Whitwell Jennifer L, Weigand Stephen D, Duffy Joseph R, Clark Heather M, Strand Edythe A, Machulda Mary M, Spychalla Anthony J, Senjem Matthew L, Jack Clifford R, Josephs Keith A
From the Department of Radiology (J.L.W., A.J.S., M.L.S., C.R.J.), Department of Health Sciences Research (Biostatistics) (S.D.W.), Department of Neurology (Speech Pathology) (J.R.D., H.M.C., E.A.S.), Department of Psychiatry & Psychology (Neuropsychology) (M.M.M.), Department of Information Technology (M.L.S.), and Department of Neurology (Behavioral Neurology and Movement Disorders) (K.A.J.), Mayo Clinic, Rochester, MN.
Neurology. 2017 Nov 28;89(22):2271-2279. doi: 10.1212/WNL.0000000000004685. Epub 2017 Nov 1.
To determine whether baseline clinical and MRI features predict rate of clinical decline in patients with progressive apraxia of speech (AOS).
Thirty-four patients with progressive AOS, with AOS either in isolation or in the presence of agrammatic aphasia, were followed up longitudinally for up to 4 visits, with clinical testing and MRI at each visit. Linear mixed-effects regression models including all visits (n = 94) were used to assess baseline clinical and MRI variables that predict rate of worsening of aphasia, motor speech, parkinsonism, and behavior. Clinical predictors included baseline severity and AOS type. MRI predictors included baseline frontal, premotor, motor, and striatal gray matter volumes.
More severe parkinsonism at baseline was associated with faster rate of decline in parkinsonism. Patients with predominant sound distortions (AOS type 1) showed faster rates of decline in aphasia and motor speech, while patients with segmented speech (AOS type 2) showed faster rates of decline in parkinsonism. On MRI, we observed trends for fastest rates of decline in aphasia in patients with relatively small left, but preserved right, Broca area and precentral cortex. Bilateral reductions in lateral premotor cortex were associated with faster rates of decline of behavior. No associations were observed between volumes and decline in motor speech or parkinsonism.
Rate of decline of each of the 4 clinical features assessed was associated with different baseline clinical and regional MRI predictors. Our findings could help improve prognostic estimates for these patients.
确定基线临床和MRI特征是否能预测进行性言语失用症（AOS）患者的临床衰退率。
对34例进行性AOS患者进行纵向随访，随访最多4次，每次随访均进行临床测试和MRI检查。这些患者单独患有AOS或伴有语法性失语。采用包含所有随访（n = 94）的线性混合效应回归模型，评估预测失语症、运动性言语、帕金森症和行为恶化率的基线临床和MRI变量。临床预测因素包括基线严重程度和AOS类型。MRI预测因素包括基线额叶、运动前区、运动区和纹状体灰质体积。
基线时帕金森症越严重，其衰退速度越快。以声音扭曲为主（AOS 1型）的患者，失语症和运动性言语的衰退速度更快；而以言语分段为主（AOS 2型）的患者，帕金森症的衰退速度更快。在MRI上，我们观察到左侧Broca区和中央前回相对较小但右侧保留的患者，失语症衰退速度最快。双侧外侧运动前皮层体积减小与行为衰退速度加快有关。未观察到灰质体积与运动性言语或帕金森症衰退之间的关联。
所评估的4种临床特征的衰退率与不同的基线临床和局部MRI预测因素相关。我们的研究结果有助于改善对这些患者的预后评估。
