia (n=25), progressive supranuclear palsy (n=101) and corticobasal syndrome (n=68). A principal components analysis identified six dimensions of clinical features. Using Cox proportional hazards and logistic regression we identified the association between each of these dimensions and functionally independent survival (time from clinical assessment to care home admission), and absolute survival (time to death). Analyses adjusted for the covariates of age, gender and diagnostic group. Secondary analysis excluded specific diagnostic groups.
Results
Behavioural disturbance, including impulsivity and apathy, was associated with reduced functionally independent survival (OR 2.46, p<0.001), even if patients with bvFTD were removed from the analysis. Motor impairments were associated with reduced absolute survival, even if patients with PSP and CBS were removed from the analysis.
Conclusion
Our results may help individualised prognostication and planning of disease-modifying trials and support a transdiagnostic approach to symptomatic treatment trials in patients with clinical syndromes associated with frontotemporal lobar degeneration.
“…Второе место после БКЯ в группе деменций с быстрым прогрессированием почти единодушно отводится лобно-височной деменции (ЛВД) и, прежде всего, ее поведенческому варианту [58][59][60][61][62]. Однако исследователи признают, что верификация диагноза ЛВД нередко следует за диагнозом психического заболевания, что искажает представление о скорости прогрессировании болезни [58,63].…”
Section: нозологические различия в скорости прогрессирования деменцииunclassified
“…По мнению авторов, они отражают бóльшую скорость во многом естественного прогрессирования ЛВД в сопоставлении с БА. Дефицит беглости письменной речи и более выраженная атрофия в двигательных отделах коры также рассматриваются в качестве предикторов быстрого прогрессирования ЛВД [62].…”
Section: нозологические различия в скорости прогрессирования деменцииunclassified
Background: the clinical experience testifies to the fact, that the increase of dementia severity in late age occurs quickly in some cases and in others it proceeds slowly, which is reflected in the total duration of the disease and survival. One of the aspects of dementias research is the study of significant differences in dementia progression rates.The objective of the review was to generalize the obtained data on progression rates of late age dementias of various genesis, first of all due to Alzheimer’s disease and its associated disorders.Material and methods: papers in MedLine/PubMed bases from1990 to 2020 were selected and analyzed according to the key words: “old age”, “dementia”, “Alzheimer’s disease”, “vascular dementia”, “progression”, “progression rate”, “disease trajectories”, as well as relevant papers in the references of the analyzed works.Conclusion: the history of research of old age dementias natural course was presented in the review of scientific publications. According to the results of development of progression rates detection methods, singling out of dementias with rapid and slow increase in the severity of cognitive decline was substantiated. Works devoted to the study of frequency and nosological belonging of dementias with different progression rates were considered. In the most recent studies prognostic models with detection of various trajectories of the course of the disease were developed. The concept of various dementias progression rates admittedly has practical meaning for provision of diagnostic and treatment assistance and planning of medical and social support measures for patients with dementia and their families. Differentiation of dementia progression clinical patterns during formation of comparable groups of patients seems appropriate for investigation of new therapy methods, as well as in clinical-biological studies of pathogenesis.
IMPORTANCE Behavioral disturbances are core features of frontotemporal dementia (FTD); however, symptom progression across the course of disease is not well characterized in genetic FTD.OBJECTIVE To investigate behavioral symptom frequency and severity and their evolution and progression in different forms of genetic FTD.
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