Higuchi Y, Iwaki T, Tateishi J
Department of Neuropathology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Neuropathol Appl Neurobiol. 1995 Jun;21(3):246-54. doi: 10.1111/j.1365-2990.1995.tb01056.x.
Progressive supranuclear palsy is neuropathologically characterized by neuronal degeneration of the basal ganglia, brain stem, and cerebellum. In addition, cortical neuronal degeneration associated with neurofibrillary tangles formation has been identified over wide areas of the brain in patients with progressive supranuclear palsy. We studied the distribution of alpha B-crystallin-positive degenerating neurons in cases with progressive supranuclear palsy, and compared them with those in Pick's disease. Alzheimer's disease, senile dementia of Alzheimer type, and normal aged individuals. A large number of alpha B-crystallin-positive neurons was found in the cerebral cortices of four out of nine patients with progressive supranuclear palsy. In particular, alpha B-crystallin-positive ballooned neurons were frequently observed in deep cortical pyramidal cell layers of the limbic and paralimbic systems in these diseases. The involvement of the limbic and paralimbic systems may thus contribute to personality changes as well as to memory and cognitive impairment in some patients with progressive supranuclear palsy.
进行性核上性麻痹在神经病理学上的特征是基底神经节、脑干和小脑的神经元变性。此外，在进行性核上性麻痹患者的大脑广泛区域已发现与神经原纤维缠结形成相关的皮质神经元变性。我们研究了进行性核上性麻痹病例中αB-晶状体蛋白阳性变性神经元的分布，并将其与匹克病、阿尔茨海默病、阿尔茨海默型老年痴呆症以及正常老年人的情况进行了比较。在9例进行性核上性麻痹患者中的4例的大脑皮质中发现了大量αB-晶状体蛋白阳性神经元。特别是，在这些疾病的边缘和边缘旁系统的深层皮质锥体细胞层中经常观察到αB-晶状体蛋白阳性的气球样神经元。因此，边缘和边缘旁系统的受累可能导致一些进行性核上性麻痹患者出现人格改变以及记忆和认知障碍。
