Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA.
Department of Neurology, Mayo Clinic, Rochester, MN, USA.
Alzheimers Dement. 2019 Sep;15(9):1218-1228. doi: 10.1016/j.jalz.2019.04.011. Epub 2019 Aug 6.
The corticobasal syndrome (CBS) is associated with several neuropathologic disorders, including corticobasal degeneration and Alzheimer's disease (AD).
In this report, we studied 43 AD patients with CBS (AD-CBS) and compared them with 42 AD patients with typical amnestic syndrome (AD-AS), as well as 15 cases of corticobasal degeneration and CBS pathology.
Unlike AD-AS, AD-CBS had prominent motor problems, including limb apraxia (90%), myoclonus (81%), and gait disorders (70%). Alien limb phenomenon was reported in 26% and cortical sensory loss in 14%. Language problems were also more frequent in AD-CBS, and memory impairment was less frequent. AD-CBS had more tau pathology in perirolandic cortices but less in superior temporal cortex than AD-AS. In addition, AD-CBS had greater neuronal loss in the substantia nigra.
AD-CBS is a clinicopathological subtype of AD with an atypical distribution of Alzheimer-type tau pathology. Greater neuronal loss in the substantia nigra may contribute to Parkinsonism which is not a feature of typical AD.
皮质基底节综合征（CBS）与多种神经病理学疾病相关，包括皮质基底节变性和阿尔茨海默病（AD）。
在本报告中，我们研究了 43 例具有 CBS 的 AD 患者（AD-CBS），并将其与 42 例具有典型遗忘综合征的 AD 患者（AD-AS）以及 15 例皮质基底节变性和 CBS 病理学患者进行了比较。
与 AD-AS 不同，AD-CBS 有明显的运动问题，包括肢体失用症（90%）、肌阵挛（81%）和步态障碍（70%）。26%的患者报告有异己手现象，14%的患者报告有皮质感觉丧失。AD-CBS 的语言问题也更为常见，记忆障碍则较少。AD-CBS 在大脑皮层运动区周围的 tau 病理学比 AD-AS 更为明显，但在颞上回的 tau 病理学则较少。此外，AD-CBS 的黑质神经元丢失更为严重。
AD-CBS 是 AD 的一种临床病理亚型，其阿尔茨海默型 tau 病理学分布不典型。黑质神经元丢失较多可能导致帕金森病，而帕金森病并非典型 AD 的特征。
