Introduction:

There is a shortage of validated instruments to estimate disease progression in frontotemporal dementia (FTD).

Objectives:

To evaluate the ability of the FTD Rating Scale (FTD-FRS) to detect functional and behavioral changes in patients diagnosed with the behavioral variant of FTD (bvFTD), primary progressive aphasia (PPA), and Alzheimer disease (AD) after 12 months of the initial evaluation, compared to the Clinical Dementia Rating scale−frontotemporal lobar degeneration (CDR-FTLD) and the original Clinical Dementia Rating scale (CDR).

Methods:

The sample consisted of 70 individuals, aged 40+ years, with at least 2 years of schooling, 31 with the diagnosis of bvFTD, 12 with PPA (8 with semantic variant and 4 with non-fluent variant), and 27 with AD. The FTD-FRS, the CDR, and the 2 additional CDR-FTLD items were completed by a clinician, based on the information provided by the caregiver with frequent contact with the patient. The Addenbrooke Cognitive Examination-Revised was completed by patients. After 12 months, the same protocol was applied.

Results:

The FTD-FRS, CDR-FTLD, and CDR detected significant decline after 12 months in the 3 clinical groups (exception: FTD-FRS for PPA). The CDR was less sensitive to severe disease stages.

Conclusions:

The FTD-FRS and the CDR-FTLD are especially useful tools for dementia staging in AD and in the FTD spectrum.

中文翻译：

---

额颞叶痴呆和阿尔茨海默病的疾病进展：分期量表的贡献。

介绍：

缺乏经过验证的工具来估计额颞叶痴呆 (FTD) 的疾病进展。

目标：

评估 FTD 评定量表 (FTD-FRS) 检测被诊断患有 FTD 行为变异 (bvFTD)、原发性进行性失语症 (PPA) 和阿尔茨海默病 (AD) 12 个月后的功能和行为变化的能力。初始评估，与临床痴呆评定量表-额颞叶变性 (CDR-FTLD) 和原始临床痴呆评定量表 (CDR) 进行比较。

方法：

样本由 70 人组成，年龄在 40 岁以上，受过至少 2 年的学校教育，31 人被诊断为 bvFTD，12 人有 PPA（8 人有语义变异，4 人有非流利变异），27 人有 AD。FTD-FRS、CDR 和 2 个额外的 CDR-FTLD 项目由临床医生根据经常与患者接触的护理人员提供的信息完成。Addenbrooke Cognitive 检查-修订版由患者完成。12 个月后，应用相同的方案。

结果：

3 个临床组的 FTD-FRS、CDR-FTLD 和 CDR 在 12 个月后检测到显着下降（PPA 的 FTD-FRS 除外）。CDR 对严重疾病阶段不太敏感。

结论：

FTD-FRS 和 CDR-FTLD 是对 AD 和 FTD 谱中的痴呆分期特别有用的工具。