Abstract Details

Title Incidence of syndromes associated with Frontotemporal Lobar Degeneration

Topic General Neurology

Presentation(s) S19 - Neuroepidemiology (4:06 PM-4:18 PM)

Poster/Presentation
Number 004

Objective To assess FTLD epidemiology across Europe. The FRONTotemporal dementia Incidence European Research Study (FRONTIERS) was established for this purpose.

Background Diagnostic incidence data for syndromes associated with Frontotemporal Lobar Degeneration (FTLD) in multinational studies is urgent in light of upcoming therapeutic approaches.

Design/Methods Retrospective population-based study conducted from June 1^st, 2018 to May 31^st, 2019 in thirteen tertiary FTLD research clinics from the UK, Netherlands, Finland, Sweden, Spain, Bulgaria, Serbia, Germany, and Italy. All new FTLD-associated cases (hereafter: incident FTLD) during the study period were counted, with a combined catchment population of over 11,000,000 person-years. Random intercept Poisson models were used to obtain estimates of the European FTLD incidence rate accounting for geographic heterogeneity.

Results

Based on 267 identified cases (mean age±standard deviation=66.7±9.02; female=41.6%), the annual incidence rate for FTLD in Europe was 2.36 per 100,000 person-years (95% Confidence Intervals [CI]=1.59-3.51). There was a progressive increase in FTLD incidence across age, reaching its peak at the age of 71. Overall, the incidence was higher among men (2.84 per 100,000 person-years; 95% CI=1.88-4.27) than among women (1.91 per 100,000 person-years; 95% CI=1.26-2.91). Behavioural variant frontotemporal dementia was the most common phenotype (40.07%), followed by primary progressive aphasia (28.46%) and extrapyramidal phenotypes (25.84%). Frontotemporal dementia with amyotrophic lateral sclerosis was the rarest phenotype (5.62%). Almost 35% of FTLD patients presented a family history of dementia. The estimated number of new FTLD cases in Europe is approximately 12,000 people per year.

Conclusions

FTLD-associated syndromes are more common than previously recognized, and diagnosis should be considered at any age. Improved knowledge of FTLD epidemiology may contribute to appropriate health and social care planning, and in the design of future clinical trials.

Authors/Disclosures
Giancarlo Logroscino, MD, PhD, FAAN (University of Bari) PRESENTER	Dr. Logroscino has nothing to disclose.
	No disclosure on file
	No disclosure on file
Orla Hardiman, MD, FRCPI, FAAN (Trinity Biomedical Sciences Institute)	Dr. Hardiman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Wave Pharmaceuticals. Dr. Hardiman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cytokinetics . Dr. Hardiman has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Hardiman has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Taylor and Francis. The institution of Dr. Hardiman has received research support from Science Foundation Ireland. The institution of Dr. Hardiman has received research support from HRB.
Albert C. Ludolph, MD	Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Ludolph has received personal compensation in the range of $0-$499 for serving as a Consultant for FundaMental Pharma . Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lilly. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Zambon . Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche Pharma. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alector. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amylyx. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biologix FZCo.. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Teva. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Ludolph has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Zambon.
	No disclosure on file
	No disclosure on file
Anne Remes, MD, PhD	No disclosure on file
James Rowe, BA BM BCh PhD	James Rowe, BA BM BCh PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. James Rowe, BA BM BCh PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for SV Health. James Rowe, BA BM BCh PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astex. James Rowe, BA BM BCh PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Asceneuron. James Rowe, BA BM BCh PhD has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for GE Healthcare. The institution of James Rowe, BA BM BCh PhD has received research support from Janssen. The institution of James Rowe, BA BM BCh PhD has received research support from Lilly. The institution of James Rowe, BA BM BCh PhD has received research support from AZ Medimmune. James Rowe, BA BM BCh PhD has received publishing royalties from a publication relating to health care.
Harro Seelaar, MD, PhD (Erasmus University Medical Center)	Dr. Seelaar has nothing to disclose.
	No disclosure on file
Elka Stefanova	No disclosure on file
Latchezar D. Traykov, MD (UMBLA ALEKSANDROVSKA)	No disclosure on file
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	No disclosure on file
Niall Pender, PhD	No disclosure on file
	No disclosure on file
	No disclosure on file
Alazne Gabilondo Lopez, MD (Neurologia - Hospital Comarcal del Bidasoa)	Alazne Gabilondo Lopez, MD has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
John Van Swieten, MD	Dr. Van Swieten has nothing to disclose.
Paivi Hartikainen, MD (Kuopio University Hospital)	Paivi Hartikainen, MD has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Paivi Hartikainen, MD has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squibb. Paivi Hartikainen, MD has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Paivi Hartikainen, MD has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Paivi Hartikainen, MD has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Antonella Alberici, MD	No disclosure on file
	No disclosure on file
Chiara C. Zecca, MD (Ente ospedaliero cantonale)	The institution of Dr. Zecca has received research support from Ente Ospedaliero Cantonale.
Barbara Borroni, MD	Dr. Borroni has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alector. Dr. Borroni has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wiley.

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