Global Brain Health Institute.
Department of Neurology, Buddhist Tzu Chi General Hospital, Hualien, Taiwan.
Curr Opin Neurol. 2019 Apr;32(2):255-265. doi: 10.1097/WCO.0000000000000673.
Knowledge on primary progressive aphasia (PPA) has expanded rapidly in the past few decades. Clinical characteristics, neuroimaging correlates, and neuropathological features of PPA are better delineated. This facilitates scientific studies on the disease pathophysiology and allows speech and language therapy to be more precisely targeted. This review article begins with a summary of the current understanding of PPA and discusses how PPA can serve as a model to promote scientific discovery in neurodegenerative diseases.
Studies on the different variants of PPA have demonstrated the high compatibility between clinical presentations and neuroimaging features, and in turn, enhances the understanding of speech and language neuroanatomy. In addition to the traditional approach of lesion-based or voxel-based mapping, scientists have also adopted functional connectivity and network topology approaches that permits a more multidimensional understanding of neuroanatomy. As a result, pharmacological and cognitive therapeutic strategies can now be better targeted towards specific pathological/molecular and cognitive subtypes.
Recent scientific advancement in PPA potentiates it to be an optimal model for studying brain network vulnerability, neurodevelopment influences and the effects of nonpharmacological intervention in neurodegenerative diseases.
原发性进行性失语症（PPA）的知识在过去几十年中迅速发展。PPA 的临床特征、神经影像学相关性和神经病理学特征得到了更好的描绘。这有助于对疾病病理生理学进行科学研究，并使言语和语言治疗更有针对性。本文首先总结了目前对 PPA 的认识，并讨论了 PPA 如何作为促进神经退行性疾病科学发现的模型。
对 PPA 不同变体的研究表明，临床表现和神经影像学特征之间具有很高的兼容性，从而增强了对言语和语言神经解剖学的理解。除了传统的基于病变或体素的映射方法外，科学家们还采用了功能连接和网络拓扑方法，从而可以更全面地理解神经解剖学。因此，现在可以针对特定的病理/分子和认知亚型，更好地靶向药物和认知治疗策略。
PPA 的最新科学进展使其成为研究大脑网络脆弱性、神经发育影响以及神经退行性疾病中非药物干预效果的理想模型。
