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Abstract
Objective The goal of the present work, based on a collaborative research registry in Italy (the Salento-Brescia Registry), was to assess the incidence of frontotemporal lobar degeneration (FTLD) and to define the frequencies of different FTLD phenotypes in the general population.
Methods The study was conducted from January 1, 2017, to December 31, 2017, in 2 Italian provinces: Lecce (in Puglia) in the south (area 2,799.07 km2, inhabitants 802,082) and Brescia (in Lombardy) in the north (area 4,785.62 km2, inhabitants 1,262,678). During the study period, all new cases of FTLD (incident FTLD) were counted, and all patients' records were reviewed. The incidence was standardized to the Italian general population in 2017.
Results In the 2 provinces, 63 patients with FTLD were diagnosed. The incidence rate for FTLD was 3.05 (95% confidence interval [CI] 2.34–3.90) per 100,000 person-years (py), while the age-sex standardized incidence rate was 3.09 (95% CI 2.95–3.23) per 100,000 py. In the Italian population, the lifetime risk was 1:400. There was a progressive increase in FTLD incidence across age groups, reaching its peak in the 75- to 79-year-old group, with an incidence rate of 15.97 (95% CI 8.94–26.33) per 100,000 py. The behavioral variant of frontotemporal dementia was the most common phenotype (37%). No difference in crude incidence rate between the 2 provinces was observed.
Conclusion FTLD is a more common form of dementia than previously recognized, with a risk spanning in a wide age range and with maximum incidence in the mid-70s. Improved knowledge of FTLD epidemiology will help to provide appropriate public health service policies.
Glossary
- AD=
- Alzheimer disease;
- bvFTD=
- behavioral variant of frontotemporal dementia;
- CBS=
- corticobasal syndrome;
- CDCD=
- Centres for Cognitive Disorders and Dementias;
- CI=
- confidence interval;
- DSM-III-R=
- Diagnostic and Statistical Manual of Mental Disorders, 3rd edition, revised;
- FTLD=
- frontotemporal lobar degeneration;
- GP=
- general practitioner;
- nfvPPA=
- nonfluent variant of primary progressive aphasia;
- PSP=
- progressive supranuclear palsy;
- py=
- person-years;
- SLAP=
- Sclerosi Laterale Amiotrofica–Puglia;
- SLAPDem=
- Sclerosi Laterale Amiotrofica–Puglia including dementia;
- svPPA=
- semantic variant of primary progressive aphasia
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received August 2, 2018.
- Accepted in final form January 19, 2019.
- © 2019 American Academy of Neurology
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