Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients

Dell’Aquila, Claudia; Zoccolella, Stefano; Cardinali, Valentina; Mari, Michele; Iliceto, Giovanni; Tartaglione, Bruno; Lamberti, Paolo; Logroscino, Giancarlo

doi:10.1016/j.parkreldis.2013.06.014

Cited by 88 publications

(81 citation statements)

References 25 publications

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“…Eleven studies were North American [3,7,19,31,32,33,34,35,36,37,38], eleven were Western European [8,10,39,40,41,42,43,44,45,46,47] and four were from Southeast and East Asia [5,48,49,50]. One was multi-continental [4].…”

Section: Resultsmentioning

confidence: 99%

“…While most studies we examined did not find a link between survival and age [4,7,8,10,22,42,43,46], sex [4,7,8,22,38,42,43,46] or education [7,8,46], some reported shorter survival with older age at onset [5,19,35,45,47] and one with male sex [45]. One study found longer survival in males [55] and positive association between occupational attainment and survival (and not with education).…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Kansal

Mareddy

Sloane

et al. 2016

Dement Geriatr Cogn Disord

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“…De ziekteprevalentie is ongeveer vergelijkbaar met MSA, 4 maar de ziekte begint later dan bij MSA, namelijk gemiddeld rond 63 tot 66 jaar. 6,37 De prevalentie van slikstoornissen is hoog, afhankelijk van het ziektestadium en van de wijze van meten tot vrijwel 100%. 8 Behalve de parkinsonachtige verschijnselen (bradykinesie en rigiditeit), kan de orofaryngeale musculatuur ook spastisch worden, waardoor de verminderde kauw-en slikcapaciteit niet goed meer te compenseren is en moeizaam slikken en verslikken vaker optreedt, in combinatie met moeizaam ophoesten.…”

Section: Multisysteem Atrofieunclassified

Orofaryngeale slikstoornissen bij neurodegeneratieve aandoeningen

Kalf

Wit²

2014

Tijdschr Gerontol Geriatr

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Neurodegenerative diseases are progressive degenerations of the brain with increasing movement disorders, like gait, speech and swallowing disorders combined with cognitive disorders, like dementia. Neurodegenerative diseases have in common that aspiration pneumonia is the most frequent cause of death in the end-stage of the disease. This article provides an overview of the prevalence, characteristics and treatments of oropharyngeal dysphagia resulting from multiple system atrophy (MSA), progressive supranuclear palsy (PSP), Huntington's disease (HD), multiple sclerosis (MS) and dementia. The prevalence of dysphagia ranges from 32% in MS, 57% in patients with dementia to 73% in patients with MSA and almost 100% in patients with PSP or HD. Treatment is aimed at swallowing with less effort, swallowing more safely and/or optimal nutritional intake. When cognitive decline and behavioural changes increase, passive compensations like food adaptations and comfortable mealtime conditions become the principal interventions.

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“…APSs are characterized by a rapid progression to disability and death. According to clinical and clinicopathologic studies, MSA, PSP, and CBD share a comparably short survival time of about 7-8 y from symptom onset or less than 3-4 y from clinical diagnosis (1)(2)(3)(4)(5)(6)(7)(8). Survival in PD is distinctly better: although some population-based studies did not find higher mortality (9), others convincingly demonstrated an increased age-adjusted mortality in PD (10,11).…”

mentioning

confidence: 99%

“…Survival in PD is distinctly better: although some population-based studies did not find higher mortality (9), others convincingly demonstrated an increased age-adjusted mortality in PD (10,11). As in APS (1,2,5,8), higher age at onset is also associated with a higher PD mortality (9,10), but median survival time was still 10.3 y in a recent population-based cohort with a high average age of 70 y at diagnosis (9). The cumulative incidence of PD with dementia (PDD) increases with age and disease duration up to 80%-90% (12), being associated with a strong increase in mortality (9)(10)(11).…”

mentioning

confidence: 99%

¹⁸F-FDG PET Is an Early Predictor of Overall Survival in Suspected Atypical Parkinsonism

et al. 2015

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Early prognostic stratification is desirable in patients with suspected atypical parkinsonian syndromes (APSs) for optimal treatment and counseling. We investigated the prognostic value of imaging diseasespecific metabolism patterns with 18 F-FDG PET compared with that of clinical diagnosis. Methods: Seventy-eight patients with suspected APS at study inclusion underwent a follow-up of up to 5.9 y after prospective 18 F-FDG PET imaging. Survival data were analyzed by Kaplan-Meier and Cox regression analyses according to diagnostic classifications provided by 18 F-FDG PET at baseline and clinical diagnoses after a median follow-up of 1 y after PET. Results: Forty-four of 78 patients were alive 4.7 ± 0.6 y after PET. Patients diagnosed with an APS by PET or 1-y clinical follow-up showed a significantly shorter median survival time (4.1 y, age-adjusted hazard ratios [HRs] 5 3.8 for both classifiers) than those diagnosed with Lewy-body diseases (LBDs; majority Parkinson disease [PD]; median survival time not reached). Subgroup classifications of progressive supranuclear palsy/ corticobasal degeneration (PSP/CBD) or multiple-system atrophy (MSA) by PET and clinical follow-up were associated with significantly shorter survival than PD. Age-adjusted mortality was significantly increased for PSP/CBD (HR 5 5.2) and MSA (HR 5 5.6) classified by PET, but for PSP/CBD only when diagnosed by clinical follow-up (HR 5 4.5). Patients with a PET pattern suggestive of PD with dementia/dementia with Lewy bodies (PDD/DLB) exhibited a trend toward shorter survival than those with PD (P 5 0.07), whereas patients classified as PDD/DLB by clinical follow-up did not (P 5 0.65). Conclusion: 18 F-FDG PET is an early predictor of survival in patients with clinically suspected APS. Detection of cortical or subcortical hypometabolism by 18 F-FDG PET is an unfavorable predictor. Risk stratification by 18 F-FDG PET appears to be at least as predictive as the 1-y follow-up clinical diagnosis. This finding strongly supports the early inclusion of PET imaging in patient care.

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Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients

Cited by 88 publications

References 25 publications

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Orofaryngeale slikstoornissen bij neurodegeneratieve aandoeningen

¹⁸F-FDG PET Is an Early Predictor of Overall Survival in Suspected Atypical Parkinsonism

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Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients

Cited by 88 publications

References 25 publications

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

Orofaryngeale slikstoornissen bij neurodegeneratieve aandoeningen

18F-FDG PET Is an Early Predictor of Overall Survival in Suspected Atypical Parkinsonism

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¹⁸F-FDG PET Is an Early Predictor of Overall Survival in Suspected Atypical Parkinsonism