Comparative clinico-molecular pathological study of corticobasal degeneration and progressive supranuclear palsy

进行性核上性麻痹（PSP）是一种以帕金森综合征和痴呆为特征的神经退行性疾病。但老年PSP患者并不一定同时表现为帕金森综合征和痴呆。我们回顾性研究了51例尸检证实的PSP病例的临床资料，以阐明其临床病理多样性。51例PSP患者中21例有典型的符合PSP的神经系统症状，20例无明显的帕金森综合征，能正常生活。20例无明显帕金森综合征的PSP患者中，14例仅表现痴呆（平均年龄85.5±7.5），6例无明显帕金森综合征或痴呆（平均年龄90.5±9.8）。无帕金森综合征的PSP患者的平均年龄明显高于有帕金森综合征的PSP患者。但PSP患者的脑重量在有无帕金森综合征之间无显著性差异。2例PSP患者表现为小脑共济失调，但病变的分布及严重程度与其他PSP患者相同。部分老年PSP患者，虽然数量很少，但仅表现为痴呆。

Progressive supranuclear palsy(PSP) is a neurodegenerative disorder that is characterized by parkinsonism and dementia. However, the aged patients with PSP did not always show both parkinsonism and dementia. We investigated retrospectively the clinical data of 51 autopsy-proven cases with PSP to clarify their clinicopathological multiplicity. Among 51 patients with PSP,21 cases showed typical neurological symptoms compatible to PSP, but 20 cases did not show apparent parkinsonism and could go about their daily life without any problem. Among 20 PSP patients lacking obvious parkinsonism,14 cases presented only dementia (mean age:85.5±7.5) and six cases did not show obvious parkinsonism nor dementia (mean age:90.5±9.8). The mean age of PSP cases without parkinsonism was significantly higher than that of PSP cases with parkinsonism. But there is no significant difference in the brain weight of PSP patients between those with and without parkinsonism. Only two cases with PSP showed cerebellar ataxia, but the distribution and severity of lesions were same as those of other PSP cases. Parts pf the aged PSP patients, though a few in number, showed only dementia.