Department of Radiology and Biomedical Imaging, University of California, San Francisco, California, USA.
Department of Neurology, University of California, San Francisco, California, USA.
Hum Brain Mapp. 2023 Oct 1;44(14):4833-4847. doi: 10.1002/hbm.26408. Epub 2023 Jul 30.
Overlapping clinical presentations in primary progressive aphasia (PPA) variants present challenges for diagnosis and understanding pathophysiology, particularly in the early stages of the disease when behavioral (speech) symptoms are not clearly evident. Divergent atrophy patterns (temporoparietal degeneration in logopenic variant lvPPA, frontal degeneration in nonfluent variant nfvPPA) can partially account for differential speech production errors in the two groups in the later stages of the disease. While the existing dogma states that neurodegeneration is the root cause of compromised behavior and cortical activity in PPA, the extent to which neurophysiological signatures of speech dysfunction manifest independent of their divergent atrophy patterns remain unknown. We test the hypothesis that nonword deficits in lvPPA and nfvPPA arise from distinct patterns of neural oscillations that are unrelated to atrophy. We use a novel structure-function imaging approach integrating magnetoencephalographic imaging of neural oscillations during a non-word repetition task with voxel-based morphometry-derived measures of gray matter volume to isolate neural oscillation abnormalities independent of atrophy. We find reduced beta band neural activity in left temporal regions associated with the late stages of auditory encoding unique to patients with lvPPA and reduced high-gamma neural activity over left frontal regions associated with the early stages of motor preparation in patients with nfvPPA. Neither of these patterns of reduced cortical oscillations was explained by cortical atrophy in our statistical model. These findings highlight the importance of structure-function imaging in revealing neurophysiological sequelae in early stages of dementia when neither structural atrophy nor behavioral deficits are clinically distinct.
原发性进行性失语症 (PPA) 变体的重叠临床表现对诊断和理解病理生理学提出了挑战,尤其是在疾病的早期阶段,此时行为(言语)症状并不明显。不同的萎缩模式(在 logopenic 变体 lvPPA 中颞顶叶退化,在非流利变体 nfvPPA 中额部退化)可以部分解释两组在疾病后期言语产生错误的差异。虽然现有的理论认为神经退行性变是 PPA 中行为和皮质活动受损的根本原因,但言语功能障碍的神经生理特征在多大程度上独立于其不同的萎缩模式表现出来尚不清楚。我们检验了以下假设:lvPPA 和 nfvPPA 中的无词缺陷源于与萎缩无关的不同神经振荡模式。我们使用一种新的结构-功能成像方法,将神经振荡的脑磁图成像与基于体素形态测量学的灰质体积测量相结合,以分离与萎缩无关的神经振荡异常。我们发现,lvPPA 患者左颞区与听觉编码晚期相关的β频段神经活动减少,nfvPPA 患者左额区与运动准备早期相关的高频γ频段神经活动减少。在我们的统计模型中,这些皮质振荡的减少模式都不能用皮质萎缩来解释。这些发现强调了结构-功能成像在揭示痴呆早期神经生理后果方面的重要性,因为在这个阶段,无论是结构萎缩还是行为缺陷都没有明显的临床差异。