The cognitive profiles of CADASIL and sporadic small vessel disease
The cognitive profiles of CADASIL and sporadic small vessel disease
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DOI：
10.1212/01.wnl.0000216270.02610.7e
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发表时间：
2006-05-23
期刊：
影响因子：
9.9
通讯作者：
Markus, H. S.
中科院分区：
文献类型：
--
作者：
Charlton, R. A.;Morris, R. G.;Markus, H. S.
背景：血管性痴呆治疗试验的解释被共存的阿尔茨海默病（AD）病理所混淆。发病较年轻的遗传性疾病大脑常染色体显性动脉病伴皮层下梗死和脑白质病（CADASIL）提供了一种纯血管性痴呆模型，其中不太可能出现这种混淆。为了验证CADASIL作为模型的使用，重要的是要证明它会导致类似的认知障碍。方法：对CADASIL患者（n = 34，其中14例卒中）、伴有腔隙性卒中并合并白质变的散发性小血管疾病（SVD）患者（n = 54）和健康对照（n = 25）进行相同的神经心理学评估。结果：两种疾病的神经心理损害模式相似，早期表现为明显的执行功能障碍。CADASIL和SVD患者在Trails切换测试（CADASIL p = 0.006; SVD p < 0.001）和语言流畅性测试（CADASIL p = 0.015; SVD p = 0.004）上的表现较对照组差。SVD组在即时记忆（p = 0.050）和延迟记忆（p = 0.049）上也表现较差。当只有CADASIL合并卒中的患者被纳入与SVD受试者的分析时，所有这些受试者都有卒中，观察到非常相似的认知概况。唯一的区别是语言流畅性，CADASIL受试者表现较差（p = 0.044）。结论：大脑常染色体显性动脉病变合并皮质下梗死和脑白质病（CADASIL）和小血管病患者表现出相似的认知缺陷模式。这表明CADASIL提供了一种与散发性小血管疾病相关的纯血管性痴呆模型。
Background: Interpretation of treatment trials in vascular dementia is confounded by the presence of coexistent Alzheimer disease (AD) pathology. The younger onset genetic disease cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) offers a model of pure vascular dementia, in which such confounding is unlikely. To validate CADASIL's use as a model it is important to show it results in a similar cognitive impairment. Methods: The same neuropsychological assessment was administered to patients with CADASIL (n = 34, 14 of whom had had stroke), sporadic small vessel disease (SVD) presenting with lacunar stroke and having confluent leukoaraiosis (n = 54), and healthy controls (n = 25). Results: A similar pattern of neuropsychological impairment was seen in the two diseases, with prominent early executive dysfunction. Patients with CADASIL and SVD performed worse than controls on Trails switching test (CADASIL p = 0.006; SVD p < 0.001), and on verbal fluency test (CADASIL p = 0.015; SVD p = 0.004). The SVD group also performed worse on immediate ( p = 0.050) and delayed (p = 0.049) memory. When only patients with CADASIL with stroke were included in analysis with SVD subjects, all of whom had had stroke, a very similar cognitive profile was seen. The only difference was on verbal fluency, where CADASIL subjects performed worse (p = 0.044). Conclusion: Patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and small vessel disease show a similar pattern of cognitive deficits. This suggests that CADASIL provides a model of pure vascular dementia relevant for sporadic small vessel disease vascular dementia.