VA Greater Los Angeles Healthcare System, Los Angeles, CA, USA.
Am J Alzheimers Dis Other Demen. 2011 Feb;26(1):72-7. doi: 10.1177/1533317510391239.
Extrapyramidal signs (EPS) may vary across 3 major subtypes of primary progressive aphasia (PPA): progressive nonfluent aphasia (PNFA), semantic dementia (SD), and progressive logopenic aphasia (PLA).
We reviewed initial neurological examinations from a clinical PPA cohort (PNFA = 49, SD = 26, PLA = 28) to determine the prevalence of specific categories of EPS.
The presence of any EPS was more common in PNFA (38.8%) and PLA (35.7%) than in SD (3.8%). The PNFA group exhibited the highest prevalence of bradykinesia (PNFA: 22.4%, SD: 3.8%, PLA: 0.0%) and rigidity (PNFA: 30.6%, SD: 0.0%, PLA: 10.7%). Calculated positive likelihood ratios indicated bradykinesia (12.1) or rigidity (5.5) was more strongly associated with PNFA than other PPAs.
These findings suggest that on initial presentation, specific EPS may help distinguish PPA subtypes when linguistic and/or neuroimaging profiles are indistinct. Moreover, EPS could represent a marker of underlying tauopathy, linking clinical presentation to neuropathology in PPA.
原发性进行性失语症(PPA)的 3 种主要亚型(进行性非流利性失语症 [PNFA]、语义性痴呆症 [SD] 和进行性命名性失语症 [PLA])可能存在不同的锥体外系症状(EPS)。
我们回顾了临床 PPA 队列的初始神经检查结果(PNFA=49 例,SD=26 例,PLA=28 例),以确定特定类别 EPS 的患病率。
PNFA(38.8%)和 PLA(35.7%)的 EPS 阳性率高于 SD(3.8%)。PNFA 组的运动迟缓(PNFA:22.4%,SD:3.8%,PLA:0.0%)和僵硬(PNFA:30.6%,SD:0.0%,PLA:10.7%)患病率最高。计算得出的阳性似然比表明,运动迟缓(12.1)或僵硬(5.5)与 PNFA 的相关性强于其他 PPA。
这些发现表明,在初始表现时,特定的 EPS 可能有助于区分语言和/或神经影像学特征不明确的 PPA 亚型。此外,EPS 可能代表了 tau 蛋白病的标志物,将临床表现与 PPA 的神经病理学联系起来。