Clinical and pathological evidence for a frontal variant of Alzheimer disease
Clinical and pathological evidence for a frontal variant of Alzheimer disease
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DOI：
10.1001/archneur.56.10.1233
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发表时间：
1999-10-01
影响因子：
--
通讯作者：
Cotman, CW
中科院分区：
文献类型：
--
作者：
Johnson, JK;Head, E;Cotman, CW
目的：目的探讨阿尔茨海默病（AD）患者的临床和病理特征，这些患者在额叶功能测试中表现出早期和不成比例的严重损害。我们假设，这些患者将表现出更大程度的神经系统缠结（NFT）或老年斑病理在额叶比患者与典型的AD。设计和结果测量：我们检查了神经心理学概况和老年斑和NFT积累在额叶，内嗅，颞，3例AD患者在痴呆早期表现出不成比例的额叶损伤，结果：与典型AD组相比，额叶AD组在额叶功能测验和韦氏成人智力量表修订版区组设计测验中的成绩均显著下降。在其他测试中未发现显著的组间差异。脑组织样本的分析表明，尽管相当的内嗅，颞叶和顶叶NFT负荷，额叶AD组显示出显着较高的NFT负荷在额叶皮质比典型的AD组。额皮质和内嗅皮质的老年斑块病理学没有区分两组。我们确定了一个病理学确诊的AD患者亚组，这些患者在痴呆早期出现额叶功能不成比例的损害，额叶的NFT病理程度高于预期，提示存在具有独特临床和病理特征的AD额叶变体。
Objective: To evaluate the clinical and pathological features of a subgroup of patients with Alzheimer disease (AD) who exhibited early and disproportionately severe impairments on tests of frontal lobe functioning. We hypothesized that these patients would exhibit a greater degree of either neurofibrillary tangle (NFT) or senile plaque pathology in the frontal lobes than would patients with typical AD.Design and Outcome Measures: We examined the neuropsychological profiles and senile plaque and NFT accumulation in the frontal, entorhinal, temporal, and parietal cortices in 3 patients with AD who exhibited disproportionate frontal impairments during early stages of dementia (frontal AD) and 3 matched patients with typical AD (typical AD).Results: Compared with the typical AD group, the frontal AD group performed significantly worse on 2 tests of frontal lobe functioning and on the Wechsler Adult Intelligence Scale-Revised Block Design test. No significant group differences were found on other tests. Analysis of brain tissue samples demonstrated that, despite comparable entorhinal, temporal, and parietal NFT loads, the frontal AD group showed a significantly higher NFT load in the frontal cortex than the typical AD group. Senile plaque pathology in the frontal and entorhinal cortices did not differentiate the 2 groups.Conclusions: We identified a subgroup of patients with pathologically confirmed AD who presented in the early stages of dementia with disproportionate impairments on tests of frontal lobe functioning and had a greater-than-expected degree of NFT pathology in the frontal lobes, suggesting the existence of a frontal variant of AD that has distinctive clinical and pathological features.