Galton C J, Patterson K, Xuereb J H, Hodges J R
University Neurology Unit, Department of Histopathology, Addenbrooke's Hospital, Cambridge, UK.
Brain. 2000 Mar;123 Pt 3:484-98. doi: 10.1093/brain/123.3.484.
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.
人们越来越意识到，一些缓慢进展的局灶性皮质综合征可能是阿尔茨海默病的首发症状，但病理证据一直很少。本临床病理系列报告了我们在经病理证实的非典型以及典型阿尔茨海默病表现方面的经验。我们报告并比较了四种表现模式：以初始遗忘综合征为典型模式（n = 4例）、进行性视觉功能障碍（n = 1例）、进行性双侧顶叶综合征（n = 2例）和进行性失语（n = 6例）。失语表现包括流利性和非流利性失语综合征。神经心理学特征和神经影像学清楚地反映了所呈现的临床特征，并且在这些病例中显示出与病理分布的密切关系。值得注意的是，在几例失语病例中内侧颞叶结构（海马体和/或内嗅皮质）未受影响，而在那些呈现出明显视觉空间障碍的病例中枕顶叶严重受累。我们的数据证明了阿尔茨海默病表现的广泛谱系。在试图对神经退行性疾病进行早期准确的病前诊断时，认识阿尔茨海默病的非典型表现很重要。
