进行性核上性麻痹是一组较罕见的神经系统变性疾病.其病因及发病机制尚不明确.目前关于进行性核上性麻痹的早期诊断及预后评估,尚无确切的生物学标记物.本文介绍进行性核上性麻痹的病理及病理解剖、诊断标准、亚型谱及临床表现、影像学、量表评价、治疗方法及康复进展、并发症防治及危险因素等.患者应基于多学科团队管理,包括障碍的康复及姑息治疗、教育及支持,以提高患者及照料者的生活质量.%Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease. The etiology and pathogenesis are still unclear. Nowadays there is a lack of an effective molecular marker which can effectively assist the early diagnosis and prognosis of this disease. This article reviewed the pathological anatomy, diagnostic criteria, subtypes and clinical manifestations, imaging studies, scales evaluation, treat-ment and rehabilitation, complications and risk factors of PSP. Patients with PSP should be managed by a multidisciplinary team. The man-agement of PSP should include the rehabilitation of disorder and palliative treatment, as well as support and education, to improve the quali-ty of life for patients and their caregivers.
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