Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan.
Mov Disord. 2014 Feb;29(2):238-44. doi: 10.1002/mds.25746. Epub 2013 Nov 20.
The aim of this study was to investigate corticobasal syndrome with respect to underlying pathologies, the ability of current clinical criteria to detect early stages of disease, and symptoms and signs predicting background pathologies. We retrospectively analyzed the clinicopathological findings from patients with corticobasal syndrome. We also analyzed whether those findings fulfilled the diagnostic criteria for corticobasal degeneration (CBD). Finally, we investigated characteristic clinical features that are specific to each background pathology. Of 10 consecutive autopsied patients who had corticobasal syndrome (mean age ± standard deviation, 67.9 ± 9.3 years; male:female ratio, 6:4), three had corticobasal degeneration pathology, three had progressive supranuclear palsy, three had Alzheimer's disease, and one had atypical four-repeat tauopathy. Nine patients fulfilled Mayo criteria, and all 10 patients fulfilled modified Cambridge criteria at the later stage, but only two patients fulfilled either clinical criteria within 2 years of disease onset. Five patients fulfilled the clinical criteria for possible CBD (p-CBD), and one patient fulfilled the clinical research criteria for probable sporadic CBD (cr-CBD) at the later stage. Only two patients fulfilled the criteria for either p-CBD or cr-CBD within 2 years of disease onset. Although we could not find any predictive characteristic clinical features that were specific to CBD pathology, only patients with progressive supranuclear palsy developed apraxia of eyelid opening and cerebellar ataxia. Myoclonus and memory impairment, especially if they appear at an early stage of the disease, may predict Alzheimer's disease pathology. Sensitivity of the available clinical criteria for corticobasal syndrome was poor within 2 years of disease onset.
这项研究的目的是探讨皮质基底节综合征（corticobasal syndrome，CBS）的潜在病理学基础、当前临床标准检测疾病早期阶段的能力，以及预测潜在病理学的症状和体征。我们回顾性分析了皮质基底节综合征患者的临床病理发现。我们还分析了这些发现是否符合皮质基底节变性（corticobasal degeneration，CBD）的诊断标准。最后，我们研究了每种潜在病理学特有的特征性临床特征。10 例连续尸检皮质基底节综合征患者（平均年龄±标准差，67.9±9.3 岁；男：女比例，6:4）中，3 例为皮质基底节变性病理学，3 例为进行性核上性麻痹，3 例为阿尔茨海默病，1 例为非典型四重复tau 病。9 例患者符合 Mayo 标准，所有 10 例患者在后期均符合改良剑桥标准，但只有 2 例患者在发病后 2 年内符合任何临床标准。5 例患者符合可能 CBD（p-CBD）的临床标准，1 例患者在后期符合可能散发性 CBD（cr-CBD）的临床研究标准。只有 2 例患者在发病后 2 年内符合 p-CBD 或 cr-CBD 的标准。尽管我们没有发现任何特定于 CBD 病理学的预测性特征性临床特征，但只有进行性核上性麻痹患者出现睑裂张开性失用和小脑共济失调。疾病早期出现的肌阵挛和记忆障碍可能预示着阿尔茨海默病病理学。发病后 2 年内，现有皮质基底节综合征临床标准的敏感性较差。
