摘要
[目的]探讨进行性核上性麻痹的发病机制、病理特点、诊断及治疗。[方法]分析4例进行性核上性麻痹患者的临床资料,并复习相关文献。[结果]4例患者均于55岁后缓慢起病,临床表现为姿势不稳、帕金森综合征、垂直性核上性眼肌麻痹、假性球麻痹和痴呆等症状。进行性核上性麻痹的发病机制尚不明确。氧化应激和线粒体功能失调等可能导致了tau蛋白的沉积。病理表现为皮层及皮层下结构神经细胞丢失,神经纤维缠结和胶质增生。其诊断和神经影像学已经得到发展,但尚无有效治疗措施。[结论]进行性核上性麻痹是一种误诊率很高的疾病,临床医生应予重视。
[Objective] To investigate the pathogenesis, pathology, diagnosis and treatment of progressive supranuclear palsy. [ Methods] The clinical data of four patients with progressive supranuclear palsy were analyzed, and the related literatures of this disease were reviewed. [Results] The onset of the four cases was slowly progressive after 55-year old, characterized by postural instability, Parkinsonism, vertical supranuclear gaze palsy, pseudobulbar palsy and dementia. The pathogenesis of progressive supranuclear palsy was still uncertain. Oxidative damage and mitochondrial dysfunction might contribute to tau deposition. The pathological changes involved with both cortical and subcortical structures of the cerebrum, and manifestated by neuronal loss, spherical neurofibrillary tangles and gliosis. The diagnosis and neuroimage was improved, but there was no effective pharmaceutical intervention. [Conclusion] The disease of progressive supranuclear palsy is commonly misdiagnosed. Clinicians should pay more attention to it.
出处
《现代预防医学》
CAS
北大核心
2007年第5期964-965,968,共3页
Modern Preventive Medicine
关键词
进行性核上性麻痹
神经病理
诊断
Progressive supranuclear palsy
Neuropathology
Diagnosis
