Tsuboi Yoshio, Josephs Keith A, Boeve Bradley F, Litvan Irene, Caselli Richard J, Caviness John N, Uitti Ryan J, Bott Allen D, Dickson Dennis W
Department of Neurology, Fukoka University, Fukoka, Japan.
Mov Disord. 2005 Aug;20(8):982-8. doi: 10.1002/mds.20478.
The objective of this study is to better define the pathological characteristics of pathologically proven progressive supranuclear palsy (PSP) presenting with the corticobasal syndrome (CBS). PSP is characterized by early falls, vertical supranuclear ophthalmoplegia, and axial rigidity, whereas asymmetric limb features, including rigidity, bradykinesia, apraxia, alien limb phenomena, and cortical sensory loss are characteristic of CBS. We investigated clinicopathological characteristics of 5 cases of PSP that presented with CBS (CBS-PSP). Comprehensive pathological analysis was undertaken to determine the presence of concomitant pathological processes as well as quantitative tau burden in cortical regions of CBS-PSP, compared with 8 typical PSP cases (Typ-PSP). The clinical features in the CBS-PSP cases included asymmetrical features, apraxia, alien limb phenomena, and progressive aphasia. All cases had Parkinsonism, and vertical supranuclear ophthalmoplegia was noted in all but 1 case of CBS-PSP. Secondary neuropathological diagnoses included argyrophilic grain disease (AGD) in 1 of the 8 cases of Typ-PSP, whereas Alzheimer's disease (AD), Lewy body disease, AGD, and vascular disease was found in 3 cases of CBS-PSP. Image analysis of cortical tau burden performed in 8 Typ-PSP and 3 CBS-PSP cases revealed a significant increased tau burden in mid-frontal and inferior-parietal cortices in the CBS-PSP cases. This study demonstrates that when PSP presents as CBS, it is most likely due to either a concurrent cortical pathology from a secondary process such as AD or from the primary pathology of PSP extending into cortical areas that are primarily and commonly affected in CBD.
本研究的目的是更好地界定经病理证实的、表现为皮质基底节综合征（CBS）的进行性核上性麻痹（PSP）的病理特征。PSP的特点是早期跌倒、垂直性核上性眼肌麻痹和轴性强直，而CBS的特征是不对称的肢体表现，包括强直、运动迟缓、失用症、异己肢体现象和皮质感觉丧失。我们调查了5例表现为CBS的PSP（CBS-PSP）的临床病理特征。进行了全面的病理分析，以确定CBS-PSP皮质区域伴随病理过程的存在以及定量tau负荷，并与8例典型PSP病例（Typ-PSP）进行比较。CBS-PSP病例的临床特征包括不对称表现、失用症、异己肢体现象和进行性失语。所有病例均有帕金森综合征，除1例CBS-PSP外，其余病例均有垂直性核上性眼肌麻痹。继发性神经病理学诊断包括8例Typ-PSP中有1例为嗜银颗粒病（AGD），而3例CBS-PSP中发现有阿尔茨海默病（AD）、路易体病、AGD和血管病。对8例Typ-PSP和3例CBS-PSP病例进行的皮质tau负荷图像分析显示，CBS-PSP病例中额中回和顶下皮质的tau负荷显著增加。本研究表明，当PSP表现为CBS时，最可能的原因是继发过程（如AD）并发的皮质病理，或PSP的原发性病理延伸至CBD中主要且常见受累的皮质区域。
