Univ. Lille, Inserm, CHU Lille, Lille Neuroscience & Cognition, CNRMAJ, LiCEND, DistAlz, F-59000, Lille, France.
Biostastitic department, CHU Lille, DistALz, Lille, France.
Alzheimers Res Ther. 2021 Jan 8;13(1):19. doi: 10.1186/s13195-020-00753-9.
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.
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).
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.
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.
由于临床表现异质性、与阿尔茨海默病（AD）和精神障碍的鉴别诊断困难，以及临床标准不断演变，额颞叶变性（FTD）的流行病学和自然史仍然难以捉摸。为了更好地描述 FTD 患者，我们依赖于一个具有标准化诊断程序的区域记忆诊所网络的数据库，并选择 AD 患者作为对照。
纳入 2010 年 1 月至 2016 年 12 月期间首次转诊至我们网络且最后临床诊断为退行性或血管性痴呆的患者。比较 FTD 与 AD 以及不同 FTD 综合征，分为语言变异型（lvFTD）、行为变异型（bvFTD）和主要以运动症状为特征的 FTD（mFTD）。使用 Mini Mental State Examination（MMSE）每年下降来估计认知进展。
在 6 年的时间里，被转诊到我们网络的患者中，有 690 例最终被诊断为 FTD，18831 例被诊断为 AD。FTD 综合征患者占所有病因性痴呆的 2.6%。年龄标准化发病率为每 100000 人年 2.90 例，发病率高峰在 75 至 79 岁之间。与 AD 相比，FTD 综合征患者的转诊延迟和诊断延迟时间更长。FTD 综合征患者首次就诊时 MMSE 评分高于 AD，但进展相似。mFTD 患者的生存率最短，而 bvFTD、lvFTD 和 AD 患者的生存率无显著差异。与 AD 患者相比，FTD 患者，尤其是行为变异型患者，接受了更多的抗抑郁药、抗焦虑药和抗精神病药。
FTD 综合征与 AD 在基线特征、进展速度和治疗方面存在差异。尽管在一个有组织的记忆诊所网络中广泛使用新的诊断标准，但 FTD 综合征的诊断时间更长，占痴呆病例的比例较低，表明持续存在漏诊。与最近的出版物一致，发病率的晚高峰警告人们不要将 FTD 视为仅为早发性痴呆。
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