Abstract
ObjectivesFrontotemporal dementia (FTD) is considered to be a mainly early-onset neurodegenerative disorder with a strong hereditary component. The aim of the study was to investigate age-related incidence and family history in FTD compared to other dementia disorders, especially Alzheimer's disease (AD).MethodsThe Swedish Dementia Registry (SveDem) registers all new cases of dementia diagnosed by the participating centres, including data on demographics, diagnosis, and investigations used. Data for the period 2008–2011 were extracted and compared with age-related population data on a regional and national level.ResultsThere were 20 305 patients registered in SveDem during 2008–2011, whereof 352 received a diagnosis of FTD. Mean age at diagnosis for FTD was 69.6 years and almost 70% of FTD cases were 65 years or older at the time of diagnosis. Both FTD and AD showed an increased incidence with age, which reached a maximum in the age group 80–84 years at 6.04 and 202 cases per 100 000 person-years, respectively. The proportion of cases with a positive family history was significantly lower in FTD than in AD.ConclusionsContrary to general opinion within the field, data from SveDem show that the incidence of FTD increases with age, and that the majority of cases are diagnosed after the age of 65 years. In addition, data from SveDem might suggest that the importance of hereditary factors in general is similar in FTD and AD. The recognition of these findings has important consequences for the diagnosis, treatment and care of patients with FTD.
Highlights
The frontotemporal dementias (FTD) are a group of neurodegenerative disorders affecting primarily the frontal and temporal lobes of the brain, leading to various combinations of behavioural, cognitive and motor symptoms [1,2]
The disorder was first described by Pick more than 100 years ago, formal clinical and neuropathological criteria were not developed until the mid-90s [4,5]
20 305 cases of dementia were registered in SveDem during the period 2008–2011
Summary
The frontotemporal dementias (FTD) are a group of neurodegenerative disorders affecting primarily the frontal and temporal lobes of the brain, leading to various combinations of behavioural, cognitive and motor symptoms [1,2]. Studies of the incidence in FTD are much fewer, but have found similar rates in annual incidence for early-onset FTD (,65 years) of 2.7–4.1 cases per 100 000 person-years in the 45–64 year age group [9,10,12,13,14]. The existence of late-onset cases of FTD ($65 years) has been recognized, with a reported range for age at onset of 21–84 years [1,2,6,9], the view of FTD as an early-onset dementia has largely prevailed [1,9,10,15]. More recent community-based studies have shown much higher incidence in late-onset FTD at 16.7 per 100 000 person-years, compared to 1.3 in early-onset FTD [14,16]. It is possible that FTD might be underdiagnosed in the elderly population [2,14,16]
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