Atypical and typical presentations of Alzheimer's disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases
Atypical and typical presentations of Alzheimer's disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases
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DOI：
10.1093/brain/123.3.484
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发表时间：
2000-03-01
期刊：
影响因子：
14.5
通讯作者：
Hodges, JR
中科院分区：
文献类型：
--
作者：
Galton, CJ;Patterson, K;Hodges, JR
越来越多的人认识到，一些缓慢进行性局灶性皮质综合征可能是阿尔茨海默病的表现特征，但病理学证据很少。这个临床病理系列介绍了我们的经验，病理证实的非典型以及典型的阿尔茨海默氏病的介绍。本文报告并比较了四种类型的失语症：典型类型为初始遗忘综合征（n = 4例）、进行性视功能障碍（n = 1例）、进行性双顶综合征（n = 2例）和进行性失语（n = 6例）。神经心理学和神经影像学检查清楚地反映了这些病例的临床特征，并与病理分布密切相关。值得注意的是，在几个失语症病例中，内侧颞叶结构（海马和/或内嗅皮层）的保留以及在表现为明显视觉空间障碍的病例中，严重的枕顶叶受累。我们的数据表明阿尔茨海默病表现的范围很广。当试图对神经退行性疾病进行早期准确的病前诊断时，识别阿尔茨海默病的非典型表现是重要的。
There has been increasing awareness that some slowly progressive focal cortical syndromes can be the presenting features of Alzheimer's disease, but pathological evidence has been sparse. This clinico-pathological series presents our experience with pathologically proven atypical as well as typical Alzheimer's disease presentations. We report and compare four patterns of presentation: a typical pattern with initial amnesic syndrome (n = 4 cases), progressive visual dysfunction (n = 1), progressive biparietal syndrome (n = 2) and progressive aphasia (n = 6), The aphasic presentations include both fluent and non-fluent aphasic syndromes. The neuropsychological profiles and neuroimaging clearly reflect the presenting clinical features, and show a close relationship to the distribution of pathology in these cases. Of note was the sparing of medial temporal structures (hippocampus and/or entorhinal cortex) in several aphasic cases and the severe occipito-parietal involvement in those with prominent visuospatial disorders at presentation, Our data demonstrate the wide spectrum of Alzheimer's disease presentations. The recognition of atypical presentations of Alzheimer's disease is important when attempting to make an early accurate pre-morbid diagnosis of neurodegenerative disease.