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进行性核上性麻痹(progressive supranuclearpalsy,PSP)又称Steele-Richardson-Olszewski综合征。该病在美国适龄居民中患病率为1.39人/10万,但实际患病率可能远不止这一数字,因为很多病例在其发病很长一段时间后才明确诊断,而且有许多病例很可能至死亡时仍未明确诊断或误诊为其他疾病,尤其帕金森病。一、病理大体标本可见苍白球、黑质和脑干萎缩,第Ⅲ、Ⅳ脑室及侧脑室扩大;黑质和蓝斑脱色;Brodmann 4区中度萎缩。镜下特征性神经病理改变是基底节和脑干分布大量的神经纤维缠结(neurofibrillary tan-gles,NFT)和线型神经纤维网结构(neuropilthreads),同时伴神经元缺失、星形胶质细胞增生。典型病例NFT分布在苍白球、下丘脑核、纹状体、
Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome. The prevalence of this disease is 1.39 per 100 000 inhabitants of the United States of America, but the actual prevalence may be far more than this figure because many cases are diagnosed only after a long period of their onset and many cases are likely Still not yet diagnosed or misdiagnosed as other diseases at death, especially Parkinson’s disease. First, the gross pathology can be seen globus pallidus, substantia nigra and brain stem atrophy, Ⅲ, Ⅳ ventricles and lateral ventricle enlargement; substantia nigra and blue spot decolorization; Brodmann 4 moderate atrophy. The characteristic neuropathological changes under the microscope are a large number of neurofibrillary tan-gles (NFTs) and linear neuropilthreads distributed in the basal ganglia and brainstem, accompanied by neuronal loss, astrocytes Hyperplasia. Typical cases of NFT distribution in the globus pallidus, hypothalamic nucleus, striatum,