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ObjectiveThe 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.MethodsThe 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.ResultsIn 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.ConclusionFTLD 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.

Objective: The aim of this study is to validate the Alzheimer9s disease (AD) biomarker model by comparing clinical features and conversion to AD and other dementias among groups of patients with mild cognitive impairment (MCI) with different abnormal biomarker profiles. Background A pathophysiologic model of AD has been recently proposed in which beta amyloid accumulation occurs earlier (indexed by abnormal CSF Abeta42), followed by tau-mediated neuronal injury and dysfunction (abnormal CSF tau or FDG-PET) and lastly atrophic changes (abnormal hippocampal volume, HV). Design/Methods: The patients of this study were 58 with MCI coming to observation to the Translational Outpatient Memory Clinic of the Italian National Centre for AD in Brescia, Italy, in whom all the above AD biomarkers were collected. Patients were divided into 3 groups of no abnormal biomarker, AD biomarker pattern (including 3 subgroups of early=only abnormal Abeta42, intermediate=abnormal Abeta42 and FDG-PET or tau, and late=abnormal Abeta42, FDG-PET or tau, and HV), and any other biomarker combination. Results: MCI patients with AD biomarker pattern had lower behavioural disturbances by the NeuroPsychiatry Inventory than patients with ant other biomarker combination (p Conclusions: The results of this study provide evidence in favour of the dynamic biomarker model and support the use of biomarkers for the diagnosis of MCI due to AD according to the new recently published research criteria. Disclosure: Dr. Galluzzi has nothing to disclose. Dr. Geroldi has nothing to disclose. Dr. Amicucci has nothing to disclose. Dr. Bocchio-Chiavetto has nothing to disclose. Dr. Bonetti has nothing to disclose. Dr. Cotelli has nothing to disclose. Dr. Gennarelli has nothing to disclose. Dr. Ghidoni has nothing to disclose. Dr. Paghera has nothing to disclose. Dr. Zanetti has nothing to disclose. Dr. Frisoni has received personal compensation for activities Eli Lilly & Company, Bristol-Myers Squibb Company, Bayer Healthcare, Lundbeck Research USA, Inc., Elan Corporation, AstraZeneca Pharmaceuticals, Pfizer Inc, Baxter, Taurx, and Wyeth Pharmaceuticals. Dr. Frisoni has received personal compensation in an editorial capacity for Lancet Neurology, Aging Clinical & Experimental Research, Neurobiology of Aging, Alzheimer9s Diseases and Associated Disorders, and Neurogenerative Diseases. Dr. Frisoni has received research support from Wyeth Corporation, Eli Lilly & Company, Lundbeck Research USA, Inc.