Department of Neurology, University Hospital Münster, Münster, Germany.
J Neurol Neurosurg Psychiatry. 2015 Jul;86(7):809-15. doi: 10.1136/jnnp-2014-308773. Epub 2014 Sep 23.
Despite refined criteria for behavioural variant frontotemporal dementia (bvFTD), its differentiation from Alzheimer's dementia (AD) remains difficult at early clinical presentation. Apraxia is not considered as a supportive feature for the diagnosis of bvFTD, but for AD. However, only few studies have quantified praxis disturbances in mild disease stages and their specificity for AD compared with bvFTD remains indistinct. We explore apraxia in bvFTD and investigate the differential validity of apraxia screening tests to distinguish between AD, bvFTD and healthy controls (HC).
We compared composite apraxia scores assessed with standardised neuropsychological screening tests as well as performance in praxis subdomains in patients who fulfil current clinical criteria for AD (N=20), bvFTD (N=20), and in HC (N=20).
Composite scores of apraxia screening tests provided high diagnostic accuracy for detecting mild stages of both neurodegenerative disorders compared with HC (sensitivity: 75-95%; specificity: 70-90%). Both patient groups showed pronounced impairments in limb praxis, especially in imitation of hand and finger postures (bvFTD: 71.7%; AD: 55.5%; HC: 86.7%) and pantomime of object use (bvFTD: 88.6%; AD: 81.4%; HC: 97.5%). Beyond that, patients with bvFTD displayed a unique profile of deficits for imitating face postures (bvFTD: 69%; AD: 88%; HC: 95.5%).
Praxis disturbances are important but under-represented diagnostic features in mild stages of AD and bvFTD. Apraxia screening tests are easily applicable diagnostic tools, which may support clinical diagnoses of both neurodegenerative diseases. The analysis of individual apraxia profiles can effectively facilitate differential diagnosis of AD and bvFTD.
尽管行为变异额颞叶痴呆(bvFTD)的诊断标准已经得到了完善,但在早期临床阶段,它与阿尔茨海默病(AD)的区分仍然很困难。失用症不被认为是 bvFTD 诊断的支持特征,但对于 AD 则是。然而,只有少数研究在轻度疾病阶段量化了运动障碍,并比较了其对 AD 和 bvFTD 的特异性,结果仍不明确。我们探索了 bvFTD 中的失用症,并研究了失用症筛查测试对 AD、bvFTD 和健康对照组(HC)的鉴别有效性。
我们比较了符合 AD(N=20)、bvFTD(N=20)和 HC(N=20)当前临床标准的患者使用标准化神经心理学筛查测试评估的综合失用症评分,以及在执行失用症子领域方面的表现。
与 HC 相比,失用症筛查测试的综合评分对检测两种神经退行性疾病的轻度阶段具有较高的诊断准确性(敏感性:75-95%;特异性:70-90%)。两组患者在手和手指姿势的模仿(bvFTD:71.7%;AD:55.5%;HC:86.7%)和物体使用的模仿(bvFTD:88.6%;AD:81.4%;HC:97.5%)方面都表现出明显的运动障碍。除此之外,bvFTD 患者还表现出模仿面部姿势的独特缺陷(bvFTD:69%;AD:88%;HC:95.5%)。
在 AD 和 bvFTD 的轻度阶段,运动障碍是重要但被低估的诊断特征。失用症筛查测试是易于应用的诊断工具,可支持两种神经退行性疾病的临床诊断。分析个体失用症特征谱可有效促进 AD 和 bvFTD 的鉴别诊断。