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. 2021 Jan 8;13(1):19.
doi: 10.1186/s13195-020-00753-9.

Characteristics and progression of patients with frontotemporal dementia in a regional memory clinic network

Mélanie Leroy  1 , Maxime Bertoux  1 , Emilie Skrobala  2 , Elisa Mode  3 , Catherine Adnet-Bonte  1 , Isabelle Le Ber  4   5 , Stéphanie Bombois  1 , Pascaline Cassagnaud  1 , Yaohua Chen  1 , Vincent Deramecourt  1 , Florence Lebert  1 , Marie Anne Mackowiak  1 , Adeline Rollin Sillaire  1 , Marielle Wathelet  6 , Florence Pasquier  1 , Thibaud Lebouvier  7 ; Méotis network
Collaborators, Affiliations

Characteristics and progression of patients with frontotemporal dementia in a regional memory clinic network

Mélanie Leroy et al. Alzheimers Res Ther. .

Abstract

Background: Due to heterogeneous clinical presentation, difficult differential diagnosis with Alzheimer's disease (AD) and psychiatric disorders, and evolving clinical criteria, the epidemiology and natural history of frontotemporal lobar degeneration (FTD) remain elusive. In order to better characterize FTD patients, we relied on the database of a regional memory clinic network with standardized diagnostic procedures and chose AD patients as a comparator.

Methods: Patients that were first referred to our network between January 2010 and December 2016 and whose last clinical diagnosis was degenerative or vascular dementia were included. Comparisons were conducted between FTD and AD as well as between the different FTD syndromes, divided into language variants (lvFTD), behavioral variant (bvFTD), and FTD with primarily motor symptoms (mFTD). Cognitive progression was estimated with the yearly decline in Mini Mental State Examination (MMSE).

Results: Among the patients that were referred to our network in the 6-year time span, 690 were ultimately diagnosed with FTD and 18,831 with AD. Patients with FTD syndromes represented 2.6% of all-cause dementias. The age-standardized incidence was 2.90 per 100,000 person-year and incidence peaked between 75 and 79 years. Compared to AD, patients with FTD syndromes had a longer referral delay and delay to diagnosis. Patients with FTD syndromes had a higher MMSE score than AD at first referral while their progression was similar. mFTD patients had the shortest survival while survival in bvFTD, lvFTD, and AD did not significantly differ. FTD patients, especially those with the behavioral variant, received more antidepressants, anxiolytics, and antipsychotics than AD patients.

Conclusions: FTD syndromes differ with AD in characteristics at baseline, progression rate, and treatment. Despite a broad use of the new diagnostic criteria in an organized memory clinic network, FTD syndromes are longer to diagnose and account for a low proportion of dementia cases, suggesting persistent underdiagnosis. Congruent with recent publications, the late peak of incidence warns against considering FTD as being exclusively a young-onset dementia.

Keywords: Dementia; Epidemiology; Frontotemporal dementia; Progression.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Characteristics and progression of patients with FTD syndromes in the Méotis network (incident cases from 2010 to 2016). a Etiologies of dementia in the incident cases. b Mixed linear model of the evolution of the MMSE over time in patients with FTD syndromes and AD. c Survival in patients with FTD syndromes and AD. AD, Alzheimer’s disease; FTD, frontotemporal dementia; bvFTD, behavioral variant of the frontotemporal dementia; lvFTD, speech variant of the frontotemporal dementia; mFTD, motor variant of the frontotemporal dementia; Other, other type of dementia due to a neurodegenerative or vascular disease
Fig. 2
Fig. 2
Drug treatments used in FTD syndromes compared to AD. AD, Alzheimer’s disease; AChEI, anticholinesterase inhibitor; bvFTD, behavioral variant of the frontotemporal dementia; lvFTD, speech variant of the frontotemporal dementia; mFTD, motor variant of the frontotemporal dementia
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