Neuroscience Research Australia, Randwick, Australia.
Neurology. 2013 Mar 5;80(10):897-903. doi: 10.1212/WNL.0b013e318285c15b. Epub 2013 Feb 6.
To track cognitive and language changes over time in patients with logopenic (lv-PPA) and semantic (sv-PPA) variants of primary progressive aphasia (PPA).
Thirteen consecutive patients with lv-PPA and 11 patients with sv-PPA underwent yearly evaluation for a mean of 3 years. Nineteen patients (11 lv-PPA, 8 sv-PPA) had Pittsburgh compound B PET scans. Outcome variables included the Mini-Mental State Examination, Addenbrooke's Cognitive Examination-revised (ACE-R) with its 5 cognitive subscores, and 3 language tasks based on single word processing. Mixed-models regressions were used to estimate the differential rate of decline between cohorts.
Despite equivalent level of baseline impairment, the lv-PPA cohort showed more rapid and generalized cognitive decline that involved nonverbal domains, with the majority of cases meeting criteria for dementia within 12 months. By contrast, cognitive changes in the sv-PPA cohort were slower and remained confined to verbally mediated tasks.
Patients with lv-PPA are on the cusp of global dementia that typically develops quite rapidly, contrasting with the long period of circumscribed semantic impairment seen in patients with sv-PPA. The ACE-R appears capable of monitoring decline, which is relevant to therapeutic trials.
追踪以失读型(lv-PPA)和语义型(sv-PPA)为主的原发性进行性失语症(PPA)患者认知和语言随时间的变化。
连续纳入 13 名 lv-PPA 患者和 11 名 sv-PPA 患者,平均随访 3 年。19 名患者(11 名 lv-PPA,8 名 sv-PPA)进行了匹兹堡化合物 B PET 扫描。主要结局变量包括简易精神状态检查、改良 Addenbrooke 认知评估(ACE-R)及其 5 项认知子评分和 3 项基于单词处理的语言任务。采用混合模型回归估计两组间的差异率下降。
尽管基线损伤水平相当,但 lv-PPA 组表现出更快和更广泛的认知衰退,涉及非言语领域,多数病例在 12 个月内符合痴呆标准。相比之下,sv-PPA 组的认知变化较慢,仍局限于与言语相关的任务。
lv-PPA 患者处于典型快速发展的全面痴呆的边缘,与 sv-PPA 患者所经历的长时间局限于语义的损害形成鲜明对比。ACE-R 似乎能够监测病情的衰退,这与治疗试验相关。