Manchester Academic Health Sciences Centre, Cerebral Function Unit, Greater Manchester Neuroscience Centre, Salford Royal NHS Foundation Trust, Salford, UK.
Neurology. 2013 May 14;80(20):1881-7. doi: 10.1212/WNL.0b013e318292a342. Epub 2013 Apr 17.
We aimed to assess sensitivity and specificity of the updated criteria for behavioral variant frontotemporal dementia (bvFTD) based on a large autopsy-confirmed cohort of patients with dementia.
Two hundred thirty-nine consecutive pathologically confirmed dementia patients, clinically assessed in a specialist cognitive unit were identified. Patients with predominant aphasia, motor disorders, or insufficient clinical information were excluded. Frontotemporal Dementia Consensus criteria were applied to anonymized clinical data taken from patients' initial assessment by raters who were blinded to clinical and pathologic diagnosis.
The final study cohort comprised 156 patients with predominantly early-onset dementia. The updated criteria for possible bvFTD had a sensitivity of 95% and specificity of 82%. Probable bvFTD criteria had a sensitivity of 85% and specificity of 95%. False positives were predominantly patients with presenile Alzheimer disease.
Revised diagnostic criteria show encouragingly high sensitivity and specificity when applied to patients with early-onset dementia. They therefore provide a useful tool both for specialist researchers and general clinicians. There is a need for further prospective studies of sensitivity and specificity involving a broader spectrum of patients with dementia.
我们旨在评估基于大型尸检确诊的痴呆患者队列的行为变异额颞叶痴呆（bvFTD）更新标准的敏感性和特异性。
确定了 239 例连续的经病理证实的痴呆患者，这些患者在认知专科进行了临床评估。排除了以语言障碍、运动障碍为主或临床资料不足的患者。应用额颞叶痴呆共识标准对匿名的临床数据进行评估，这些数据来自于评估者对患者的初始评估，评估者对临床和病理诊断是盲法的。
最终的研究队列包括 156 例以早期发病的痴呆为主的患者。可能的 bvFTD 更新标准的敏感性为 95%，特异性为 82%。可能的 bvFTD 标准的敏感性为 85%，特异性为 95%。假阳性主要是早发性阿尔茨海默病患者。
修订后的诊断标准在应用于早发性痴呆患者时显示出令人鼓舞的高敏感性和特异性。因此，它们为专科研究人员和普通临床医生提供了一种有用的工具。需要进一步进行涉及更广泛痴呆患者的敏感性和特异性的前瞻性研究。
