Fédération des Maladies du Système Nerveux, Research and Resource Memory Centre, Pavillon Jean Lhermitte, Hôpital de la Salpêtrière, 47 Boulevard de l'Hôpital, 75013 Paris, France.
Brain. 2011 Jul;134(Pt 7):2036-43. doi: 10.1093/brain/awr130.
While the clinical presentation of posterior cortical atrophy is clearly distinct from typical Alzheimer's disease, neuropathological studies have suggested that most patients with posterior cortical atrophy have Alzheimer's disease with an atypical visual presentation. We analysed in vivo pathophysiological markers of Alzheimer's disease such as cerebrospinal fluid biomarkers and positron emission tomography imaging with ¹¹C-labelled Pittsburgh compound-B in posterior cortical atrophy to determine whether biochemical profile and fibrillar amyloid-β burden topography are associated with the clinical presentation. Nine patients with posterior cortical atrophy and nine with typical Alzheimer's disease individually matched for age, duration and severity of the disease and 10 cognitively normal age-matched controls were included. ¹¹C-labelled Pittsburgh compound-B images were analysed both using volumes of interest and on a voxel-wise basis using statistical parametric mapping, taking into account the individual regional cortical atrophy. Cerebrospinal fluid biomarkers did not differ between posterior cortical atrophy and patients with Alzheimer's disease. Compared with normal controls, both posterior cortical atrophy and Alzheimer's disease groups showed increased ¹¹C-labelled Pittsburgh compound-B uptake. No significant difference was found in regional or global ¹¹C-labelled Pittsburgh compound-B binding between posterior cortical atrophy and Alzheimer's disease groups with both volumes of interest and voxel-wise basis using statistical parametric mapping methods. Our findings demonstrate that cerebrospinal fluid biomarkers and positron emission tomography imaging with ¹¹C-labelled Pittsburgh compound-B may be useful in identifying an atypical visual form of Alzheimer's disease. The similar topography of fibrillar amyloid-β deposition between typical Alzheimer's disease and posterior cortical atrophy groups suggests that, although amyloid-β accumulation plays a critical role in the pathogenesis of Alzheimer's disease, other factors such as neurofibrillary tangles may contribute to the different clinical features observed in posterior cortical atrophy.
虽然后部皮质萎缩的临床特征明显不同于典型的阿尔茨海默病，但神经病理学研究表明，大多数后部皮质萎缩患者的阿尔茨海默病表现为不典型的视觉表现。我们分析了后部皮质萎缩患者的阿尔茨海默病的体内病理生理标志物，如脑脊液生物标志物和正电子发射断层扫描成像与 ¹¹C 标记的匹兹堡化合物-B，以确定生化特征和纤维状淀粉样β负担的分布是否与临床表现相关。纳入了 9 名后部皮质萎缩患者和 9 名年龄、疾病持续时间和严重程度相匹配的典型阿尔茨海默病患者以及 10 名认知正常的年龄匹配对照者。¹¹C 标记的匹兹堡化合物-B 图像使用感兴趣区和基于体素的方法进行分析，同时考虑到个体的区域皮质萎缩。后部皮质萎缩患者和阿尔茨海默病患者的脑脊液生物标志物无差异。与正常对照组相比，后部皮质萎缩组和阿尔茨海默病组的 ¹¹C 标记的匹兹堡化合物-B 摄取均增加。使用基于体素的方法进行统计参数映射分析，无论是使用感兴趣区还是基于体素的方法，在后皮质萎缩组和阿尔茨海默病组之间均未发现区域性或全局 ¹¹C 标记的匹兹堡化合物-B 结合的显著差异。我们的研究结果表明，脑脊液生物标志物和正电子发射断层扫描成像与 ¹¹C 标记的匹兹堡化合物-B 可能有助于识别阿尔茨海默病的不典型视觉形式。典型阿尔茨海默病和后部皮质萎缩组之间纤维状淀粉样β沉积的相似分布表明，尽管淀粉样β的积累在阿尔茨海默病的发病机制中起着关键作用，但其他因素，如神经原纤维缠结，可能导致后部皮质萎缩中观察到的不同临床特征。
