What Is the Life Expectancy in Frontotemporal Lobar Degeneration?

Onyike, Chiadi U.

doi:10.1159/000333347

Cited by 17 publications

(16 citation statements)

References 25 publications

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“…It has been designed to quantification capacities that are immediately pertinent to everyday living, thus it is informed by a disablement perspective (Barberger-Gateau et al, 2004; Barberger-Gateau, Fabrigoule, Amieva, Helmer, & Dartigues, 2002) in an “etiologically neutral” approach to dementia related functional decline. Preliminary data show inter-rater reliability of ratings of FTD severity to be better than reliability achieved using the CDR, with both systems showing comparable reliability for rating AD (Onyike et al, 2011). …”

Section: Measurementmentioning

confidence: 93%

“…Statistically defined thresholds were computed to define levels of severity. The Dementia Disability Rating (DDR) (Onyike et al, 2011), developed to measure disability in heterogeneous dementia phenotypes, takes a different approach. It has been designed to quantification capacities that are immediately pertinent to everyday living, thus it is informed by a disablement perspective (Barberger-Gateau et al, 2004; Barberger-Gateau, Fabrigoule, Amieva, Helmer, & Dartigues, 2002) in an “etiologically neutral” approach to dementia related functional decline.…”

Section: Measurementmentioning

confidence: 99%

“…Early survival analyses showed median survival from diagnosis of FTD to be 7-13 years in clinic cohorts and 6-8 years in neuropathology series (Onyike, 2011). The clinical studies find that SD has the longest median survival (12 years), and PNFA and bvFTD show comparable survival (9 years) (Nunnemann et al, 2011; Roberson et al, 2005).…”

Section: Life Expectancy and Survivalmentioning

confidence: 99%

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The epidemiology of frontotemporal dementia

Onyike

Diehl‐Schmid

2013

International Review of Psychiatry

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426

303

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Frontotemporal dementia, a heterogeneous neurodegenerative disorder, is a common cause of young-onset dementia (i.e., dementia developing in midlife or earlier). The estimated point prevalence is 15–22/100,000, and incidence 2.7–4.1/100,000. Some 25% are late-life onset cases. Population studies show nearly equal distribution by gender, which contrasts with myriad clinical and neuropathology reports. FTD is frequently familial and hereditary; five genetic loci for causal mutations have been identified, all showing 100% penetrance. Non-genetic risk factors for are yet to be identified. FTD shows poor life expectancy but with survival comparable to that of Alzheimer disease. Recent progress includes the formulation of up-to-date diagnostic criteria for the behavioral and language variants, and the development of new and urgently needed instruments for monitoring and staging the illness. There is still need for descriptive populations studies, to fill gaps in our knowledge about minority groups and developing regions. More pressing, however, is the need for reliable physiologic markers for disease. There is a present imperative to develop a translational science to form the conduit for transferring neurobiological discoveries and insights from bench to bedside.

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Section: Measurementmentioning

confidence: 93%

Section: Measurementmentioning

confidence: 99%

Section: Life Expectancy and Survivalmentioning

confidence: 99%

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The epidemiology of frontotemporal dementia

Onyike

Diehl‐Schmid

2013

International Review of Psychiatry

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426

303

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“…A 2011 summary [2] identified a range of 3- 14 years from illness onset (i.e., the putative time at which the earliest symptom signifying the illness appeared), varying with phenotype and differing between clinical cohorts (which used prospective observations) and autopsy samples (which were retrospective). The median survival was shown to be 7-13 years in clinical cohorts and 6-8 years in autopsy series, but these observations were not a quantitative analysis of the extant data and did not extend to all phenotypes.…”

Section: Introductionmentioning

confidence: 99%

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Kansal

Mareddy

Sloane

et al. 2016

Dement Geriatr Cogn Disord

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Background: Survival in frontotemporal dementia (FTD) is not well understood. We conducted a mixed effects meta-analysis of survival in FTD to examine phenotype differences and contributory factors. Methods: The PubMed, Medline, EMBASE, CINAHL, PsycINFO and Cochrane databases were searched for studies describing survival or natural history of behavioral variant FTD (bvFTD), progressive non-fluent aphasia (PNFA), semantic dementia (SD), FTD with amyotrophic lateral sclerosis (FTD-ALS), progressive supranuclear palsy and corticobasal degeneration. There were no language restrictions. Results: We included 27 studies (2,462 subjects). Aggregate mean and median survival were derived for each phenotype and, for comparison, Alzheimer's disease (AD) (using data from the selected studies). Survival was shortest in FTD-ALS (2.5 years). Mean survival was longest in bvFTD and PNFA (8 years) and median survival in SD (12 years). AD was comparable in survival to all except FTD-ALS. Age and sex did not affect survival; the education effect was equivocal. Heterogeneity in FTD survival was largely, but not wholly, explained by phenotypes. Conclusions: Survival differs for FTD phenotypes but, except for FTD-ALS, compares well to AD survival. Elucidating the potential causes of within-phenotype heterogeneity in survival (such as complicating features and comorbidities) may open up opportunities for tailored interventions.

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“…It has been estimated that median survival from diagnosis for YOD (derived from a study in sampling mainly AD and VaD cases) is 6 years 30 . Median survival from diagnosis of FTD is about 7-13 years in clinic cohorts and 6-8 years in neuropathological series 31 . Survival is much shorter in FTD-ALS, showing median survival of 27 months 32 .…”

Section: Methodsmentioning

confidence: 99%

Young-Onset Dementia Epidemiology Applied to Neuropsychiatry Practice

Devineni

Onyike

2015

Psychiatric Clinics of North America

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Synopsis There is epidemiological evidence that a substantial number of adults suffer YOD. The diversity of types and syndromes makes recognition and diagnosis difficult. An algorithmic approach to interpreting clinical data, informed by clinical epidemiology, integrates data pertaining to defining syndromes, and their chronology and tempo, family history, and other neuropsychiatric features and neurological signs, to reach a preliminary diagnosis and direct diagnostic tests and their interpretation. Screening for YOD in the psychiatric context is a rational process in which vigilance is combined with careful searches for ‘red flags’ that signal a neurodegenerative etiology.

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What Is the Life Expectancy in Frontotemporal Lobar Degeneration?

Cited by 17 publications

References 25 publications

The epidemiology of frontotemporal dementia

The epidemiology of frontotemporal dementia

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Young-Onset Dementia Epidemiology Applied to Neuropsychiatry Practice

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