Frontotemporal Dementia in Elderly Individuals
Frontotemporal Dementia in Elderly Individuals
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DOI：
10.1001/archneurol.2011.3323
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发表时间：
2012-08-01
影响因子：
--
通讯作者：
Perry, Robert
中科院分区：
文献类型：
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作者：
Baborie, Atik;Griffiths, Tim D.;Perry, Robert
目的：为了确定额颞叶变性（FTLD）是否确实存在于老年人中，是否具有与早老发作的FTLD不同的临床和神经病理学特征。设计：回顾性配对队列研究。设置：英格兰东北部地区神经科学中心。患者：我们比较了11例老年人FTLD和19例早老发作FTLD的临床病理学特征。结果：回顾性病例记录分析显示，大多数老年FTLD患者的行为特征与眶额和基底额受累一致，与早发型FTLD相似，尽管91%（10/11）的老年FTLD患者存在症状性记忆丧失，而早发型FTLD患者仅为36%（7/19）。神经病理学上，FTLD老年患者组包括7例FTLD-TDP-43、1例泛素阳性FTLD、2例FTLD-tau/Pick病和1例FTLD-tau/神经元缠结为主的TDP-43痴呆，其组成与早老发作FTLD相似。然而，海马硬化在老年FTLD患者中比早老发作FTLD患者更常见（82%对37%），并且在老年FTLD患者中更严重（P <0.05）。相比之下，严重的额叶和颞叶萎缩在老年FTLD患者中（额叶：45%;颞叶：27%）比早老发作FTLD患者（额叶：63%;颞叶：78%）更不常见。FTLD老年患者占3.2%的所有老年痴呆症患者尸检在纽卡斯尔总Hospital.Conclusions：额颞叶变性老年患者作为一个单独的实体存在从早老发作FTLD。其主要特征包括：（1）临床上频繁的记忆丧失和行为改变，主要是语言和语义功能障碍;（2）神经病理学上显著的海马硬化，但不太明显的皮质叶萎缩。临床上，老年患者的FTLD未被充分认识，应在表现为“非典型阿尔茨海默病”表型的老年受试者中考虑。
Objective: To determine whether cases of frontotemporal lobar degeneration (FTLD) do exist in elderly individuals and have clinical and neuropathological features distinct from those with presenile onset.Design: Retrospective matched cohort study.Setting: Regional Neuroscience Centre, North East England.Patients: We compared clinicopathological features of 11 cases of FTLD in elderly individuals with 19 cases of presenile-onset FTLD.Results: Retrospective case note analysis showed that most elderly patients with FTLD had behavioral features consistent with orbitofrontal and basofrontal involvement, similar to presenile-onset FTLD, though symptomatic memory loss was present in 91% (10 of 11) of elderly patients with FTLD compared with only 36% (7 of 19) of patients with presenile-onset FTLD. Neuropathologically, the group of elderly patients with FTLD comprised 7 with FTLD-TDP-43, 1 with ubiquitin-positive FTLD, 2 with FTLD-tau/Pick disease, and 1 with FTLD-tau/neurofibrillary tangle-predominant dementia with TDP-43, a composition similar to presenile-onset FTLD. However, hippocampal sclerosis was more common in elderly patients with FTLD than patients with presenile-onset FTLD (82% vs 37%) and more severe in elderly patients with FTLD (P < .05). By contrast, severe atrophy of the frontal and temporal lobes was less common in elderly patients with FTLD (frontal: 45%; temporal: 27%) than patients with presenile-onset FTLD (frontal: 63%; temporal: 78%). Elderly patients with FTLD represented 3.2% of all elderly patients with dementia autopsied at Newcastle General Hospital.Conclusions: Frontotemporal lobar degeneration in elderly patients does exist as a separate entity from presenile-onset FTLD. Its main features include (1) clinically frequent memory loss and behavioral change predominating over language and semantic dysfunction and (2) neuropathologically prominent hippocampal sclerosis but less pronounced cortical lobar atrophy. Clinically, FTLD in elderly patients is underrecognized and should be considered in the elderly subjects presenting with an "atypical Alzheimer disease" phenotype.