Department of Neurosurgery, Frenchay Hospital, Bristol, UK.
Neurol Sci. 2011 Feb;32(1):159-63. doi: 10.1007/s10072-010-0386-2. Epub 2010 Aug 25.
Idiopathic Parkinson's disease (PD) and progressive supranuclear palsy (PSP) are distinct clinicopathological entities characterized by α-synuclein and tau pathology, respectively. They have occasionally been reported to co-exist in the same patient. We describe a rare case of a 73-year-old Caucasian woman diagnosed as idiopathic PD 5 years before her death yet at autopsy had not only PD, but also PSP. Although this patient fulfilled clinical criteria for idiopathic PD and did not have supranuclear ophthalmoplegia, she had several atypical features, including early postural instability with falls, early dysphagia, and a relatively rapid course. In conclusion, this case and a literature review highlight the co-existence of synuclein and tau pathology in the same patient and demonstrate that multiple diagnoses may exist in patients presenting with parkinsonism. The clinical heterogeneity seen in parkinsonism may reflect the occurrence of combined pathology.
特发性帕金森病 (PD) 和进行性核上性麻痹 (PSP) 是两种不同的临床病理实体,分别以α-突触核蛋白和 tau 病理学为特征。它们偶尔会在同一患者中同时存在。我们描述了一个罕见的病例,一名 73 岁的白人女性,在她去世前 5 年被诊断为特发性 PD,但尸检不仅发现了 PD,还发现了 PSP。尽管该患者符合特发性 PD 的临床标准,且没有核上性眼肌麻痹,但她有几个非典型特征,包括早期姿势不稳伴跌倒、早期吞咽困难和相对较快的病程。总之,这个病例和文献复习强调了同一患者中存在突触核蛋白和 tau 病理学的共存,并表明在出现帕金森症的患者中可能存在多种诊断。帕金森症的临床异质性可能反映了合并病理的发生。