Kertesz Andrew, Davidson Wilda, McCabe Patricia, Takagi Kenji, Munoz David
Department of Clinical Neurological Sciences, St. Joseph's Health Care London, University of Western Ontario, London, Ontario, Canada.
J Int Neuropsychol Soc. 2003 Jul;9(5):710-9. doi: 10.1017/S1355617703950041.
A referred cohort of 67 clinically defined PPA patients were compared to 99 AD patients with formal language and nonverbal cognitive tests in a case control design. Language fluency was determined at the first and last follow up visits. Quantitation of sulcal and ventricular atrophy on MRI was carried out in 46 PPA and 53 AD patients. Most PPA patients (57%) are relatively fluent when first examined. Visuospatial and memory functions are initially preserved. Aphemic, stuttering, "pure motor" presentation, or agrammatic aphasia are seen less frequently. Later most PPAs become logopenic and nonfluent, even those with semantic aphasia (dementia). In contrast, AD patients were more fluent and had relatively lower comprehension, but better overall language performance. MRI showed significant left sided atrophy in most PPA patients. Subsequent to PPA, 25 patients developed behavioral manifestations of frontotemporal dementia and 15 the corticobasal degeneration syndrome, indicating the substantial clinical overlap of these conditions. Language testing, particularly fluency scores supported by neuroimaging are helpful differentiating PPA from AD. The fluent-nonfluent dichotomy in PPA is mostly stage related. The aphemic-logopenic-agrammatic and semantic distinction is useful, but the outcomes converge.
在一项病例对照研究设计中，将67例临床确诊的原发性进行性失语（PPA）患者与99例阿尔茨海默病（AD）患者进行对照，采用正式的语言和非语言认知测试。在首次和末次随访时测定语言流畅性。对46例PPA患者和53例AD患者进行了MRI上脑沟和脑室萎缩的定量分析。大多数PPA患者（57%）在首次检查时语言相对流畅。视觉空间和记忆功能最初得以保留。失语、口吃、“纯运动性”表现或语法性失语较少见。后来，大多数PPA患者会出现音韵性失语和非流畅性失语，即使是那些患有语义性失语（痴呆）的患者。相比之下，AD患者语言更流畅，理解能力相对较低，但总体语言表现较好。MRI显示大多数PPA患者左侧有明显萎缩。在PPA之后，25例患者出现了额颞叶痴呆的行为表现，15例出现了皮质基底节变性综合征，表明这些疾病在临床上有很大的重叠。语言测试，特别是神经影像学支持的流畅性评分，有助于区分PPA和AD。PPA中流畅性与非流畅性的二分法大多与阶段相关。失语-音韵性失语-语法性失语和语义性失语的区分是有用的，但结果趋同。
