BACKGROUND AND PURPOSE: Behavioural disturbances are the core features of frontotemporal dementia (FTD); however, symptom progression is still not well characterized during the entire course of the disease. The aim of the present study was to investigate behavioural symptoms at baseline and during the disease course in a large cohort of patients with behavioural variant FTD (bvFTD), non-fluent/agrammatic variant primary progressive aphasia (nfvPPA) and semantic variant primary progressive aphasia (PPA). METHODS: We evaluated 403 patients with FTD, 167 of whom had at least 1-year follow-up evaluation (for a total of 764 assessments). Behavioural symptoms were assessed and rated through the Neuropsychiatric Inventory (NPI) and Frontal Behavioural Inventory (FBI). Disease severity was evaluated through the Frontotemporal Lobar Degeneration -Clinical Dementia Rating scale (FTLD-CDR). Linear mixed models were used to model behavioural measures (NPI, FBI and the five FBI-behavioural core criteria scores) as a function of disease severity (FTLD-CDR score) and clinical phenotype. RESULTS: At baseline, patients with bvFTD showed more behavioural disturbances compared with those with nfvPPA (P = 0.004). Negative symptoms (apathy and loss of empathy) showed a trend to an increase throughout the course of the disease in both bvFTD and PPA (P < 0.001 until intermediate stages). Positive symptoms (disinhibition, perseverations and hyperorality) increased until intermediate phases (P < 0.001) followed by a progressive reduction in later phases, whereas they were less common in nfvPPA throughout the disease course. CONCLUSION: We demonstrated that behavioural disturbances differed in FTD and with disease severity. Positive symptoms appeared to improve in the advanced stages, highlighting the importance of taking into account the disease severity as outcome measure in clinical trials.
BACKGROUND AND PURPOSE: Behavioural disturbances are the core features of frontotemporal dementia (FTD); however, symptom progression is still not well characterized during the entire course of the disease. The aim of the present study was to investigate behavioural symptoms at baseline and during the disease course in a large cohort of patients with behavioural variant FTD (bvFTD), non-fluent/agrammatic variant primary progressive aphasia (nfvPPA) and semantic variant primary progressive aphasia (PPA). METHODS: We evaluated 403 patients with FTD, 167 of whom had at least 1-year follow-up evaluation (for a total of 764 assessments). Behavioural symptoms were assessed and rated through the Neuropsychiatric Inventory (NPI) and Frontal Behavioural Inventory (FBI). Disease severity was evaluated through the Frontotemporal Lobar Degeneration -Clinical Dementia Rating scale (FTLD-CDR). Linear mixed models were used to model behavioural measures (NPI, FBI and the five FBI-behavioural core criteria scores) as a function of disease severity (FTLD-CDR score) and clinical phenotype. RESULTS: At baseline, patients with bvFTD showed more behavioural disturbances compared with those with nfvPPA (P = 0.004). Negative symptoms (apathy and loss of empathy) showed a trend to an increase throughout the course of the disease in both bvFTD and PPA (P < 0.001 until intermediate stages). Positive symptoms (disinhibition, perseverations and hyperorality) increased until intermediate phases (P < 0.001) followed by a progressive reduction in later phases, whereas they were less common in nfvPPA throughout the disease course. CONCLUSION: We demonstrated that behavioural disturbances differed in FTD and with disease severity. Positive symptoms appeared to improve in the advanced stages, highlighting the importance of taking into account the disease severity as outcome measure in clinical trials.
Authors: Hannah E Silverman; Jeannie M Ake; Masood Manoochehri; Brian S Appleby; Danielle Brushaber; Katrina L Devick; Bradford C Dickerson; Julie A Fields; Leah K Forsberg; Nupur Ghoshal; Neill R Graff-Radford; Murray Grossman; Hilary W Heuer; John Kornak; Maria I Lapid; Irene Litvan; Ian R Mackenzie; Mario F Mendez; Chiadi U Onyike; Belen Pascual; Maria Carmela Tartaglia; Bradley F Boeve; Adam L Boxer; Howard J Rosen; Stephanie Cosentino; Edward D Huey; Megan S Barker; Jill S Goldman Journal: Alzheimers Dement Date: 2021-12-02 Impact factor: 16.655
Authors: Hannah E Silverman; Yunglin Gazes; Megan S Barker; Masood Manoochehri; Jill S Goldman; Eric M Wassermann; Michael C Tierney; Stephanie Cosentino; Jordan Grafman; Edward D Huey Journal: J Alzheimers Dis Date: 2020 Impact factor: 4.472
Authors: Alberto Benussi; Enrico Premi; Stefano Gazzina; Chiara Brattini; Elisa Bonomi; Antonella Alberici; Lize Jiskoot; John C van Swieten; Raquel Sanchez-Valle; Fermin Moreno; Robert Laforce; Caroline Graff; Matthis Synofzik; Daniela Galimberti; Mario Masellis; Carmela Tartaglia; James B Rowe; Elizabeth Finger; Rik Vandenberghe; Alexandre de Mendonça; Fabrizio Tagliavini; Isabel Santana; Simon Ducharme; Chris R Butler; Alexander Gerhard; Johannes Levin; Adrian Danek; Markus Otto; Giovanni Frisoni; Roberta Ghidoni; Sandro Sorbi; Isabelle Le Ber; Florence Pasquier; Georgia Peakman; Emily Todd; Martina Bocchetta; Jonathan D Rohrer; Barbara Borroni Journal: JAMA Netw Open Date: 2021-01-04
Authors: Alberto Benussi; Nicholas J Ashton; Thomas K Karikari; Antonella Alberici; Claudia Saraceno; Roberta Ghidoni; Luisa Benussi; Henrik Zetterberg; Kaj Blennow; Barbara Borroni Journal: Alzheimers Res Ther Date: 2021-11-15 Impact factor: 6.982