The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia
The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia
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DOI：
10.1212/wnl.55.9.1368
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发表时间：
2000-11-14
期刊：
影响因子：
9.9
通讯作者：
Munoz, DG
中科院分区：
文献类型：
--
作者：
Kertesz, A;Martinez-Lage, P;Munoz, DG
目的：为皮质基底节变性综合征（CBD）与原发性进行性失语和额颞叶痴呆显著重叠，以及CBD是Pick复合体的一部分的假设提供证据。背景：皮质基底节变性主要被描述为一种运动障碍，但认知障碍也越来越多地被注意到。方法：对35例临床诊断为CBDs的患者进行临床、神经心理学和神经影像学随访。在运动障碍和认知神经病学诊所前瞻性地观察了29例患者，其中5例进行了尸检。增加了其他6例符合CBD临床标准的尸检病例，并对记录进行了回顾性审查。结果如下：所有15例运动障碍患者在发病后不久或数年后均出现行为、认知或语言缺陷。表现为认知问题（n = 20）、进行性失语（n = 13）或额颞叶痴呆（n = 7）的患者随后发展为运动障碍。11例尸检病例患有CBD或其他形式的皮克综合症。结论：CBD、额颞叶痴呆和原发性进行性失语之间存在临床重叠。这些临床综合征之间也存在病理学重叠。认识到这种重叠将有助于诊断，避免将CBD视为“异质性”。
Objective: To provide evidence for the hypothesis that the corticobasal degeneration syndrome (CBDs) overlaps significantly with primary progressive aphasia and frontotemporal dementia, and that CBDs is part of the Pick complex. Background: Corticobasal degeneration has been mainly described as a movement disorder, but cognitive impairment is also increasingly noted. Methods: Thirty-five cases of clinically diagnosed CBDs were followed-up with clinical, neuropsychological, and neuroimaging investigations. Twenty-nine patients were seen prospectively in movement disorder and cognitive neurology clinics; five of these came to autopsy. Six other autopsied cases that fulfilled the clinical criteria of CBDs were added with retrospective review of records. Results: All 15 patients presenting with movement disorders developed behavioral, cognitive, or language deficits shortly after onset or after several years. Patients presenting with cognitive problems (n = 20), progressive aphasia (n = 13), or frontotemporal dementia (n = 7) developed the movement disorder subsequently. Eleven cases with autopsy had CBD or other forms of the Pick complex. Conclusions: There is a clinical overlap between CBD, frontotemporal dementia, and primary progressive aphasia. There is also a pathologic overlap between these clinical syndromes. The recognition of this overlap will facilitate the diagnosis and avoid consideration of CBD as 'heterogenous".