Hof P R, Vogt B A, Bouras C, Morrison J H
Fishberg Research Center for Neurobiology, Mount Sinai School of Medicine, New York, NY 10029, USA.
Vision Res. 1997 Dec;37(24):3609-25. doi: 10.1016/S0042-6989(96)00240-4.
In recent years, the existence of visual variants of Alzheimer's disease characterized by atypical clinical presentation at onset has been increasingly recognized. In many of these cases post-mortem neuropathological assessment revealed that correlations could be established between clinical symptoms and the distribution of neurodegenerative lesions. We have analyzed a series of Alzheimer's disease patients presenting with prominent visual symptomatology as a cardinal sign of the disease. In these cases, a shift in the distribution of pathological lesions was observed such that the primary visual areas and certain visual association areas within the occipito-parieto-temporal junction and posterior cingulate cortex had very high densities of lesions, whereas the prefrontal cortex had fewer lesions than usually observed in Alzheimer's disease. Previous quantitative analyses have demonstrated that in Alzheimer's disease, primary sensory and motor cortical areas are less damaged than the multimodal association areas of the frontal and temporal lobes, as indicated by the laminar and regional distribution patterns of neurofibrillary tangles and senile plaques. The distribution of pathological lesions in the cerebral cortex of Alzheimer's disease cases with visual symptomatology revealed that specific visual association pathways were disrupted, whereas these particular connections are likely to be affected to a less severe degree in the more common form of Alzheimer's disease. These data suggest that in some cases with visual variants of Alzheimer's disease, the neurological symptomatology may be related to the loss of certain components of the cortical visual pathways, as reflected by the particular distribution of the neuropathological markers of the disease.
近年来，以起病时非典型临床表现为特征的阿尔茨海默病视觉变异型的存在越来越受到认可。在许多这类病例中，尸检神经病理学评估显示，临床症状与神经退行性病变的分布之间可以建立相关性。我们分析了一系列以突出的视觉症状为主要疾病体征的阿尔茨海默病患者。在这些病例中，观察到病理病变分布发生了变化，枕颞顶叶交界处和后扣带回皮质内的初级视觉区和某些视觉联合区病变密度非常高，而前额叶皮质的病变比阿尔茨海默病通常观察到的要少。先前的定量分析表明，在阿尔茨海默病中，初级感觉和运动皮质区的受损程度低于额叶和颞叶的多模式联合区，神经原纤维缠结和老年斑的层状和区域分布模式表明了这一点。有视觉症状的阿尔茨海默病病例大脑皮质中病理病变的分布显示，特定的视觉联合通路被破坏，而在更常见的阿尔茨海默病形式中，这些特定的连接可能受到的影响程度较轻。这些数据表明，在一些阿尔茨海默病视觉变异型病例中，神经症状可能与皮质视觉通路某些成分的丧失有关，这一点在疾病神经病理学标志物的特定分布中得到了体现。
