Höglinger Günter U, Respondek Gesine, Stamelou Maria, Kurz Carolin, Josephs Keith A, Lang Anthony E, Mollenhauer Brit, Müller Ulrich, Nilsson Christer, Whitwell Jennifer L, Arzberger Thomas, Englund Elisabet, Gelpi Ellen, Giese Armin, Irwin David J, Meissner Wassilios G, Pantelyat Alexander, Rajput Alex, van Swieten John C, Troakes Claire, Antonini Angelo, Bhatia Kailash P, Bordelon Yvette, Compta Yaroslau, Corvol Jean-Christophe, Colosimo Carlo, Dickson Dennis W, Dodel Richard, Ferguson Leslie, Grossman Murray, Kassubek Jan, Krismer Florian, Levin Johannes, Lorenzl Stefan, Morris Huw R, Nestor Peter, Oertel Wolfgang H, Poewe Werner, Rabinovici Gil, Rowe James B, Schellenberg Gerard D, Seppi Klaus, van Eimeren Thilo, Wenning Gregor K, Boxer Adam L, Golbe Lawrence I, Litvan Irene
Department of Neurology, Technische Universität München, Munich, Germany.
German Center for Neurodegenerative Diseases (DZNE), Munich, Germany.
Mov Disord. 2017 Jun;32(6):853-864. doi: 10.1002/mds.26987. Epub 2017 May 3.
PSP is a neuropathologically defined disease entity. Clinical diagnostic criteria, published in 1996 by the National Institute of Neurological Disorders and Stroke/Society for PSP, have excellent specificity, but their sensitivity is limited for variant PSP syndromes with presentations other than Richardson's syndrome.
We aimed to provide an evidence- and consensus-based revision of the clinical diagnostic criteria for PSP.
We searched the PubMed, Cochrane, Medline, and PSYCInfo databases for articles published in English since 1996, using postmortem diagnosis or highly specific clinical criteria as the diagnostic standard. Second, we generated retrospective standardized clinical data from patients with autopsy-confirmed PSP and control diseases. On this basis, diagnostic criteria were drafted, optimized in two modified Delphi evaluations, submitted to structured discussions with consensus procedures during a 2-day meeting, and refined in three further Delphi rounds.
Defined clinical, imaging, laboratory, and genetic findings serve as mandatory basic features, mandatory exclusion criteria, or context-dependent exclusion criteria. We identified four functional domains (ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction) as clinical predictors of PSP. Within each of these domains, we propose three clinical features that contribute different levels of diagnostic certainty. Specific combinations of these features define the diagnostic criteria, stratified by three degrees of diagnostic certainty (probable PSP, possible PSP, and suggestive of PSP). Clinical clues and imaging findings represent supportive features.
Here, we present new criteria aimed to optimize early, sensitive, and specific clinical diagnosis of PSP on the basis of currently available evidence. © 2017 International Parkinson and Movement Disorder Society.
进行性核上性麻痹（PSP）是一种经神经病理学定义的疾病实体。美国国立神经疾病与中风研究所/PSP协会于1996年发布的临床诊断标准具有出色的特异性，但对于除理查森综合征之外其他表现形式的PSP变异综合征，其敏感性有限。
我们旨在对PSP的临床诊断标准进行基于证据和共识的修订。
我们检索了自1996年以来以英文发表的文章的PubMed、Cochrane、Medline和PSYCInfo数据库，将尸检诊断或高度特异性的临床标准用作诊断标准。其次，我们从尸检确诊的PSP患者和对照疾病患者中生成回顾性标准化临床数据。在此基础上，起草诊断标准，在两次改进的德尔菲评估中进行优化，在为期2天的会议期间提交至采用共识程序的结构化讨论，并在另外三轮德尔菲评估中进行完善。
明确的临床、影像学、实验室和遗传学发现用作强制性基本特征、强制性排除标准或依情况而定的排除标准。我们确定了四个功能领域（眼球运动功能障碍、姿势不稳、运动不能和认知功能障碍）作为PSP的临床预测指标。在这些领域中的每一个领域内，我们提出了三种临床特征，它们对诊断确定性的贡献程度不同。这些特征的特定组合定义了诊断标准，并按诊断确定性的三个程度（可能的PSP、可能的PSP和提示PSP）进行分层。临床线索和影像学发现为支持性特征。
在此，我们提出新的标准，旨在基于现有证据优化PSP的早期、敏感且特异的临床诊断。©2017国际帕金森病和运动障碍协会。
