经尸检证实的皮质基底节变性的认知和运动评估。
Cognitive and motor assessment in autopsy-proven corticobasal degeneration.
作者信息
Murray R, Neumann M, Forman M S, Farmer J, Massimo L, Rice A, Miller B L, Johnson J K, Clark C M, Hurtig H I, Gorno-Tempini M L, Lee V M-Y, Trojanowski J Q, Grossman M
机构信息
Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4283, USA.
出版信息
Neurology. 2007 Apr 17;68(16):1274-83. doi: 10.1212/01.wnl.0000259519.78480.c3.
OBJECTIVE
To investigate the clinical features of autopsy-proven corticobasal degeneration (CBD).
METHODS
We evaluated symptoms, signs, and neuropsychological deficits longitudinally in 15 patients with autopsy-proven CBD and related these observations directly to the neuroanatomic distribution of disease.
RESULTS
At presentation, a specific pattern of cognitive impairment was evident, whereas an extrapyramidal motor abnormality was present in less than half of the patients. Follow-up examination revealed persistent impairment of apraxia and executive functioning, worsening language performance, and preserved memory. The motor disorder emerged and worsened as the condition progressed. Statistical analysis associated cognitive deficits with tau-immunoreactive pathology that is significantly more prominent in frontal and parietal cortices and the basal ganglia than temporal neocortex and the hippocampus.
CONCLUSION
The clinical diagnosis of corticobasal degeneration should depend on a specific pattern of impaired cognition as well as an extrapyramidal motor disorder, reflecting the neuroanatomic distribution of disease in frontal and parietal cortices and the basal ganglia.
目的
研究经尸检证实的皮质基底节变性（CBD）的临床特征。
方法
我们对15例经尸检证实的CBD患者的症状、体征和神经心理学缺陷进行了纵向评估，并将这些观察结果直接与疾病的神经解剖分布相关联。
结果
初诊时，一种特定的认知障碍模式很明显，而不到一半的患者存在锥体外系运动异常。随访检查显示失用症和执行功能持续受损，语言能力恶化，记忆保留。随着病情进展，运动障碍出现并加重。统计分析表明，认知缺陷与tau免疫反应性病理相关，额叶、顶叶皮质和基底神经节中的这种病理比颞叶新皮质和海马体更明显。
结论
皮质基底节变性的临床诊断应取决于特定的认知障碍模式以及锥体外系运动障碍，这反映了额叶、顶叶皮质和基底神经节中疾病的神经解剖分布。
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