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Neurology. 2019 May 14;92(20):e2355-e2363. doi: 10.1212/WNL.0000000000007498. Epub 2019 Apr 12.

Incidence of frontotemporal lobar degeneration in Italy: The Salento-Brescia Registry study.

Author information

1
From the Center for Neurodegenerative Diseases and the Aging Brain (G.L., M.P., C.Z., R.C., R. Tortelli, P.B., R.B.), Department of Clinical Research in Neurology, and Department of Basic Medical Sciences, Neuroscience and Sense Organs (G.L., M.P., P.B.), University of Bari "Aldo Moro"; "Pia Fondazione Cardinale G. Panico" (G.L., M.P., C.Z., R.C., R. Tortelli, P.B., R.B.), Tricase, Lecce; IRCCS Centro San Giovanni di Dio Fatebenefratelli (G.B., S.F., L.B., R.G., S.F.C.); Department of Clinical and Experimental Sciences (R. Turrone, A.P., A.A., B.B.), Neurology Unit, University of Brescia; Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA) (P.B.), University of Firenze; and Department of Neurology (E.B.), Ospedali Riuniti, Foggia, Italy. giancarlo.logroscino@uniba.it.
2
From the Center for Neurodegenerative Diseases and the Aging Brain (G.L., M.P., C.Z., R.C., R. Tortelli, P.B., R.B.), Department of Clinical Research in Neurology, and Department of Basic Medical Sciences, Neuroscience and Sense Organs (G.L., M.P., P.B.), University of Bari "Aldo Moro"; "Pia Fondazione Cardinale G. Panico" (G.L., M.P., C.Z., R.C., R. Tortelli, P.B., R.B.), Tricase, Lecce; IRCCS Centro San Giovanni di Dio Fatebenefratelli (G.B., S.F., L.B., R.G., S.F.C.); Department of Clinical and Experimental Sciences (R. Turrone, A.P., A.A., B.B.), Neurology Unit, University of Brescia; Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA) (P.B.), University of Firenze; and Department of Neurology (E.B.), Ospedali Riuniti, Foggia, Italy.

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.

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