Kertesz A, Martinez-Lage P, Davidson W, Munoz D G
Department of Clinical Neurological Sciences, St. Joseph's Hospital, University of Western Ontario, London, Canada.
Neurology. 2000 Nov 14;55(9):1368-75. doi: 10.1212/wnl.55.9.1368.
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.
Corticobasal degeneration has been mainly described as a movement disorder, but cognitive impairment is also increasingly noted.
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.
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.
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."
为以下假说提供证据，即皮质基底节变性综合征（CBDs）与原发性进行性失语和额颞叶痴呆有显著重叠，且CBDs是皮克复合体的一部分。
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受到关注。
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研究。29例患者在运动障碍和认知神经科门诊接受前瞻性观察；其中5例进行了尸检。另外6例符合CBDs临床标准的尸检病例通过回顾性病历审查纳入研究。
所有15例出现运动障碍的患者在发病后不久或数年后出现行为、认知或语言缺陷。出现认知问题（n = 20）、进行性失语（n = 13）或额颞叶痴呆（n = 7）的患者随后出现运动障碍。11例尸检病例患有CBD或其他形式的皮克复合体。
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重叠。认识到这种重叠将有助于诊断，并避免将CBD视为“异质性”疾病。
