Paulus W, Selim M
Ludwig-Boltzmann-Institut für Klinische Neurobiologie, Wien, Austria.
Acta Neuropathol. 1990;81(1):89-94. doi: 10.1007/BF00662643.
A man, aged 58 years, suffered from progressive dementia, parkinsonism, and gaze paralysis for 30 months. Autopsy revealed severe degeneration of the substantia nigra, numerous swollen chromatolytic neurons within the cerebral cortex, scattered basal neurofibrillary tangles, and gliosis of the cerebral white matter and basal ganglia. Unusual globular inclusions positive for tau protein were detected within neurons of the upper cortical layers. Although the pathological findings were comparable with corticonigral degeneration with neuronal achromasia, several clinical and pathological features characteristic for progressive supranuclear palsy, progressive subcortical gliosis, and Pick's disease in this and the nine previously reported cases hampered the unequivocal nosological placement.
一名58岁男性，患有进行性痴呆、帕金森症和凝视麻痹30个月。尸检显示黑质严重变性，大脑皮质内有许多肿胀的染色质溶解神经元，散在的基底神经原纤维缠结，以及脑白质和基底神经节的胶质增生。在上皮层神经元内检测到tau蛋白阳性的异常球状包涵体。尽管病理结果与伴有神经元色素脱失的皮质黑质变性相当，但该病例以及之前报道的9例病例中具有的一些进行性核上性麻痹、进行性皮质下胶质增生和匹克病的临床和病理特征，妨碍了明确的疾病分类定位。
