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Video-tutorial for the Movement Disorder Society criteria for progressive supranuclear palsy

Open AccessPublished:September 22, 2020DOI:https://doi.org/10.1016/j.parkreldis.2020.06.030

      Highlights

      • Comprehensive video-based tutorial for the correct application of the MDS-PSP criteria.
      • Displays clinical features associated with PSP pathology.
      • Promotes an early and accurate clinical diagnosis of PSP.

      Abstract

      Background

      The International Parkinson and Movement Disorder Society-endorsed Progressive Supranuclear Palsy Study Group published clinical diagnostic criteria for progressive supranuclear palsy in 2017, aiming to optimize early, sensitive and specific diagnosis.

      Objective

      To assist physicians in the application of these criteria, we developed a video-based tutorial in which all core clinical features and clinical clues are depicted and explained.

      Methods

      Patients provided written informed consent to the publication of their videos. High-quality videos along with essential descriptions were collected by the study group members. Most educational videos were selected in a structured consensus process.

      Results

      We provide 68 videos of all core clinical features and clinical clues defined by the diagnostic criteria, along with instructive descriptions of the depicted patients, examination techniques and clinical findings.

      Conclusions

      This comprehensive video-based tutorial will support physicians in the application of the diagnostic criteria of progressive supranuclear palsy.

      Keywords

      1. Introduction

      Progressive supranuclear palsy (PSP) is a neuropathologically defined disease entity [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ]. The pathological hallmarks of PSP are intracellular aggregations of hyperphosphorylated four-repeat tau protein in the form of neurofibrillary tangles, tau deposits in astrocytes (“astrocytic tufts”) and oligodendroglia (“coiled bodies”) [
      • Rösler T.W.
      • Tayaranian Marvian A.
      • Brendel M.
      • Nykänen N.P.
      • Höllerhage M.
      • Schwarz S.C.
      • Hopfner F.
      • Koeglsperger T.
      • Respondek G.
      • Schweyer K.
      • Levin J.
      • Villemagne V.L.
      • Barthel H.
      • Sabri O.
      • Müller U.
      • Meissner W.G.
      • Kovacs G.G.
      • Höglinger G.U.
      Four-repeat tauopathies.
      ].
      The Movement Disorder Society (MDS) criteria for the clinical diagnosis of PSP (MDS-PSP criteria) were published in 2017 [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ]. Diagnostic challenges addressed in these criteria include the heterogeneity of clinical features associated with PSP, clinical overlaps with and distinctions from other diseases, and the limited additional value of diagnostic biomarkers [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ].
      The previously used Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy diagnostic criteria [
      • Litvan I.
      • Agid Y.
      • Calne D.
      • Campbell G.
      • Dubois B.
      • Duvoisin R.C
      • Goetz C.G.
      • Golbe L.I.
      • Grafman J.
      • Growdon J.H.
      • Hallett M.
      • Jankovic J.
      • Quinn N.P.
      • Tolosa E.
      • Zee D.S.
      Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome).
      ] had lower sensitivity, because they relied exclusively on the identification of patients with Richardson's syndrome [
      • Respondek G.
      • Roeber S.
      • Kretzschmar H.
      • Troakes C.
      • Al-Sarraj S.
      • Gelpi E.
      • Gaig C.
      • Chiu W.Z.
      • van Swieten J.C.
      • Oertel W.H.
      • Höglinger G.U.
      Accuracy of the national institute for neurological disorders and stroke/society for progressive supranuclear palsy and neuroprotection and natural history in Parkinson plus syndromes criteria for the diagnosis of progressive supranuclear palsy.
      ,
      • Respondek G.
      • Stamelou M.
      • Kurz C.
      • Ferguson L.W.
      • Rajput A.
      • Chiu W.Z.
      • van Sweeten J.C.
      • Troakes C.
      • Al-Sarraj S.
      • Gelpi E.
      • Gaig C.
      • Tolosa E.
      • Oertel W.H.
      • Giese A.
      • Roeber S.
      • Arzberger T.
      • Wagenpfeil S.
      • Höglinger G.U.
      Movement Disorder Society-endorsed PSP Study Group, the phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases.
      ]. The MDS-PSP criteria have improved sensitivity by taking into account the broader spectrum of clinical presentations of PSP [
      • Ali F.
      • Martin P.R.
      • Bortha H.
      • Ahlskog J.E.
      • Bower J.H.
      • Masumoto J.Y.
      • Maraganore D.
      • Hassan A.
      • Eggers S.
      • Boeve B.F.
      • Knopman D.S
      • Drubach D.
      • Petersen R.C.
      • Dunkley E.D.
      • van Gerpen J.
      • Uitti R.
      • Whitwell J.L.
      • Dickson D.W.
      • Josephs K.A.
      Sensitivity and specificity of diagnostic criteria for progressive supranuclear palsy.
      ,
      • Gazzina S.
      • Respondek G.
      • Compta Y.
      • Allinson K.S.J.
      • Spillantini M.G.
      • Molina-Porcel L.
      • Guasp-Verdaguer M.
      • Moftakhar S.
      • Reich S.G.
      • Hall D.
      • Litvan I.
      • Höglinger G.U.
      • Movement Disorder Society-endorsed Psp Study Group
      • Rowe J.B.
      Neuropathological Validation of the MDS-PSP Criteria with PSP and Other Frontotemporal Lobar Degeneration.
      ]. Consequently, their application is more complex compared to prior criteria.
      The MDS-PSP criteria are based on ‘mandatory inclusion criteria’, ‘mandatory exclusion criteria’, ‘context-dependent exclusion criteria’, ‘core clinical features’, and ‘supportive features’ (including ‘clinical clues’ and ‘imaging findings’) [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ]. Core clinical features (Table 1) are defined in four functional domains, i.e. ocular motor dysfunction, postural instability, akinesia, and cognitive dysfunction, labelled O, P, A, or C, respectively. In each domain, three levels of core clinical features are defined, labelled 1, 2 or 3, respectively, with lower numbers indicating a higher diagnostic certainty to a diagnosis of PSP [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ]. Operationalized definitions for the core clinical features and clinical clues were developed based on a systematic analysis of the literature, analysis of a large clinico-pathological cohort and expert consensus [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ]. Specific combinations of core clinical features and clinical clues indicate the degree of diagnostic certainty and the predominance types of PSP patient [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ].
      Table 1MDS-PSP-criteria – ‘core clinical features’ and supportive ‘clinical clues’.
      Core clinical features
      Domain: Ocular motor dysfunction
      O1Vertical supranuclear gaze palsy
      O2Slow velocity of vertical saccades
      O3Subtle ocular motor dysfunction, i.e.
      O3-1frequent macro square wave jerks, or
      O3-2“eyelid opening apraxia”
      Domain: Postural instability
      P1Repeated unprovoked falls within 3 years
      P2Tendency to fall on the pull-test within 3 years
      P3More than 2 steps backward on the pull-test within 3 year
      Domain: Akinesia
      A1Progressive gait freezing within 3 years
      A2Parkinsonism, akinetic-rigid, predominantly axial and levodopa-resistant
      A3Parkinsonism, with tremor and/or asymmetric and/or levodopa-responsive
      Domain: Cognitive dysfunction
      C1Speech/language disorder, i.e. at least 1 of the following features:

      C1-1Nonfluent agrammatic variant of primary progressive aphasia (nfaPPA), or
      C1-2progressive apraxia of speech (AOS)
      C2Frontal cognitive/behavioral presentation, i.e. at least 3 of the following features:
      C2-1Apathy
      C2-2Bradyphrenia
      C2-3Dysexecutive syndrome (e.g., pathological Luria sequence)
      C2-4Reduced phonemic verbal fluency (e.g., reduced “D, F, A or S” words per minute)
      C2-5Impulsivity, disinhibition, or perseveration (e.g., socially inappropriate behaviors, overstuffing the mouth when eating, motor recklessness, applause sign, palilalia, echolalia)
      C3Corticobasal syndrome, i.e. at least 1 sign each from the following 2 groups
      C3-1Cortical signs
      C3-1aOrobuccal or limb apraxia
      C3-1bCortical sensory deficit
      C3-1cAlien limb phenomena (more than simple levitation)
      C2-2Movement disorders signs
      C3-2aLimb rigidity
      C3-2bLimb akinesia
      C3-2cLimb myoclonus
      C3-2dLimb dystonia
      2. Clinical clues
      CC1Levodopa-resistance
      CC2Hypokinetic, spastic dysarthria
      CC3Dysphagia
      CC4Photophobia
      Legend: For detailed operationalized definitions of the ‘core clinical features’ and supportive ‘clinical clues’ see the original MDS-PSP criteria publication [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ]. In the definition of C3, “limb dystonia” was unintentionally omitted in the original publication [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ] and shall be considered as 4th qualifying movement disorders sign for corticobasal syndrome.
      To facilitate the application of the MDS-PSP criteria and to promote best clinical practice in PSP diagnosis, the MDS-endorsed PSP Study Group developed this video-based tutorial, in which the examination techniques and characteristic pathological signs for the diagnostic features are illustrated.

      2. Methods

      The MDS-endorsed PSP Study Group formed a steering committee (G.U·H., G.R. and V·I.) to coordinate the development of the video tutorial. The steering committee established guidelines for videotaping of the core clinical features and clinical clues, as defined in the MDS-PSP criteria. All videos had to be taken in high-definition; the area of interest had to be bright with minimal shadows; the background had to be clean and non-distracting. Existing videos were identified from the study group members’ archives, and new videos were recorded. All patients provided written informed consent for publication of their videos. Videos meeting these guidelines were uploaded to a secure, password-protected online platform. Information about age, clinical phenotype and diagnostic certainty of the patients were assembled. In a first review, the steering committee evaluated quality and content of all uploaded videos. Underrepresented clinical features were identified and the study group members were encouraged to provide additional videos of these specific features. In a second review, the steering committee proposed an initial selection for the tutorial, based on technical and educational quality, eliminating videos not meeting the pre-specified quality criteria. All study group members were then asked to provide written feedback on this initial selection. All comments were taken into consideration and the final selection of videos was made through Delphi consensus. Videos were cut to present the most instructive content in a compact time frame using OpenShot Video Editor. Title captions at the beginning of each video were introduced using MLTmelt and ImageMagic.

      3. Results

      From over 100 submitted videos, 68 representative videos were selected to depict the core clinical features' and supportive ‘clinical clues’ (Table 1), as defined and operationalized in the MDS-PSP criteria [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ].
      These videos and the corresponding legends are found in the online supporting material.
      We did not provide videos for the features apathy (part of the frontal cognitive/behavioral presentation C2) and limb rigidity (part of corticobasal syndrome C3), because these features turned out to be difficult to visualize in a video.
      For certain clinical features more than one representative video was selected to show their variable quality and severity.

      4. Discussion

      An early and accurate clinical diagnosis of PSP is increasingly important to provide patients with reliable information about prognosis, treatment options, and access to clinical trials [
      • Rösler T.W.
      • Tayaranian Marvian A.
      • Brendel M.
      • Nykänen N.P.
      • Höllerhage M.
      • Schwarz S.C.
      • Hopfner F.
      • Koeglsperger T.
      • Respondek G.
      • Schweyer K.
      • Levin J.
      • Villemagne V.L.
      • Barthel H.
      • Sabri O.
      • Müller U.
      • Meissner W.G.
      • Kovacs G.G.
      • Höglinger G.U.
      Four-repeat tauopathies.
      ]. The MDS-PSP criteria take into account early clinical features as well as the broad clinical spectrum of PSP, creating the basis for an earlier and more sensitive, but still specific diagnosis of PSP [
      • Ali F.
      • Martin P.R.
      • Bortha H.
      • Ahlskog J.E.
      • Bower J.H.
      • Masumoto J.Y.
      • Maraganore D.
      • Hassan A.
      • Eggers S.
      • Boeve B.F.
      • Knopman D.S
      • Drubach D.
      • Petersen R.C.
      • Dunkley E.D.
      • van Gerpen J.
      • Uitti R.
      • Whitwell J.L.
      • Dickson D.W.
      • Josephs K.A.
      Sensitivity and specificity of diagnostic criteria for progressive supranuclear palsy.
      ,
      • Gazzina S.
      • Respondek G.
      • Compta Y.
      • Allinson K.S.J.
      • Spillantini M.G.
      • Molina-Porcel L.
      • Guasp-Verdaguer M.
      • Moftakhar S.
      • Reich S.G.
      • Hall D.
      • Litvan I.
      • Höglinger G.U.
      • Movement Disorder Society-endorsed Psp Study Group
      • Rowe J.B.
      Neuropathological Validation of the MDS-PSP Criteria with PSP and Other Frontotemporal Lobar Degeneration.
      ]. Therefore, it is now most important to increase awareness for the MDS-PSP criteria and the clinical spectrum of PSP among physicians.
      For this purpose, we established this comprehensive video-based tutorial for the correct application of the MDS-PSP criteria. This tutorial aims to reach physicians and clinician scientists from all levels of experience, to assist in the standardized application of the MDS-PSP criteria by displaying the clinical features associated with PSP pathology.
      This video tutorial does not display ‘mandatory inclusion criteria’, ‘mandatory exclusion criteria’, ‘context-dependent exclusion criteria’, and supportive ‘imaging findings’, which are also essential components of the MDS-PSP criteria. Therefore, we wish to emphasise the need to refer to the original publication of the MDS-PSP criteria [
      • Höglinger G.U.
      • Respondek G.
      • Stamelou M.
      • Josephs K.A.
      • Lang A.E.
      • Mollenhauer B.
      • Müller 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.
      Movement Disorder Society-endorsed PSP Study Group, Clinical diagnosis of progressive supranuclear palsy: the movement disorder society criteria, Mov.
      ] and to the more recently published guidelines for a correct application of the diagnostic criteria [
      • Grimm M.J.
      • Respondek G.
      • Stamelou M.
      • Arzberger T.
      • Ferguson L.
      • Gelpi E.
      • Giese A.
      • Grossman M.
      • Irwin D.J.
      • Pantelyat A.
      • Rajput A.
      • Roeber S.
      • van Swieten J.C.
      • Troakes C.
      • Antonini A.
      • Bhatia K.P.
      • Colosimo C.
      • van Eimeren T.
      • Kassubek J.
      • Levin J.
      • Meissner W.G.
      • Nilsson C.
      • Oertel W.H.
      • Piot I.
      • Poewe W.
      • Wenning G.K.
      • Boxer A.
      • Golbe L.I.
      • Josephs K.A.
      • Litvan I.
      • Morris H.R.
      • Whitwell J.L.
      • Compta Y.
      • Corvol J.C.
      • Lang A.E.
      • Rowe J.B.
      • Höglinger G.U.
      Movement Disorder Society-endorsed PSP Study Group, How to apply the movement disorder society criteria for diagnosis of progressive supranuclear palsy.
      ].
      We expect this video-based tutorial not only to facilitate the application of the MDS-PSP criteria, but moreover to increase awareness among clinicians of the broad spectrum of clinical presentations of PSP, fostering an early, sensitive and still specific clinical diagnosis.

      Author roles

      1. Project: A. Conception, B. Organization, C. Execution;
      2. Manuscript Preparation: A. Writing the First Draft, B. Review and Critique.
      Vassilena Iankova: 1B, 1C, 2A
      Gesine Respondek: 1A, 1B, 1C, 2B;
      Gerard Saranza: 1C, 2B;
      Cèlia Painous: 1C, 2B;
      Ana Cámara: 1C, 2B;
      Yaroslau Compta: 1C, 2B;
      Ikuko Aiba: 1C, 2B;
      Bettina Balint: 1C, 2B;
      Nikolaos Giagkou: 1C, 2B;
      Keith A. Josephs: 1C, 2B;
      Mika Otsuki: 1C;
      Lawrence I. Golbe: 1C, 2B;
      Kailash P Bhatia: 1C, 2B;
      Maria Stamelou: 1C, 2B;
      Anthony E Lang: 1C, 2B;
      Günter U. Höglinger: 1A, 1B, 1C, 2B;

      Financial disclosures of all authors for the preceding 12 months

      Vassilena Iankova has nothing to disclose. Gesine Respondek has nothing to disclose. Gerard Saranza has nothing to disclose. Cèlia Painous has received support from Boston and has a competitive Río Hortega predoctoral grant from the Spanish Ministry of Health .
      Ana Cámara has received funding or support from Abbvie and Medtronic as well as from the H2020 lPI043760 grant. She has a competitive PERIS grant from the Department of Health of the Generalitat de Catalunya ( SLT008/18/00191 ).
      Yaroslau Compta has served as a consultant for Abbvie and Zambon, and has received honoraria for scientific presentations from Abbvie, Alter, Bial, Medtronic, Merz, Teva, UCB, and Zambon. He is currently an associate editor of Parkinsonism & Related Disorders (Elsevier). He has competitive grants from Instituto de Salud Carlos III (ISCIII) / Spanish Ministry of Health ( PI17/00096 ) and the European Commission H2020 program ( lPI043760 ). YC’s center receives support from the CERCA program of Generalitat de Catalunya and the Maria de Maeztu program of the Spanish Ministry of Education , and is part of the European Reference Network for Rare Neurological Diseases (ERN-RND) .
      Ikuko Aiba serves as a consultant for Abbvie and Biogen.Bettina Balint is supported by the Robert Bosch Foundation .Nikolaos Giagkou has nothing to disclose.
      Keith A. Josephs is funded by the National Institute of Health .Mika Otsuki has nothing to disclose.
      Lawrence I. Golbe receives research support from Biogen and AbbVie and serves as a consultant for CurePSP, Biogen, AbbVie, UCB, Asceneuron, GLG, Piramal, Pinteon, NeuroTrax and Novartis .
      Kailash P Bhatia holds research grants from NIHR RfPB , MRC Wellcome Strategic grant (Ref. no.: WT089698 ) and PD UK (Ref. no.: G-1009 ), and has received honoraria/financial support to speak/attend meetings from GSK , Boehringer-Ingelheim, Ipsen , Merz, Sun Pharma , Allergan , Teva Lundbeck and Orion pharmaceutical companies . KB receives royalties from Oxford University press and a stipend for MDCP editorship.
      Maria Stamelou serves as assistant editor in Movement Disorders Journal, has received travel and speaker honoraria from Biogen , UCB , Abbvie and Specifar, MDS and EAN , royalties from Oxford and Cambridge University Press, has served in as advisory board to Biogen and Specifar and receives research support from PPMI.
      Anthony E Lang: Consultancies: Abbvie, Acorda, AFFiRis, Biogen, Bristol Myers Squibb, Intracellular, Janssen, Jazz, Lilly, Lundbeck, Merck, Ono, Paladin, Roche, Seelos, Syneos, Sun Pharma, Theravance, and Corticobasal Degeneration Solutions; Advisory Boards: Jazz Pharma, PhotoPharmics, Sunovion; Honoraria:Sun Pharma, AbbVie and Sunovion: Grants: Brain Canada, Canadian Institutes of Health Research, Corticobasal Degeneration Solutions, Edmond J Safra Philanthropic Foundation, Michael J. Fox Foundation, the Ontario Brain Institute, Parkinson Foundation, Parkinson Canada, and W. Garfield Weston Foundation; Royalties: Elsevier, Saunders, Wiley-Blackwell, Johns Hopkins Press, and Cambridge University Press.
      Günter U. Höglinger has ongoing research collaborations with Orion, Prothena; serves as a consultant for Abbvie, Alzprotect, Asceneuron, Biogen, Biohaven, Novartis, Sanofi, UCB; received honoraria for scientific presentations from Abbvie, Biogen, Roche, Teva, UCB, Zambon, has received research support from CurePSP , the German Academic Exchange Service (DAAD) , German Parkinson’s Disease Foundation (DPG) , German PSP Association (PSP Gesellschaft) , German Research Foundation (DFG) and the German Ministry of Education and Research (BMBF) , International Parkinson’s Fonds (IPF ); has received institutional support from the German Center for Neurodegenerative Diseases (DZNE) .

      Financial disclosure

      None.

      Funding source for study

      The project was supported by the German Center for Neurodegenerative Diseases e.V. (DZNE) , German PSP Gesellschaft , and the International Parkinson & Movement Disorder Society .

      Declaration of competing interest

      None.

      Acknowledgements

      The authors would like to thank Naomi Matsuda, PT, Yoriko Omote, PT, Hiroshi Nishimura, OT, Kousuke Kobayashi, ST , of the Department of Rehabilitation, National Hospital Organization Higashinagoya National Hospital, Nagoya, Japan and Rina Hashimoto, MD, PhD, Satoko Sakakibara, MD of the Department of Neurology, National Hospital Organization Higashinagoya National Hospital, Nagoya, Japan, for their support.

      Appendix A. Supplementary data

      The following are the Supplementary data to this article:

      Appendix.

      These MDS-endorsed PSP study group members contributed to this work: Anthony E. Lang, Bettina Balint, Gesine Respondek, Günter Höglinger, Ikuko Aiba, Kailash P Bhatia, Keith A Josephs, Lawrence I Golbe, Maria Stamelou, Vassilena Iankova, Yaroslau Compta.

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