Rascovsky K, Salmon D P, Lipton A M, Leverenz J B, DeCarli C, Jagust W J, Clark C M, Mendez M F, Tang-Wai D F, Graff-Radford N R, Galasko D
University of California, San Diego, La Jolla, CA 92093-0948, USA.
Neurology. 2005 Aug 9;65(3):397-403. doi: 10.1212/01.wnl.0000171343.43314.6e.
To compare survival and rates of cognitive and functional decline in patients with autopsy-confirmed frontotemporal dementia (FTD) and Alzheimer disease (AD) in a large multicenter study.
Despite advances in the clinical characterization of FTD, little is known about its rate of progression. Characterizing survival and rate of decline in FTD is important because it can provide prognostic guidelines and benchmarks to use in the evaluation of disease-modifying drugs.
Seventy patients with FTD and 70 patients with AD who were followed by seven Alzheimer disease research centers until confirmation of diagnosis at autopsy were matched for overall age, education, and Mini-Mental State Examination (MMSE) score at initial evaluation. Survival and rates of cognitive and functional decline were compared.
Patients with FTD had significantly shorter survival from initial evaluation to death than patients with AD (FTD = 4.2 years, AD = 6.0 years; log-rank test = 5.17, p < 0.05), and they declined significantly faster over one year on the MMSE (mean annual rate of change: -6.7 points for FTD vs -2.3 points for AD). A significantly greater proportion of patients with FTD were impaired in basic activities of daily living (ADLs) at initial evaluation, and lost the capacity for independent or minimally-assisted ADLs over the subsequent year.
The results are consistent with shorter survival and faster rates of cognitive and functional decline in patients with frontotemporal dementia (FTD) compared to those with Alzheimer disease (AD). This suggests that FTD follows a more malignant disease course than AD once dementia is clinically recognized.
在一项大型多中心研究中，比较经尸检确诊的额颞叶痴呆（FTD）和阿尔茨海默病（AD）患者的生存率以及认知和功能衰退率。
尽管FTD的临床特征研究取得了进展，但其进展速度仍知之甚少。明确FTD的生存率和衰退率很重要，因为这可为评估疾病修饰药物提供预后指导和基准。
70例FTD患者和70例AD患者由7个阿尔茨海默病研究中心进行随访，直至尸检确诊，对初始评估时的总体年龄、教育程度和简易精神状态检查表（MMSE）评分进行匹配。比较生存率以及认知和功能衰退率。
从初始评估到死亡，FTD患者的生存期显著短于AD患者（FTD = 4.2年，AD = 6.0年；对数秩检验= 5.17，p < 0.05），且他们在MMSE上一年中的衰退速度明显更快（平均年变化率：FTD为-6.7分，AD为-2.3分）。在初始评估时，FTD患者中日常生活基本活动（ADL）受损的比例显著更高，且在随后一年中失去了独立或在最低程度协助下进行ADL的能力。
结果表明，与阿尔茨海默病（AD）患者相比，额颞叶痴呆（FTD）患者的生存期更短，认知和功能衰退速度更快。这表明一旦临床上确诊为痴呆，FTD的疾病进程比AD更具恶性。
