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皮质基底节综合征的病理学在生前是否可预测?

Is the pathology of corticobasal syndrome predictable in life?

机构信息

Department of Clinical Neurosciences, University of Cambridge, Addenbrooke's Hospital, UK.

出版信息

Mov Disord. 2009 Aug 15;24(11):1593-9. doi: 10.1002/mds.22558.

Abstract

Corticobasal syndrome (CBS) has been associated with a heterogeneous spectrum of pathologies with an increasing number of reports of Alzheimer's type pathology. There is, however, no means of predicting pathology of CBS in vivo at present. We compared the clinical features of patients presenting with CBS who have either pathologic changes of classic corticobasal degeneration (CBD) or Alzheimer's disease (AD) at post-mortem to identify predictors of the specific pathological processes in life. Twelve patients with CBS were followed prospectively; six had AD and six had classic CBD neuropathology. After review of the presenting clinical features, we identified nine potential predictor variables, compared their frequency in the two groups, and performed a discriminant function analysis. Initial episodic memory complaints and poor performance on the combined orientation-memory subtest of the Addenbrooke's Cognitive Examination (ACE) reliably predicted AD pathology while varying combinations of early frontal-lobe type behavioral symptoms, nonfluent language disturbance, orobuccal apraxia, and utilization behavior predicted CBD pathology ante-mortem. CBS is frequently associated with Alzheimer's disease pathology. Early episodic memory impairment versus early behavioral symptomatology appears to best predict AD or CBD pathology in life.

摘要

皮质基底节综合征(CBS)与多种病理学表现相关,越来越多的报道表明其与阿尔茨海默病(AD)病理学有关。然而,目前尚无法在体内预测 CBS 的病理学改变。我们比较了尸检时表现为 CBS 且具有经典皮质基底节变性(CBD)或 AD 病理学改变的患者的临床特征,以确定在生前预测特定病理过程的指标。12 例 CBS 患者前瞻性随访;6 例有 AD,6 例有经典 CBD 神经病理学改变。在回顾了首发的临床特征后,我们确定了 9 个潜在的预测变量,比较了两组之间的频率,并进行了判别函数分析。首发时的情节记忆主诉和 Addenbrooke 认知测验(ACE)的定向记忆联合测验的表现不佳可靠地预测了 AD 病理学,而早期额叶类型的行为症状、非流利性语言障碍、口面失用症或利用行为的不同组合则预测了 CBD 病理学。CBS 常与 AD 病理学相关。早期情节记忆障碍与早期行为症状相比,似乎能更好地预测生前 AD 或 CBD 的病理学改变。

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