Age-Related Incidence and Family History in Frontotemporal Dementia: Data from the Swedish Dementia Registry

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.

Imaging and fluid biomarkers in frontotemporal dementia

“…Interestingly, it is increasingly emphasized that FTD, classically considered as an early-onset dementia, frequently manifests after the age of 65 years and may include clinical features suggestive of AD. 156,157 This stresses the need of diagnostic biomarkers specific for FTD, as the co-occurrence of Alzheimer pathology increases with age. The value of FTD biomarkers in different age groups with comorbidities remains to be elucidated.…”

Incidence of frontotemporal disorders in Olmsted County: A population‐based study

“…We suspect that FTDs might be underreported, and their impact on the general population might be higher than previously thought. FTDs are usually considered a cause of early‐onset dementia, although studies have proven that the incidence of FTDs increases with age . Experienced clinicians and updated diagnostic tools are required to accurately diagnose these disorders; FTDs, in fact, often overlap with other neurodegenerative disorders such as AD or movement disorders; the presence of a frontal variant of AD makes it even more challenging to appropriately diagnose FTDs, especially in light of the aging of the population.…”

“…FTDs are usually considered a cause of early-onset dementia, although studies have proven that the incidence of FTDs increases with age. 24 Experienced clinicians and updated diagnostic tools are required to accurately diagnose these disorders; FTDs, in fact, often overlap with other neurodegenerative disorders such as AD 25 or movement disorders; [4][5][6] the presence of a frontal variant of AD makes it even more challenging to appropriately diagnose FTDs, 26 especially in light of the aging of the population. This may impact not only the results of epidemiological studies, which may underreport the incidence and prevalence of FTDs but, most importantly, the management and the outcomes of the patients.…”

Patient’s occupation, electric & head trauma in a cohort of 88 multiple system atrophy patients compared with the general population: a hypothesis stimulating pilot study

“…The key limit to the study is that we know nothing of the cohort's family/genetic background, as many authors point towards an epigenetic under-lying pathology. [45][46][47][48][49] The other limit in the study is the problem of missing data, also the inability to more accurately identify the severity of head and electrical shock events although we have more detail on occupations from the POL regional categories. Also it is acknowledged that the ratios of head injuries and electric shocks in the general population are only estimates, but as 73% of the cohort reported a head injury and 39% mentioned an electric shock, it is fair to assume the level of these incidents in the general population are above any chance artefact.…”

“…So far we have only commented upon possible interactive environmental factors yet self-evidently many authors point towards genetic and epigenetic factors under-lying the pathology of many neurological diseases. [45][46][47][48][49] However, this project focuses specifically upon on MSA patients' occupational backgrounds and any reported life style traumatic events, following the many patients who had previously volunteered such information. Exploring the literature on such events, a link between neurological disease and electric shock was described by Dureux in 1958 43 but the evidence on electric shock and neurological disorders is inconsistent, some report either no association or the linkage is very weak 34,47 whilst others have found a significant connection.…”