Rosen Howard J, Kramer Joel H, Gorno-Tempini Maria Luisa, Schuff Norbert, Weiner Michael, Miller Bruce L
Department of Neurology, Memory and Aging Center, University of California at San Francisco, CA 94143-1207, USA.
Am J Geriatr Psychiatry. 2002 Jan-Feb;10(1):89-97.
The authors illustrate the spectrum of clinical and imaging patterns in primary progressive aphasia (PPA), a syndrome of slowly progressive speech and language impairment occurring with neurodegenerative disease. Although PPA presents with relatively isolated impairment in language, many patients progress to global cognitive or behavioral dysfunction. The syndrome may be associated with frontotemporal dementia (FTD)- or Alzheimer disease (AD)-type changes. Authors describe the clinical presentation in three cases of PPA and analyze the pattern of cerebral atrophy in each case with voxel-based morphometry. Two patients presented with nonfluent progressive aphasia. Subtle differences in the clinical features were suggestive of FTD in one case and AD in the other. Neuroimaging revealed a predominance of frontal atrophy in the first case and temporo-parietal atrophy in the second. The third case presented with the syndrome of semantic dementia and showed the typical behavioral problems associated with FTD and a pattern of left-greater-than-right temporal atrophy. Different clinical syndromes in PPA are associated with different patterns of atrophy. In the future, combined analysis of imaging and clinical characteristics may allow more accurate etiologic diagnosis.
作者阐述了原发性进行性失语（PPA）的临床和影像学表现谱，PPA是一种与神经退行性疾病相关的缓慢进展性言语和语言障碍综合征。虽然PPA表现为相对孤立的语言障碍，但许多患者会进展为全面的认知或行为功能障碍。该综合征可能与额颞叶痴呆（FTD）或阿尔茨海默病（AD）类型的改变有关。作者描述了3例PPA患者的临床表现，并采用基于体素的形态学测量方法分析了每例患者的脑萎缩模式。2例患者表现为非流利性进行性失语。临床特征的细微差异提示1例为FTD，另1例为AD。神经影像学显示，第1例以额叶萎缩为主，第2例以颞顶叶萎缩为主。第3例表现为语义性痴呆综合征，显示出与FTD相关的典型行为问题以及左侧颞叶萎缩大于右侧的模式。PPA的不同临床综合征与不同的萎缩模式相关。未来，影像学和临床特征的联合分析可能有助于更准确的病因诊断。
