Department of Neurology, University Hospital Ulm, Ulm, Germany.
Institute for Epidemiology and Medical Biometry, University of Ulm, Ulm, Germany.
Alzheimers Dement. 2021 Oct;17(10):1595-1609. doi: 10.1002/alz.12323. Epub 2021 Mar 30.
The term primary progressive aphasia (PPA) sums up the non-fluent (nfv), the semantic (sv), and the logopenic (lv) variant. Up to now, there is only limited data available concerning magnetic resonance imaging volumetry to monitor disease progression.
Structural brain imaging and an extensive assessment were applied at baseline and up to 4-year(s) follow-up in 269 participants. With automated atlas-based volumetry 56 brain regions were assessed. Atrophy progression served to calculate sample sizes for therapeutic trials.
At baseline highest atrophy appeared in parts of the left frontal lobe for nfvPPA (-17%) and of the left temporal lobe for svPPA (-34%) and lvPPA (-24%). Severest progression within 1-year follow-up occurred in the basal ganglia in nfvPPA (-7%), in the hippocampus/amygdala in svPPA (-9%), and in (medial) temporal regions in lvPPA (-6%).
PPA presents as a left-dominant, mostly gray matter sensitive disease with considerable atrophy at baseline that proceeds variant-specific.
原发性进行性失语症（PPA）包括非流利型（nfv）、语义型（sv）和流畅型失语症（lv）。到目前为止，关于磁共振成像体积测量来监测疾病进展的相关数据非常有限。
在 269 名参与者的基线和长达 4 年（s）的随访中进行了结构脑成像和广泛的评估。使用基于自动图谱的体积测量评估了 56 个脑区。通过计算萎缩进展来确定治疗试验的样本量。
在基线时，nfvPPA 的左侧额叶（-17%）和 svPPA 和 lvPPA 的左侧颞叶（-34% 和 -24%）出现了最高的萎缩。在 1 年随访期间，nfvPPA 的基底节（-7%）、svPPA 的海马体/杏仁核（-9%）和 lvPPA 的（内侧）颞叶（-6%）的萎缩进展最严重。
PPA 表现为左侧优势、主要累及灰质的疾病，在基线时有明显的萎缩，且进展具有变体特异性。
