Differentiating cognitive impairment due to corticobasal degeneration and Alzheimer disease
Differentiating cognitive impairment due to corticobasal degeneration and Alzheimer disease
复制标题
DOI：
10.1212/wnl.0000000000003770
复制
发表时间：
2017-03-28
期刊：
影响因子：
9.9
通讯作者：
Morris, John C.
中科院分区：
文献类型：
--
作者：
Day, Gregory S.;Lim, Tae Sung;Morris, John C.
目的：探讨皮质基底膜变性(CBD)和阿尔茨海默病(AD)所致认知功能障碍的临床特征。方法：比较17例尸检证实的CBD和16例AD患者的临床特征。结果：CBD和AD所致痴呆患者在主诉表现、评估前症状持续时间的中位数(范围)方面有很大的重叠(CBD=3.0[0~5.0]年，AD=2.5[0~8.0]年；P=0.9)和基线痴呆严重程度的中位数(临床痴呆评分总和：CBD=3.5[0-12.0]，AD=4.25[0.5-9.0]，p=0.49)。随后出现的不对称运动/感觉体征、反射亢进、步态异常、帕金森症、跌倒、尿失禁和眼外运动异常确定为CBD患者，在初次评估的3.1年内(95%可信区间2.9-3.3)，80%的患者具有3种辨别特征。患有CBD的患者表现出疾病严重程度的加速恶化和情景记忆、执行功能和字母流利性的下降。半定量病理评估显示，CBD患者额叶和顶叶有明显的tau病变。59%(10/17)的CBD患者存在AD神经病理改变，但与临床表型、痴呆进展率或痴呆病程无关。结论：CBD可能在其病程早期模拟AD痴呆。区分临床特征的间隔筛查可能会改善患有CBD和明显认知症状的个体的生前诊断。
Objective: To identify clinical features that reliably differentiate individuals with cognitive impairment due to corticobasal degeneration (CBD) and Alzheimer disease (AD).Methods: Clinical features were compared between individuals with autopsy-proven CBD (n =17) and AD (n =16). All individuals presented with prominent cognitive complaints and were evaluated annually with semistructured interviews, detailed neurologic examinations, and neuropsychological testing.Results: Substantial overlap was observed between individuals with dementia due to CBD and AD concerning presenting complaints, median (range) duration of symptoms before assessment (CBD = 3.0 [0-5.0] years, AD = 2.5 [0-8.0] years; p = 0.9 ), and median (range) baseline dementia severity (Clinical Dementia Rating Sum of Boxes: CBD = 3.5 [0-12.0], AD = 4.25 [0.5-9.0], p = 0.49). Subsequent emergence of asymmetric motor/sensory signs, hyperreflexia, gait abnormalities, parkinsonism, falls, urinary incontinence, and extraocular movement abnormalities identified individuals with CBD, with >= 3 discriminating features detected in 80% of individuals within 3.1 years (95% confidence interval 2.9-3.3) of the initial assessment. Individuals with CBD exhibited accelerated worsening of illness severity and declines in episodic memory, executive functioning, and letter fluency. Semiquantitative pathologic assessment revealed prominent tau pathology within the frontal and parietal lobes of CBD cases. Comorbid AD neuropathologic change was present in 59% (10 of 17) of CBD cases but did not associate with the clinical phenotype, rate of dementia progression, or dementia duration.Conclusions: CBD may mimic AD dementia early in its disease course. Interval screening for discriminating clinical features may improve antemortem diagnosis in individuals with CBD and prominent cognitive symptoms.