Dept of Cognitive Neurology, University of Western Ontario, St Joseph's Hospital, Ontario, Canada.
J Neurol Sci. 2010 Feb 15;289(1-2):138-43. doi: 10.1016/j.jns.2009.08.036. Epub 2009 Sep 5.
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD), previously described as Parkinsonian syndromes are also cognitive disorders, and biologically related to the frontotemporal dementia or Pick's disease. PSP and CBD overlap clinically, pathologically and genetically, sharing tau haplotypes and mutations. In our series of CBD/PSP patients with cognitive presentation (n=36), primary progressive aphasia (PPA) was particularly common, but behavioral onset occurred also. CBD or PSP as motor presentations developed significant language disorder in 17/19. The underlying pathology is predictably tau positive in these clinical combinations, regardless of the presentation. Other cognitive features of CBDS include apraxia, alien hand and apathy, but often frontal lobe dementia with disinhibition develops also. CBDS also has visuospatial deficit, because of the parietal involvement. PSP was considered the prototype of subcortical dementia, with bradyphrenia, poor recall and executive deficit, but cortical features were recognized to be important also. Language testing and a behavioral inventory should be part of neuropsychological tests to facilitate diagnosis and to quantify the deficit. The clinical, genetic and pathological relationship is strong between CBD /PSP and the aphasic and behavioral components of the Pick complex.
进行性核上性麻痹（PSP）和皮质基底节变性（CBD）以前被描述为帕金森综合征，也是认知障碍，与额颞痴呆或 Pick 病在生物学上有关。PSP 和 CBD 在临床上、病理学上和遗传学上重叠，具有 tau 单倍型和突变。在我们具有认知表现的 CBD/PSP 患者系列中（n=36），原发性进行性失语（PPA）特别常见，但也有行为发作。以运动表现出现的 CBD 或 PSP 在 17/19 例中出现了明显的语言障碍。无论表现如何，这些临床组合中都可预测存在 tau 阳性的潜在病理学。CBDS 的其他认知特征包括失用症、异手和冷漠，但经常也会发展为额叶痴呆伴去抑制。CBDS 也有视空间缺陷，因为涉及顶叶。PSP 被认为是皮质下痴呆的原型，具有智力迟钝、记忆力差和执行功能缺陷，但皮质特征也被认为很重要。语言测试和行为量表应作为神经心理学测试的一部分，以促进诊断并量化缺陷。CBD/PSP 与 Pick 综合征的失语和行为成分在临床、遗传和病理学上关系密切。
