Akashi T, Arima K, Maruyama N, Ando S, Inose T
National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry, Tokyo, Japan.
Clin Neuropathol. 1989 Jul-Aug;8(4):195-9.
A 60-year-old woman with a history of hypertension and chronic headache initially presented with irritative personality change and mild but steadily progressive dementia and oral tendency, left-sided hemiplegia, intense nuchal stiffness, and swallowing difficulty in the later stage. She died of bronchopneumonia at the age of 76. The brain showed marked loss of nerve cells with gliosis in the cerebral cortex and fibrillary gliosis in the white matter in addition to the typical pathological findings of progressive supranuclear palsy (PSP): extensive subcortical neurofibrillary tangles (NFTs) and loss of nerve cells with gliosis accentuated in the globus pallidus, Luys body and substantia nigra. In many case reports on PSP, the cerebral cortex is described as normal or within normal limits [Jellinger 1971, Steele et al. 1964], and to our knowledge, there is no reported case of severe cortical atrophy as seen in this case. The differential diagnosis of this case is also discussed.
一名60岁女性，有高血压和慢性头痛病史，最初表现为刺激性人格改变、轻度但逐渐进展的痴呆和口部倾向，后期出现左侧偏瘫、严重颈项强直和吞咽困难。她于76岁死于支气管肺炎。除了进行性核上性麻痹（PSP）的典型病理表现外，大脑还显示出大脑皮质神经细胞明显丢失伴胶质细胞增生，白质出现纤维性胶质细胞增生：广泛的皮质下神经原纤维缠结（NFTs）以及苍白球、路易体和黑质神经细胞丢失伴胶质细胞增生加重。在许多关于PSP的病例报告中，大脑皮质被描述为正常或在正常范围内[耶林格，1971年；斯蒂尔等人，1964年]，据我们所知，没有报告过像该病例中出现的严重皮质萎缩情况。本文还讨论了该病例的鉴别诊断。
