Brambati Simona Maria, Amici Serena, Racine Caroline A, Neuhaus John, Miller Zachary, Ogar Jenny, Dronkers Nina, Miller Bruce L, Rosen Howard, Gorno-Tempini Maria Luisa
Centre de Recherche de l'Institut Universitaire de Gériatrie de Montréal, Montréal, QC, Canada.
Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, CA, USA.
Neuroimage Clin. 2015 Jan 22;8:345-55. doi: 10.1016/j.nicl.2015.01.011. eCollection 2015.
The present study investigated the pattern of longitudinal changes in cognition and anatomy in three variants of primary progressive aphasia (PPA). Eight patients with the non-fluent variant of PPA (nfvPPA), 13 patients with the semantic variant (svPPA), seven patients with the logopenic variant (lvPPA), and 29 age-matched, neurologically healthy controls were included in the study. All participants underwent longitudinal MRI, neuropsychological and language testing at baseline and at a 1-year follow-up. Tenser-based morphometry (TBM) was applied to T1-weighted MRI images in order to map the progression of gray and white matter atrophy over a 1-year period. Results showed that each patient group was characterized by a specific pattern of cognitive and anatomical changes. Specifically, nfvPPA patients showed gray matter atrophy progression in the left frontal and subcortical areas as well as a decline in motor speech and executive functions; svPPA patients presented atrophy progression in the medial and lateral temporal lobe and decline in semantic memory abilities; and lvPPA patients showed atrophy progression in lateral/posterior temporal and medial parietal regions with a decline in memory, sentence repetition and calculations. In addition, in all three variants, the white matter fibers underlying the abovementioned cortical areas underwent significant volume contraction over a 1-year period. Overall, these results indicate that the three PPA variants present distinct patterns of neuroanatomical contraction, which reflect their clinical and cognitive progression.
本研究调查了三种原发性进行性失语（PPA）变体在认知和解剖结构方面的纵向变化模式。研究纳入了8例非流利型PPA（nfvPPA）患者、13例语义型PPA（svPPA）患者、7例音韵型PPA（lvPPA）患者以及29名年龄匹配的神经功能正常的对照者。所有参与者在基线期和1年随访期均接受了纵向MRI、神经心理学和语言测试。基于张量的形态测量法（TBM）应用于T1加权MRI图像，以描绘1年内灰质和白质萎缩的进展情况。结果显示，每个患者组都具有特定的认知和解剖学变化模式。具体而言，nfvPPA患者在左侧额叶和皮质下区域出现灰质萎缩进展，同时运动性言语和执行功能下降；svPPA患者在内侧和外侧颞叶出现萎缩进展，语义记忆能力下降；lvPPA患者在外侧/后颞叶和内侧顶叶区域出现萎缩进展，同时记忆、句子复述和计算能力下降。此外，在所有三种变体中，上述皮质区域下方的白质纤维在1年内均出现显著体积缩小。总体而言，这些结果表明，三种PPA变体呈现出不同的神经解剖学萎缩模式，这反映了它们的临床和认知进展情况。
