Predicting loss of independence and mortality in frontotemporal lobar degeneration syndromes

Murley, Alexander G; Rouse, Matthew A; Coyle‐Gilchrist, Ian; Jones, P. Simon; Li, Win; Wiggins, Julie; Lansdall, Claire; Rodríguez, Patricia Vázquez; Wilcox, A. W.; Patterson, Karalyn; Rowe, James B.

doi:10.1136/jnnp-2020-324903

Cited by 23 publications

(26 citation statements)

References 42 publications

(36 reference statements)

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“…A study published in 2021 reported that motor symptoms were associated with reduced survival in patients with frontotemporal degeneration, including parkinsonism, dystonia, and apraxia. 31 Besides, rapid eating and dysphagia are common in patients with frontotemporal degeneration, and these symptoms might increase the risk of choking, aspiration pneumonia, and mortality. 32 Importantly, people with frontotemporal degeneration might have a younger age at onset and diagnosis than people with Alzheimer's disease, and thereby the mortality rate after diagnosis might be attenuated during the followup period.…”

Section: Discussionmentioning

confidence: 99%

Mortality rates in Alzheimer's disease and non-Alzheimer's dementias: a systematic review and meta-analysis

Liang

Yang

et al. 2021

The Lancet Healthy Longevity

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Background People with dementia die prematurely. Identifying differences in mortality rates between different types of dementia might aid in the development of preventive interventions for the most vulnerable populations. The aim of this study was to compare the difference in mortality rates between individuals without dementia and individuals with various types of dementia.Methods For this systematic review and meta-analysis, we did a systematic search of MEDLINE, PubMed, Embase, and Cochrane Library from inception to July 11, 2020, for cross-sectional or cohort studies that assessed mortality and survival-related outcomes among people with different types of dementia compared with people without dementia. Single-arm studies without comparison groups and autopsy studies or family studies that used a selected sample were excluded. The Newcastle-Ottawa Scale was used by two authors (D-JL and C-SC) independently to measure the methodological quality of included studies, and two authors (F-CY and P-TT) independently extracted data. We assessed differences in all-cause mortality rate and survival time from dementia diagnosis between individuals without dementia, individuals with Alzheimer's disease, and individuals with non-Alzheimer's disease dementias. The secondary outcomes were age at death and survival time from disease onset. Random-effects meta-analyses were done. Effect sizes included hazard ratios (HRs) and mean differences (MDs) with 95% CIs. Potential moderators, including age-associated moderators, were identified through meta-regression and subgroup analyses. This study is registered with PROSPERO, CRD42020198786.Findings Our database search identified 11 973 records, and we included 78 eligible studies in our analyses, encompassing 63 125 individuals with dementia and 152 353 controls. Individuals with any type of dementia had a higher mortality rate than individuals without dementia (HR 5•90, 95% CI 3•53 to 9•86), and the HR for all-cause mortality was highest for Lewy body dementia (17•88, 5•87 to 54•46). After diagnosis, the mean survival time for people with Alzheimer's disease was 5•8 years (SD 2•0). Compared with people with Alzheimer's disease, a diagnosis of any non-Alzheimer's disease dementia was associated with a higher risk of all-cause mortality (HR 1•33, 1•21 to 1•46), a shorter survival time from diagnosis (MD -1•12 years, 95% CI -1•52 to -0•72), and a younger age at death (-1•76 years, -2•66 to -0•85). Survival time from disease onset was also shorter in people with non-Alzheimer's dementia, across types, compared with people with Alzheimer's disease, but the subgroup analysis revealed that this difference was only significant for vascular dementia (MD -1•27 years, -1•90 to -0•65) and dementia with Lewy bodies (MD -1•06 years, -1•68 to -0•44). The interactions between age and several survival-related outcomes were significant. 39 (50%) of the 78 included studies were rated as good quality, and large heterogeneity (I²>75%) was observed for most of the study outcomes. Interpretation Alzhei...

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Section: Discussionmentioning

confidence: 99%

Mortality rates in Alzheimer's disease and non-Alzheimer's dementias: a systematic review and meta-analysis

Liang

Yang

et al. 2021

The Lancet Healthy Longevity

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“…Patients with PSP often display prominent neuropsychiatric problems, including apathy and impulsivity [2][3][4] . In PSP and related disorders, apathy and impulsivity reduce survival 5 and predict loss of functional independence 6 .…”

Section: Introductionmentioning

confidence: 99%

Co-occurrence of apathy and impulsivity in progressive supranuclear palsy

Kok

Murley

Rittman

et al. 2021

Preprint

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BackgroundApathy and impulsivity are common problems in progressive supranuclear palsy (PSP) and can worsen its prognosis. They can co-exist in the same patients although their concomitant prevalence remains unclear. Their relationship to emotional lability is also unknown.ObjectivesTo estimate the co-occurrence of apathy and impulsivity and their relationship to emotional lability in PSP. To characterize the demographic, clinical, and cognitive features of PSP patients with apathy and impulsivity.MethodsIn a retrospective study of a long-term clinical cohort, we assessed the prevalence of apathy, impulsivity, and emotional lability from clinical interviews, medical records, and contemporary carer questionnaires. N=154 patients with a diagnosis of probable or possible PSP (according to the 2017 Movement Disorder Society criteria) were identified. N=64 of these patients had neuropathological confirmation of PSP. PSP patients with both apathy and impulsivity were compared in terms of demographic, clinical, and cognitive characteristics to PSP patients with either one or neither of these neuropsychiatric features.ResultsApathy and impulsivity co-existed in two-thirds of people with PSP. A fifth displayed emotional lability in addition to apathy and impulsivity. Apathy and impulsivity were more commonly co-expressed than by chance. There was no single demographic, clinical or cognitive feature that distinguished between PSP patients with versus patients without apathy and impulsivity.ConclusionsThe co-existence of apathy and impulsivity in PSP suggests that these neuropsychiatric features may share similar risk factors and etio-pathogenetic mechanisms. Apathy and impulsivity should be jointly assessed when planning symptomatic treatments for behavioural problems in PSP.

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“…One of the consequences is an impairment of response inhibition, contributing to disinhibited behaviour and impulsivity. These are associated with poor clinical outcomes [4][5][6] .…”

Section: Introductionmentioning

confidence: 99%

“…One of the consequences is an impairment of response inhibition, contributing to disinhibited behaviour and impulsivity. These are associated with poor clinical outcomes 4-6 . Impulsivity is a multi-faceted behavioural construct, including abnormal sensitivity to reward, intolerance to delayed reward and a failure to inhibit inappropriate responses 7,8 .…”

Section: Introductionmentioning

confidence: 99%

Reduced locus coeruleus integrity linked to response inhibition deficits in parkinsonian disorders

Hezemans

O’Callaghan

et al. 2021

Preprint

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Parkinson's disease and progressive supranuclear palsy (PSP) both impair response inhibition, exacerbating impulsivity. Inhibitory control deficits vary across individuals, and have been linked with worse prognosis and lack of improvement on dopaminergic therapy. Motor and cognitive control are associated with noradrenergic innervation of the cortex, arising from the locus coeruleus noradrenergic system. Here we test the hypothesis that loss of structural integrity of the locus coeruleus explains response inhibition deficits in progressive supranuclear palsy and Parkinson's disease. This cross-sectional observational study recruited 24 people with idiopathic Parkinson's disease, 14 with PSP-Richardson's syndrome, and 24 age- and sex-matched controls. All participants undertook a stop-signal task and ultrahigh field 7T-magnetic transfer weighted imaging of the locus coeruleus. Hierarchical Bayesian estimation of the parameters of 'race models' of go- versus stop-decisions was used to quantify the cognitive processes of response inhibition. We tested the multivariate relationship between locus coeruleus integrity and model parameters using partial least squares. Both disorders impaired response inhibition at the group level. Progressive supranuclear palsy caused a distinct pattern of abnormalities in inhibitory control, relative to Parkinson's disease and healthy controls, with a paradoxically reduced threshold for go responses, but longer non-decision times, and more lapses of attention. The variation in response inhibition correlated with variation in the integrity of the locus coeruleus, across participants in both clinical groups. Structural imaging of the locus coeruleus, coupled with behavioural modelling in parkinsonian disorders, confirms that locus coeruleus integrity is associated with response inhibition and its degeneration contributes to neurobehavioural changes. The noradrenergic system is therefore a promising target to treat impulsivity in these conditions. The optimisation of noradrenergic treatment is likely to benefit from stratification according to locus coeruleus integrity.

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Predicting loss of independence and mortality in frontotemporal lobar degeneration syndromes

Cited by 23 publications

References 42 publications

Mortality rates in Alzheimer's disease and non-Alzheimer's dementias: a systematic review and meta-analysis

Mortality rates in Alzheimer's disease and non-Alzheimer's dementias: a systematic review and meta-analysis

Co-occurrence of apathy and impulsivity in progressive supranuclear palsy

Reduced locus coeruleus integrity linked to response inhibition deficits in parkinsonian disorders

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