Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

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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Illustrative Cases

10.1093/med/9780198749189.003.0008 ◽

2017 ◽

Author(s):

John R. Hodges

Keyword(s):

Cognitive Impairment ◽

Mild Cognitive Impairment ◽

Progressive Supranuclear Palsy ◽

Frontotemporal Dementia ◽

Corticobasal Degeneration ◽

Semantic Dementia ◽

Case Histories ◽

The Family ◽

Fluent Aphasia ◽

Addenbrooke’S Cognitive Examination

This chapter comprises 16 case histories that illustrate methods of assessment described in the rest of this book and the use of the Addenbrooke’s Cognitive Examination (ACE)-III. Each case begins with a brief history from the patient and observations by the family followed by findings on cognitive examination focusing on the profile shown on the ACE-III, the results of imaging investigations, and a discussion of the diagnosis and its differential, with a final summary of the principal conclusions, indicating whether the services of a neuropsychologist are required or not. The cases present important common conditions (such as mild cognitive impairment, Alzheimer’s disease in the mild and moderate stages, behavioural variant frontotemporal dementia, progressive non-fluent aphasia, semantic dementia, corticobasal degeneration, progressive supranuclear palsy, and Huntington’s disease) as well as interesting neuropsychological syndromes (such as prosopagnosia, amnestic stoke, and transient epileptic amnesia).

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C9orf72andUNC13Aare shared risk loci for amyotrophic lateral sclerosis and frontotemporal dementia: A genome-wide meta-analysis

Annals of Neurology ◽

10.1002/ana.24198 ◽

2014 ◽

Vol 76 (1) ◽

pp. 120-133 ◽

Cited By ~ 43

Author(s):

Frank P. Diekstra ◽

Vivianna M. Van Deerlin ◽

John C. van Swieten ◽

Ammar Al-Chalabi ◽

Albert C. Ludolph ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Frontotemporal Dementia ◽

Meta Analysis ◽

Genome Wide ◽

A Genome ◽

Shared Risk ◽

Lateral Sclerosis

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History of English as punctuated equilibria? A meta-analysis of the rate of linguistic change in Middle English

Journal of Historical Sociolinguistics ◽

10.1515/jhsl-2019-0008 ◽

2020 ◽

Vol 6 (2) ◽

Author(s):

Terttu Nevalainen ◽

Tanja Säily ◽

Turo Vartiainen ◽

Aatu Liimatta ◽

Jefrey Lijffijt

Keyword(s):

Middle English ◽

Language Change ◽

Meta Analysis ◽

Early Modern Period ◽

Norman Conquest ◽

Linguistic Change ◽

Punctuated Equilibria ◽

Late Medieval Period ◽

History Of ◽

Using Data

AbstractIn this paper, we explore the rate of language change in the history of English. Our main focus is on detecting periods of accelerated change in Middle English (1150–1500), but we also compare the Middle English data with the Early Modern period (1500–1700) in order to establish a longer diachrony for the pace at which English has changed over time. Our study is based on a meta-analysis of existing corpus research, which is made available through a new linguistic resource, the Language Change Database (LCD). By aggregating the rates of 44 individual changes, we provide a critical assessment of how well the theory of punctuated equilibria (Dixon, Robert M. W. 1997. The rise and fall of languages. Cambridge: Cambridge University Press) fits with our results. More specifically, by comparing the rate of language change with major language-external events, such as the Norman Conquest and the Black Death, we provide the first corpus-based meta-analysis of whether these events, which had significant societal consequences, also had an impact on the rate of language change. Our results indicate that major changes in the rate of linguistic change in the late medieval period could indeed be connected to the social and cultural after-effects of the Norman Conquest. We also make a methodological contribution to the field of English historical linguistics: by re-using data from existing research, linguists can start to ask new, fundamental questions about the ways in which language change progresses.

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The Association betweenC9orf72Repeats and Risk of Alzheimer’s Disease and Amyotrophic Lateral Sclerosis: A Meta-Analysis

Parkinson s Disease ◽

10.1155/2016/5731734 ◽

2016 ◽

Vol 2016 ◽

pp. 1-8 ◽

Cited By ~ 2

Author(s):

Li Shu ◽

Qiying Sun ◽

Yuan Zhang ◽

Qian Xu ◽

Jifeng Guo ◽

...

Keyword(s):

Alzheimer’S Disease ◽

Alzheimer's Disease ◽

Amyotrophic Lateral Sclerosis ◽

Frontotemporal Dementia ◽

Meta Analysis ◽

Sporadic Als ◽

Repeat Expansions ◽

Positive Correlations ◽

The Relationship ◽

Lateral Sclerosis

C9orf72is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) in Caucasian populations. However, the relationship betweenC9orf72repeats and Alzheimer’s disease (AD) was not clear. Additionally, there were few articles assessingC9orf72in other ethnicities with ALS. In this meta-analysis, we aimed to investigate the relationship betweenC9orf72repeat expansions (≥30 repeats) and intermediate repeat copies (20–29 repeats) and AD or ALS. The results suggested positive correlations betweenC9orf72repeat expansions and the risk of Alzheimer’s disease (OR = 6.36, 95% CI = 3.13–12.92, andp<0.00001), while intermediate repeat copies ofC9orf72gene were not associated with the risk of the disease.C9orf72repeat expansions were positively correlated with the risk of familial and sporadic ALS (OR = 293.25, 95% CI = 148.17–580.38, andp<0.00001; OR = 35.57, 95% CI = 19.61–64.51, andp<0.00001). There was a positive correlation between the gene variations and ALS risk among Caucasians and Asians (OR = 57.56, 95% CI = 36.73–90.22, andp<0.00001; OR = 6.35, 95% CI = 1.39–29.02, andp=0.02).

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Association between TBK1 mutations and risk of amyotrophic lateral sclerosis/frontotemporal dementia spectrum: a meta-analysis

Neurological Sciences ◽

10.1007/s10072-018-3246-0 ◽

2018 ◽

Vol 39 (5) ◽

pp. 811-820 ◽

Cited By ~ 18

Author(s):

Rongrong Cui ◽

Miao Tuo ◽

Pengfei Li ◽

Chang Zhou

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Frontotemporal Dementia ◽

Meta Analysis ◽

Lateral Sclerosis

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A case of progressive non-fluent aphasia as onset of amyotrophic lateral sclerosis with frontotemporal dementia

International Journal of Neuroscience ◽

10.1080/00207454.2018.1516657 ◽

2019 ◽

Vol 129 (7) ◽

pp. 719-721 ◽

Cited By ~ 1

Author(s):

F. De Marchi ◽

G. Tondo ◽

M.F. Sarnelli ◽

L. Corrado ◽

V. Solara ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Frontotemporal Dementia ◽

Fluent Aphasia ◽

Lateral Sclerosis

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Progress in Clinical Neurosciences: Frontotemporal Dementia-Pick's Disease

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s0317167100004893 ◽

2006 ◽

Vol 33 (2) ◽

pp. 141-148 ◽

Cited By ~ 15

Author(s):

Andrew Kertesz

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Frontotemporal Dementia ◽

Unsolved Problem ◽

Corticobasal Degeneration ◽

Primary Progressive Aphasia ◽

Pick's Disease ◽

Pick’S Disease ◽

Diagnostic Features ◽

Pick Bodies ◽

Lateral Sclerosis

ABSTRACT:Frontotemporal dementia (clinical Pick's disease) is a relatively common, but underdiagnosed degenerative disease in the presenium. Estimated prevalence ranges from 6-12% of dementias. The behavioural, aphasic and extrapyramidal presentations are labeled FTD-behavioural variant, Primary Progressive Aphasia (PPA) and Corticobasal Degeneration/Progressive Supranuclear Palsy (CBD/PSP). The diagnostic features and course of each are described and their overlap in the evolution of the illness is emphasized. The neuropathology ranges from the most common tau negative ubiquitin positive amyotrophic lateral sclerosis (ALS) type inclusions to the tau positive classical Pick bodies and more or less distinct changes of PSP and CBD. The genetics of the relatively frequent tau mutations and the yet unsolved problem of tau negative families are discussed. The tau negative cases tend to be associated with the behavioural presentation and semantic dementia and the tau positive ones with PPA and the CBD/PSP syndrome. However the overlap is too great to split the disease. A glossary to navigate the proliferating terminology is included.

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