The MDS-PSP criteria were generated by the MDS-PSP study group in a three-step approach.
First, we performed a systematic literature review covering the time since publication of the NINDS-SPSP criteria. In brief, the steering committee (G.U.H., M.S., A.L.B., L.I.G., and I.L.) assembled expert working groups for specific questions relevant to the diagnosis of PSP. We searched the PubMed, Cochrane, Medline, and PSYCInfo databases for articles, systematic reviews, and meta-analyses published in English from 1996 to 2015, applying either postmortem diagnosis or the NINDS-SPSP criteria. Study group members were encouraged to add relevant articles to be considered throughout the project period (end of 2016), particularly those published after 2015. The literature was analyzed following the Scottish Intercollegiate Guidelines Network recommendations.37 From N = 5,903 identified articles, N = 462 met the inclusion standards. The literature-based evidence was then summarized by the working groups for imaging and clinical aspects and is published in detail in accompanying papers in this issue of Movement Disorders.38 ,39 Second, we collected the largest autopsy-confirmed case series reported so far for PSP and disease controls (CBD, MSA-P, PD, and FTLD-bvFTD) from nine brain banks with a proven track record of a close collaboration with tertiary clinical referral centers, both with excellent experience in neurodegenerative diseases (Amsterdam, Netherlands; Baltimore, MD; Barcelona, Spain; Bordeaux, France; London, UK; Lund, Sweden; Munich, Germany; Philadelphia, PA; and Saskatchewan, Canada). High-quality original natural history data were available from patients with autopsy-confirmed PSP (N = 206), CBD (N = 54), MSA-P (N = 51), PD (N = 53), and FTLD-bvFTD (N = 73). We extracted demographic data and predefined clinical features (absence/presence/onset) in a standardized manner locally from the clinical records and collected them centrally. These data were used to estimate and stratify the diagnostic value of the clinical items selected from a comprehensive literature review and are reported in detail in an accompanying paper.38 Third, on the basis of the evidence obtained in the first two steps, the steering committee drafted an initial proposal of the criteria, which was distributed to the MDS-PSP study group members. They provided written feedback to the process coordinator (G.U.H.), who incorporated the comments into optimized criteria in two modified Delphi rounds. In March 2016, the group convened for a 2-day consensus meeting in Munich to present and discuss all aspects of the criteria (structure, basic features, exclusion criteria, core functional domains, operationalized clinical features, supportive findings, imaging, biomarkers, and genetics). For each of these items, the data obtained in the first two steps were presented by the subgroup coordinators. Thereafter, the written draft of the criteria was discussed stepwise. Modifications were integrated until the entire group unanimously agreed to the items under discussion. After the meeting, the written document was circulated again and optimized in three further Delphi rounds, in particular, dealing with precise wording, operationalized definition of clinical examination guidelines, and newly evolving aspects, such as tau PET imaging. After final approval, the current manuscript was written (G.U.H.) and circulated to incorporate final modifications.
Here, we present the MDS clinical diagnostic criteria for PSP.
Hoglinger G.U., Respondek G., Stamelou M., Kurz C., Josephs K.A., Lang A.E., Mollenhauer B., Muller U., Nilsson C., Whitwell J.L., Arzberger T., Englund E., Gelpi E., Giese A., Irwin D.J., Meissner W.G., Pantelyat A., Rajput A., van Swieten J.C., Troakes C., Antonini A., Bhatia K.P., Bordelon Y., Compta Y., Corvol J.C., Colosimo C., Dickson D.W., Dodel R., Ferguson L., Grossman M., Kassubek J., Krismer F., Levin J., Lorenzl S., Morris H.R., Nestor P., Oertel W.H., Poewe W., Rabinovici G., Rowe J.B., Schellenberg G.D., Seppi K., van Eimeren T., Wenning G.K., Boxer A.L., Golbe L.I, & Litvan I. (2017). Clinical Diagnosis of Progressive Supranuclear Palsy: The Movement Disorder Society Criteria. Movement disorders : official journal of the Movement Disorder Society, 32(6), 853-864.