Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, University College London, London, UK ; Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, University College London, London, UK ; Sara Koe PSP Research Centre, Institute of Neurology, University College London, London, UK.
J Mov Disord. 2016 Jan;9(1):3-13. doi: 10.14802/jmd.15060. Epub 2016 Jan 25.
Sixty years ago, Steele, Richardson and Olszewski designated progressive supranuclear palsy (PSP) as a new clinicopathological entity in their seminal paper. Since then, in addition to the classic Richardson's syndrome (RS), different clinical phenotypic presentations have been linked with this four-repeat tauopathy. The clinical heterogeneity is associated with variability of regional distribution and severity of abnormal tau accumulation and neuronal loss. In PSP subtypes, the presence of certain clinical pointers may be useful for antemortem prediction of the underlying PSP-tau pathology. Midbrain atrophy on conventional MRI correlates with the clinical phenotype of RS but is not predictive of PSP pathology. Cerebrospinal fluid biomarkers and tau ligand positron emission tomography are promising biomarkers of PSP. A multidisciplinary approach to meet the patients' complex needs is the current core treatment strategy for this devastating disorder.
六十年前，Steele、Richardson 和 Olszewski 在他们的开创性论文中将进行性核上性麻痹（PSP）指定为一种新的临床病理实体。从那时起，除了经典的 Richardson 综合征（RS）外，这种四重复 Tau 病还与不同的临床表型表现相关。临床异质性与异常 Tau 积累和神经元丧失的区域分布和严重程度的可变性相关。在 PSP 亚型中，某些临床特征的存在可能有助于对潜在 PSP-Tau 病理学进行生前预测。常规 MRI 上的中脑萎缩与 RS 的临床表型相关，但不能预测 PSP 病理学。脑脊液生物标志物和 Tau 配体正电子发射断层扫描是 PSP 的有前途的生物标志物。多学科方法满足患者的复杂需求是目前治疗这种破坏性疾病的核心策略。
