Survival in Incident Cases with Frontotemporal Lobar Degeneration: A Registry-Based Study

Article type: Short Communication

Authors: Borroni, Barbara^{a; b; *} | Urso, Daniele^c | Zecca, Chiara^c | Binetti, Giuliano^d | Fostinelli, Silvia^d | Benussi, Luisa^e | Ghidoni, Roberta^e | Tarantino, Barbara^f | Rivolta, Jasmine^a | Dell’Abate, Maria Teresa^c | Alberici, Antonella^b | Logroscino, Giancarlo^{c; g}

Affiliations: [a] Department of Clinical and Experimental Sciences, Neurology Unit, University of Brescia, Brescia, Italy | [b] Department of Continuity of Care and Frailty, ASST Spedali Civili Brescia, Brescia, Italy | [c] Center for Neurodegenerative Diseases and the Aging Brain, Department of Clinical Research in Neurology, University of Bari “Aldo Moro”, “Pia Fondazione Cardinale G. Panico”, Tricase, Lecce, Italy | [d] MAC-Memory Clinic and Molecular Markers Laboratory, IRCCS Istituto Centro San Giovanni di Dio Fatebenefratelli, Brescia, Italy | [e] Molecular Markers Laboratory, IRCCS Istituto Centro San Giovanni di Dio Fatebenefratelli, Brescia, Italy | [f] Department of Brain and Behavioural Sciences, Medical and Genomic Statistics Unit, University of Pavia, Pavia, Italy | [g] Department of Basic Medical Sciences, Neuroscience and Sense Organs, University of Bari “Aldo Moro”, Bari, Italy

Correspondence: [*] Correspondence to: Barbara Borroni, MD, Clinica Neurologica, Dipartimento Scienze Cliniche e Sperimentali, Università degli Studi di Brescia, P.le Spedali Civili 1, 25123, Brescia, Italy. Tel.: +0039 0303995632; E-mail: [email protected].

Abstract: Population-based registries represent a unique sample to estimate survival. The aim of the present study was to assess survival rates and predictors of outcome in incidental frontotemporal lobar degeneration (FTLD). Incident cases with FTLD, included between January 1, 2017 to December 31, 2017, have been followed for five years. Median survival was 8.16 years from disease onset and 5.38 years from diagnosis. Survival rates did not differ between phenotypes. Shorter disease duration from onset to diagnosis was associated with poorer outcome (p = 0.01). FTLD is a relatively homogeneous disease in terms of survival. Future multinational population-based studies are needed to confirm these findings.

Keywords: Alzheimer’s disease, behavioral variant frontotemporal dementia, frontotemporal dementia, primary progressive aphasia, survival

DOI: 10.3233/JAD-230676

Journal: Journal of Alzheimer's Disease, vol. 96, no. 3, pp. 1019-1024, 2023