Hof P R, Perl D P, Loerzel A J, Steele J C, Morrison J H
Fishberg Research Center for Neurobiology, Mount Sinai School of Medicine, New York, NY 10029.
Brain Res. 1994 Jul 4;650(1):107-16. doi: 10.1016/0006-8993(94)90212-7.
Amyotrophic lateral sclerosis/parkinsonism-dementia complex is a highly prevalent neurodegenerative disorder among the native Chamorro population of Guam, and is characterized by widespread formation of neurofibrillary tangles. In the present study, the distribution of neurofibrillary tangles was quantitatively assessed in the cerebral cortex of cases presenting with either predominant amyotrophic lateral sclerosis or parkinsonism-dementia symptomatology. Results show that although the regional and laminar lesion distribution is qualitatively similar in both groups, cases with predominant parkinsonism-dementia generally have higher lesion densities than cases with amyotrophic lateral sclerosis. Interestingly, layer II of the entorhinal cortex was affected to the same degree in both conditions. In both groups, the CA1 field of the hippocampus, subiculum, and entorhinal cortex were the most affected areas. In the neocortex, the perirhinal and inferior temporal cortex consistently had higher lesion densities than the frontal, parietal, and cingulate cortex, whereas the visual cortex was practically devoid of lesions. Also, most of the neurofibrillary tangles were located in the supragranular layers of the neocortex, with relatively low densities in the infragranular layers, in both brain groups. Interestingly, the primary motor cortex contained more neurofibrillary tangles in parkinsonism-dementia than in amyotrophic lateral sclerosis cases. It is possible that the differences in regional neurofibrillary tangle densities reflect the variable severity of the dementing process observed between the two groups of patients. Several studies on Alzheimer's disease and related disorders indicate that the regional and laminar cortical localization of neurofibrillary tangles may parallel the degeneration of specific corticocortical projections. The present data suggest that the population of corticocortical projections involved in Guamanian cases differs substantially from that affected in Alzheimer's disease. The differential distribution and densities of the lesions may contribute to the differences in symptomatology and severity of dementia among Alzheimer's disease and Guamanian cases, although these neurodegenerative disorders as well as related illnesses may share certain etiopathogenetic mechanisms.
肌萎缩侧索硬化症/帕金森病-痴呆综合征是关岛查莫罗原住民中一种高度流行的神经退行性疾病，其特征是广泛形成神经原纤维缠结。在本研究中，对以肌萎缩侧索硬化症为主或帕金森病-痴呆症状为主的病例的大脑皮质中神经原纤维缠结的分布进行了定量评估。结果表明，虽然两组的区域和层状病变分布在质量上相似，但以帕金森病-痴呆为主的病例的病变密度通常高于以肌萎缩侧索硬化症为主的病例。有趣的是，内嗅皮质的第II层在两种情况下受到的影响程度相同。在两组中，海马体的CA1区、下托和内嗅皮质是受影响最严重的区域。在新皮质中，嗅周皮质和颞下皮质的病变密度始终高于额叶、顶叶和扣带回皮质，而视觉皮质几乎没有病变。此外，在两个脑组中，大多数神经原纤维缠结位于新皮质的颗粒上层，颗粒下层的密度相对较低。有趣的是，与肌萎缩侧索硬化症病例相比，帕金森病-痴呆病例的初级运动皮质中含有更多的神经原纤维缠结。区域神经原纤维缠结密度的差异可能反映了两组患者中观察到的痴呆过程的不同严重程度。几项关于阿尔茨海默病及相关疾病的研究表明，神经原纤维缠结的区域和层状皮质定位可能与特定皮质-皮质投射的退化平行。目前的数据表明，关岛病例中涉及的皮质-皮质投射群体与阿尔茨海默病中受影响的群体有很大不同。病变的差异分布和密度可能导致阿尔茨海默病和关岛病例在症状学和痴呆严重程度上的差异，尽管这些神经退行性疾病以及相关疾病可能共享某些病因发病机制。
