Torralva Teresa, Sposato Luciano A, Riccio Patricia M, Gleichgerrcht Ezequiel, Roca María, Toledo Jon B, Trojanowski John Q, Kukull Walter A, Manes Facundo, Hachinski Vladimir
Institute of Cognitive Neurology (INECO), Buenos Aires, Argentina; UDP-INECO Foundation Core on Neuroscience (UIFCoN), Diego Portales University, Santiago, Chile; Institute of Neurosciences, Favaloro University, Buenos Aires, Argentina; Australian Research Council (ACR), Centre of Excellence in Cognition and its Disorders, Sydney, Australia.
Department of Clinical Neurological Sciences, London Health Sciences Centre, Western Ontario University, London, Ontario, Canada.
Neurobiol Aging. 2015 Oct;36(10):2861-8. doi: 10.1016/j.neurobiolaging.2015.06.026. Epub 2015 Jul 3.
Diagnosing behavioral variant frontotemporal dementia (bvFTD) in patients with prior history of stroke or with silent brain infarcts on neuroimaging studies can be challenging. Vascular changes in patients with bvFTD are not unusual, but bvFTD tends to be ruled out in the presence of cerebrovascular disease. We aimed to identify the clinical, cognitive, and risk factor profile of bvFTD with coexistent cerebrovascular disease (V-bvFTD). We compared demographic data, clinical diagnoses, vascular risk factors, functional status, and normalized neuropsychological z-scores between patients with V-bvFTD versus bvFTD without concomitant cerebrovascular disease (NV-bvFTD) from the National Alzheimer's Coordinating Centre database. We included 391 neuropathologically-diagnosed cases of frontotemporal lobe degeneration. We excluded patients that were diagnosed with aphasic variants of frontotemporal dementia before death. Patients with V-bvFTD (n = 62) were older at the time of onset of cognitive decline (71.6 vs. 62.5 years, p < 0.001) and death (78.7 vs. 69.6, p < 0.001), more likely to be hypertensive (75.8% vs. 45.7%, p = 0.002) and to have a history of stroke (21.2% vs. 6.1%, p = 0.007) than those with NV-bvFTD (n = 329). V-bvFTD was often underdiagnosed, affected elderly patients, and had a similar cognitive profile as NV-bvFTD despite the presence of brain infarcts. In the whole cohort, we observed enhanced cognitive performance with increasing age quintiles despite larger proportions of cerebrovascular disease pathology, likely meaning that frontotemporal lobe degeneration-related primary neurodegeneration exerts a stronger impact on cognition than cerebrovascular disease. Coexisting cerebrovascular disease should not preclude the diagnosis of bvFTD.
对于既往有中风病史或神经影像学研究显示存在无症状脑梗死的患者，诊断行为变异型额颞叶痴呆（bvFTD）具有挑战性。bvFTD患者出现血管变化并不罕见，但在存在脑血管疾病的情况下，bvFTD往往会被排除。我们旨在确定合并脑血管疾病的bvFTD（V-bvFTD）的临床、认知和风险因素特征。我们比较了来自国家阿尔茨海默病协调中心数据库的V-bvFTD患者与无合并脑血管疾病的bvFTD（NV-bvFTD）患者的人口统计学数据、临床诊断、血管危险因素、功能状态和标准化神经心理学z评分。我们纳入了391例经神经病理学诊断的额颞叶变性病例。我们排除了在死亡前被诊断为额颞叶痴呆失语变体的患者。与NV-bvFTD患者（n = 329）相比，V-bvFTD患者（n = 62）在认知能力下降开始时（71.6岁对62.5岁，p < 0.001）和死亡时（78.7岁对69.6岁，p < 0.001）年龄更大，更有可能患有高血压（75.8%对45.7%，p = 0.002）和有中风病史（21.2%对6.1%，p = 0.007）。V-bvFTD常常被漏诊，影响老年患者，尽管存在脑梗死，但其认知特征与NV-bvFTD相似。在整个队列中，尽管脑血管疾病病理学比例较高，但我们观察到随着年龄五分位数的增加，认知能力有所提高，这可能意味着额颞叶变性相关的原发性神经退行性变对认知的影响比脑血管疾病更强。合并存在的脑血管疾病不应排除bvFTD的诊断。
