Kipps C M, Nestor P J, Dawson C E, Mitchell J, Hodges J R
Department of Clinical Neurosciences, University of Cambridge, UK.
Neurology. 2008 May 27;70(22):2046-52. doi: 10.1212/01.wnl.0000313366.76973.8a.
There is a need for instruments which can measure progression of disease in frontotemporal dementia (FTD), particularly with respect to the assessment of potential therapeutic agents.
The Cambridge Early Onset Dementia Clinic database was reviewed for all prospectively enrolled cases of FTD with documented scores on the Mini-Mental State Examination (MMSE) or Addenbrooke's Cognitive Examination (ACE) on at least two occasions. We identified 50 cases fulfilling these criteria: pathologic confirmation was present in 11 of 16 patients who had died, 12 of the remainder had imaging abnormalities on their initial scans, and 22 had structural scans no different from controls. We compared these groups to a cohort with early AD (n = 25) and healthy controls (n = 10).
There was clear cognitive decline (measured by the MMSE and ACE) in patients who had died, and those with documented atrophy on initial MRI scan. In contrast, patients with FTD with normal scans showed no change in cognitive scores over a much longer interval, and serial ACE measurements paralleled those of controls. Power calculations showed that the inclusion of these patients with FTD would significantly increase the number of cases needed in any therapeutic trial.
Addenbrooke's Cognitive Examination is a simple monitoring tool which can detect progression of disease in frontotemporal dementia over a 1- to 2-year interval without the need for serial imaging. We estimated that a clinical trial that enrolled subjects with abnormal MR scans would require 135 subjects per group to detect a small effect, and 35 for a medium effect.
需要能够测量额颞叶痴呆（FTD）疾病进展的工具，特别是在评估潜在治疗药物方面。
对剑桥早发性痴呆诊所数据库进行回顾，纳入所有前瞻性登记的FTD病例，这些病例至少两次记录了简易精神状态检查（MMSE）或Addenbrooke认知检查（ACE）的分数。我们确定了50例符合这些标准的病例：16例死亡患者中有11例有病理证实，其余患者中有12例在初次扫描时有影像学异常，22例的结构扫描与对照组无差异。我们将这些组与早期AD队列（n = 25）和健康对照组（n = 10）进行比较。
死亡患者以及初次MRI扫描有萎缩记录的患者出现明显的认知下降（通过MMSE和ACE测量）。相比之下，扫描正常的FTD患者在更长的时间间隔内认知分数没有变化，连续的ACE测量结果与对照组相似。功效计算表明，纳入这些FTD患者将显著增加任何治疗试验所需的病例数。
Addenbrooke认知检查是一种简单的监测工具，可在1至2年间隔内检测额颞叶痴呆的疾病进展，无需进行系列成像。我们估计，纳入MRI扫描异常受试者的临床试验每组需要135名受试者才能检测到小的效应，35名受试者才能检测到中等效应。
