Predictors of mortality in frontotemporal dementia: a retrospective study of the prognostic influence of pre‐diagnostic features

Abstract

Two groups of predictors with different influence on survival were identified in FTD. Most behavioural/psychiatric features were associated with longer survival. These features may indicate a slower disease progress and a better preserved cerebral function. By contrast, semi-mutism/mutism, neurological deficits and dysphagia were associated with shorter survival, indicating an aggressive, degenerative process.

Distinct cognitive profiles and rates of decline on the Mattis Dementia Rating Scale in autopsy-confirmed frontotemporal dementia and Alzheimer's disease

“…It is estimated that approximately 3-20% of all cases of dementia may be FTD (Barker et al, 2002;Gislason et al, 2003;Knopman et al, 1990;Neary, 1999;Ratnavalli et al, 2002;Rosso et al, 2003), and the disorder is particularly prevalent when the age of onset of dementia is younger than 65 years (Ratnavalli et al, 2002;Rosso et al, 2003). Recent studies suggest that FTD follows a particularly malignant course (Grasbeck et al, 2003;Hodges et al, 2003), but information as to the nature and rate of cognitive decline is still scarce (Binetti et al, 2000;Pasquier et al, 2004;Rascovsky et al, 2005).…”

What Is the Life Expectancy in Frontotemporal Lobar Degeneration?

“…It would appear then that survival in FTLD is comparable in many cases to that in Alzheimer's disease. This work also buttresses earlier observations [3,12] that survival is not associated with the demographic characteristics of FTLD subjects, their age at illness onset, or the severity of the dementia at the time of diagnosis, and shows again that the main causes of death in FTLD are respiratory and cardiovascular disorders, and cachexia.The stage has now become set for analyses of FTLD survival that measure correlations with clinical characteristics, such as mutism, dysphagia, falls, parkinsonism, and co-incident conditions (for example, diabetes mellitus), since there are indications in earlier work that certain clinical features may be pertinent [4,8,13] . By taking us in such directions, future research will eventually specify the parameters for estimations of FTLD life expectancy in individuals, in addition to providing, it is hoped, new treatment opportunities.…”

A Review: Mealtime Difficulties following Frontotemporal Lobar Degeneration

“…The occurrence and severity of dysphagia in the presentation of FTLD is thought to be one of the most accurate predictors of shorter survival, especially when the onset occurs prior to behavioral symptoms [13, 51]. Dysphagia which presents early in disease progression is attributed to a rapidly progressing neuropathology and is commonly a prodrome of FTD-MND [4, 51].…”

“…Individuals with FTD-MND are at an increased risk of displaying feeding behaviors characteristic of bvFTD in combination with a deteriorating swallow function [4, 54]. Oropharyngeal weakness, progressive hyperphagia, and expiratory muscle weakness puts the FTD-MND population at a high risk of aspirating food and drink [51]. Respiratory failure secondary to aspiration pneumonia is a leading cause of death in FTD-MND [22].…”

“…Dysphagia which presents early in disease progression is attributed to a rapidly progressing neuropathology and is commonly a prodrome of FTD-MND [4, 51]. …”