Odolil Adam, Wright Amy E, Keator Lynsey M, Sheppard Shannon M, Breining Bonnie, Tippett Donna C, Hillis Argye E
Department of Neurology, Johns Hopkins University School of Medicine.
Department of Physical Medicine & Rehabilitation, Johns Hopkins University School of Medicine.
Aphasiology. 2020;34(3):365-375. doi: 10.1080/02687038.2019.1594152. Epub 2019 Mar 23.
The rate of decline in language in Primary Progressive Aphasia (PPA) is highly variable and difficult to predict at baseline. The severity of diffuse white matter disease (leukoaraiosis), a marker of overall brain health, may substantially influence the rate of decline.
To test the hypothesis that leukoaraiosis is associated with a steeper decline in naming in PPA.
In this longitudinal, observational study, 29 individuals with PPA (all variants) were administered the Boston Naming Test (BNT) at baseline and 1 year later. Two raters evaluated leukoaraiosis on baseline MRI, using the Cardiovascular Health Study scale. We evaluated the effects of leukoaraiosis severity, age, education, and baseline BNT on decline measured by change in BNT accuracy with multivariable linear regression. We also evaluated the effects of these variables on the dichotomized outcome of faster decline in BNT (worst 50%) versus slower decline (best 50%) using logistic regression.
Together, leukoaraiosis, age, education, and baseline BNT score predicted change in BNT score (F(3, 25) = 8.12; p=0.0006). Change in BNT score was predicted by severity of leukoaraiosis (t =-3.81; p=0.001) and education (t= -2.45; p=0.022), independently of the other variables. When we dichotomized outcome into upper 50 percentile versus lower 50 percentile (faster decline), faster decline was predicted by all variables together (chi squared = 13.91; p = 0.008). However, only leukoaraiosis independently predicted outcome (OR=2.80; 95%CI: 1.11 to 7.03). For every 1 point increase on the CHS rating scale, there was 2.8 times higher chance of showing faster decline in naming.
Severity of leukoaraiosis is associated with steeper decline in naming in PPA. This imaging marker can aide in prognosis and planning by caregivers and stratification of participants in clinical trials.
原发性进行性失语（PPA）患者语言功能衰退的速度差异很大，在疾病初期很难预测。弥漫性白质病变（脑白质疏松症）的严重程度是整体脑健康的一个指标，可能会极大地影响衰退速度。
检验脑白质疏松症与PPA患者命名能力更快衰退相关的假设。
在这项纵向观察性研究中，29名PPA患者（所有亚型）在基线期和1年后接受了波士顿命名测试（BNT）。两名评估者使用心血管健康研究量表，在基线期MRI上评估脑白质疏松症。我们通过多变量线性回归，评估脑白质疏松症严重程度、年龄、教育程度和基线期BNT对以BNT准确性变化衡量的衰退的影响。我们还使用逻辑回归评估了这些变量对BNT更快衰退（最差的50%）与较慢衰退（最好的50%）这一二分结果的影响。
脑白质疏松症、年龄、教育程度和基线期BNT分数共同预测了BNT分数的变化（F(3, 25) = 8.12；p = 0.0006）。BNT分数的变化由脑白质疏松症的严重程度（t = -3.81；p = 0.001）和教育程度（t = -2.45；p = 0.022）预测，与其他变量无关。当我们将结果分为上50%与下50%（更快衰退）时，所有变量共同预测了更快衰退（卡方 = 13.91；p = 0.008）。然而，只有脑白质疏松症独立预测了结果（OR = 2.80；9可信区间：1.11至7.03）。在CHS评分量表上每增加1分，命名能力更快衰退的可能性就高出2.8倍。
脑白质疏松症的严重程度与PPA患者命名能力的更快衰退相关。这一影像学指标有助于护理人员进行预后判断和规划，以及在临床试验中对参与者进行分层。
