nach oben

Current Neurology and Neuroscience Reports

Erschienen in:

01.03.2018 | Movement Disorders (S Fox, Section Editor)

Progressive Supranuclear Palsy: an Update

verfasst von: Melissa J. Armstrong

Erschienen in: Current Neurology and Neuroscience Reports | Ausgabe 3/2018

Einloggen, um Zugang zu erhalten

Abstract

Purpose of review

Progressive supranuclear palsy (PSP) is a 4R tau neuropathologic entity. While historically defined by the presence of a vertical supranuclear gaze palsy and falls in the first symptomatic year, clinicopathologic studies identify alternate presenting phenotypes. This article reviews the new PSP diagnostic criteria, diagnostic approaches, and treatment strategies.

Recent findings

The 2017 International Parkinson and Movement Disorder Society PSP criteria outline 14 core clinical features and 4 clinical clues that combine to diagnose one of eight PSP phenotypes with probable, possible, or suggestive certainty. Evidence supports the use of select imaging approaches in the classic PSP-Richardson syndrome phenotype. Recent trials of putative disease-modifying agents showed no benefit.

Summary

The new PSP diagnostic criteria incorporating the range of presenting phenotypes have important implications for diagnosis and research. More work is needed to understand how diagnostic evaluations inform phenotype assessment and identify expected progression. Current treatment is symptomatic, but tau-based therapeutics are in active clinical trials.

Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy. A heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar plasy, nuchal dystonia and dementia. Arch Neurol. 1964;10(4):333–58. https://doi.org/10.1001/archneur.1964.00460160003001.CrossRefPubMed

Litvan I, Hauw JJ, Bartko JJ, Lantos PL, Daniel SE, Horoupian DS, et al. Validity and reliability of the preliminary NINDS neuropathologic criteria for progressive supranuclear palsy and related disorders. Jf Neuropathol Exp Neurol. 1996;55(1):97–105. https://doi.org/10.1097/00005072-199601000-00010.CrossRef

Hauw JJ, Daniel SE, Dickson D, Horoupian DS, Jellinger K, Lantos PL, et al. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology. 1994;44(11):2015–9. https://doi.org/10.1212/WNL.44.11.2015.CrossRefPubMed

•• Höglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, Lang AE, et al. Clinical diagnosis of progressive supranuclear palsy: the Movement Disorder Society criteria. Mov Disord. 2017;32(6):853–64. https://doi.org/10.1002/mds.26987. This publication describes the new PSP clinical diagnostic criteria incorporating the common presenting phenotypes.CrossRefPubMed

Dickson DW, Ahmed Z, Algom AA, Tsuboi Y, Josephas KA. Neuropathology of variants of progressive supranuclear palsy. Curr Opin Neurol. 2010;23(4):394–400. https://doi.org/10.1097/WCO.0b013e32833be924.CrossRefPubMed

• Respondek G, Kurz C, Arzberger T, Compta Y, Englund E, Ferguson LW, et al. Which ante mortem clinical features predict progressive supranuclear palsy pathology? Mov Disord. 2017;32(7):995–1005. https://doi.org/10.1002/mds.27034. This systematic review identifies the phenotypes associated with the pathologic diagnosis of PSP.CrossRefPubMed

Litvan I, Agid Y, Calne D, Campbell G, Dubois B, Duvoisin RC, et al. Clinical research criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome): report of the NINDS-SPSP international workshop. Neurology. 1996;47(1):1–9. https://doi.org/10.1212/WNL.47.1.1.CrossRefPubMed

Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, et al. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Brain. 2005;128(6):1247–58. https://doi.org/10.1093/brain/awh488.CrossRefPubMed

Respondek G, Stamelou M, Kurz C, Ferguson LW, Rajput A, Chiu WZ, et al. The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases. Mov Disord. 2014;29(14):1758–66. https://doi.org/10.1002/mds.26054.CrossRefPubMed

10.

Respondek G, Roeber S, Kretzschmar H, Troakes C, Al-Sarraj S, Gelpi E, et al. 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. Mov Disord. 2013;28(4):504–9. https://doi.org/10.1002/mds.25327.CrossRefPubMed

11.

• Whitwell JL, Höglinger GU, Antonini A, Bordelon Y, Boxer AL, Colosimo C, et al. Radiological biomarkers for diagnosis in PSP: where are we and where do we need to be? Mov Disord. 2017;32(7):955–71. https://doi.org/10.1002/mds.27038. This systematic review addresses current knowledge regarding diagnostic imaging in PSP.CrossRefPubMed

12.

Martin WRW, Hartlein J, Racette BA, Cairns N, Perlmutter JS. Pathologic correlates of supranuclear gaze palsy with parkinsonism. Parkinsonism Relat Disord. 2017;38:68–71. https://doi.org/10.1016/j.parkreldis.2017.02.027.CrossRefPubMed

13.

Lloyd-Smith Sequeira A, Rizzo JR, Rucker JC. Clinical approach to supranuclear brainstem saccadic gaze palsies. Front Neurol. 2017;8:429. https://doi.org/10.3389/fneur.2017.00429.CrossRefPubMedPubMedCentral

14.

Kato N, Arai K, Hattori T. Study of the rostral midbrain atrophy in progressive supranuclear palsy. J Neurol Sci. 2003;210(1-2):57–60. https://doi.org/10.1016/S0022-510X(03)00014-5.CrossRefPubMed

15.

Oba H, Yagishita A, Terada H, Barkovich AJ, Kutomi K, Yamauchi T, et al. New and reliable MRI diagnosis for progressive supranuclear palsy. Neurology. 2005;64(12):2050–5. https://doi.org/10.1212/01.WNL.0000165960.04422.D0.CrossRefPubMed

16.

Adachi M, Kawanami T, Ohshima H, Sugai Y, Hosoya T. Morning glory sign: a particular MR finding in progressive supranuclear palsy. Magn Reson Med Sci. 2004;3(3):125–32. https://doi.org/10.2463/mrms.3.125.CrossRefPubMed

17.

Massey LA, Micallef C, Paviour DC, O’Sullivan SS, Ling H, Williams DR, et al. Conventional magnetic resonance imaging in confirmed progressive supranuclear palsy and multiple system atrophy. Mov Disord. 2012;27(14):1754–62. https://doi.org/10.1002/mds.24968.CrossRefPubMed

18.

Adachi M, Kawanami T, Ohshima F. The “morning glory sign” should be evaluated using thinly sliced axial images. Magn Reson Med Sci. 2007;6(1):59–60. https://doi.org/10.2463/mrms.6.59.CrossRefPubMed

19.

Mori H, Aoki S, Ohtomo K. The “morning glory sign” may lead to false impression according to slice angle. Magn Reson Med Sci. 2007;6(3):183–4; author reply 185. https://doi.org/10.2463/mrms.6.183.CrossRefPubMed

20.

Gröschel K, Kastrup A, Litvan I, Schulz JB. Penguins and hummingbirds: midbrain atrophy in progressive supranuclear palsy. Neurology. 2006;66(6):949–50. https://doi.org/10.1212/01.wnl.0000203342.77115.bf.CrossRefPubMed

21.

Quattrone A, Nicoletti G, Messina D, Fera F, Condino F, Pugliese P, et al. MR imaging index for differentiation of progressive supranuclear palsy from Parkinson disease and the Parkinson variant of multiple system atrophy. Radiology. 2008;246(1):214–21. https://doi.org/10.1148/radiol.2453061703.CrossRefPubMed

22.

Höglinger GU, Schöpe J, Stamelou M, Kassubek J, Del Ser T, Boxer AL, et al. Longitudinal magnetic resonance imaging in progressive supranuclear palsy: a new combined score for clinical trials. Mov Disord. 2017;32(6):842–52. https://doi.org/10.1002/mds.26973.CrossRefPubMedPubMedCentral

23.

Marquié M, Normandin MD, Meltzer AC, Siao Tick Chong M, Andrea NV, Antón-Fernández A, et al. Pathological correlations of [F-18]-AV-1451 imaging in non-alzheimer tauopathies. Ann Neurol. 2017;81(1):117–28. https://doi.org/10.1002/ana.24844.CrossRefPubMedPubMedCentral

24.

Passamonti L, Vázquez Rodríguez P, Hong YT, Allinson KS, Williamson D, Borchert RJ, et al. 18F-AV-1451 positron emission tomography in Alzheimer’s disease and progressive supranuclear palsy. Brain. 2017;140:781–91. https://doi.org/10.1093/brain/aww340.PubMedPubMedCentral

25.

Smith R, Schöll M, Honer M, Nilsson CF, Englund E, Hansson O. Tau neuropathology correlates with FDG-PET, but not AV-1451-PET, in progressive supranuclear palsy. Acta Neuropathol. 2017;133(1):149–51. https://doi.org/10.1007/s00401-016-1650-1.CrossRefPubMed

26.

Ishiki A, Harada R, Okamura N, Tomita N, Rowe CC, Villemagne VL. Tau imaging with [18 F]THK-5351 in progressive supranuclear palsy. Eur J Neurol. 2017;24(1):130–6. https://doi.org/10.1111/ene.13164.CrossRefPubMed

27.

Whitwell JL, Lowe VJ, Tosakulwong N, Weigand SD, Senjem ML, Schwarz CG, et al. [18 F]AV-1451 tau positron emission tomography in progressive supranuclear palsy. Mov Disord. 2017;32(1):124–33. https://doi.org/10.1002/mds.26834.CrossRefPubMed

28.

Coakeley S, Cho SS, Koshimori Y, Rusjan P, Harris M, Ghadery C, et al. Positron emission tomography imaging of tau pathology in progressive supranuclear palsy. J Cereb Blood Flow Metab. 2017;37(9):3150–60. https://doi.org/10.1177/0271678X16683695.CrossRefPubMed

29.

Cho H, Choi JY, Hwang MS, Lee SH, Ryu YH, Lee MS, et al. Subcortical 18 F-AV-1451 binding patterns in progressive supranuclear palsy. Mov Disord. 2017;32(1):134–40. https://doi.org/10.1002/mds.26844.CrossRefPubMed

30.

Schonhaut DR, McMillan CT, Spina S, Dickerson BC, Siderowf A, Devous MD Sr, et al. 18 F-flortaucipir tau positron emission tomography distinguishes established progressive supranuclear palsy from controls and Parkinson disease: a multicenter study. Ann Neurol. 2017;82(4):622–34. https://doi.org/10.1002/ana.25060.CrossRefPubMed

31.

Perez-Soriano A, Stoessl AJ. Tau imaging in progressive supranuclear palsy. Mov Disord. 2017;32(1):91–3. https://doi.org/10.1002/mds.26851.CrossRefPubMed

32.

Magdalinou NK, Paterson RW, Schott JM, Fox NC, Mummery C, Blennow K, et al. A panel of nine cerebrospinal fluid biomarkers may identify patients with atypical parkinsonian syndromes. J Neurol Neurosurg Psychiatry. 2015;86(11):1240–7. https://doi.org/10.1136/jnnp-2014-309562.CrossRefPubMedPubMedCentral

33.

Wagshal D, Sankaranarayanan S, Guss V, Hall T, Berisha F, Lobach I, et al. Divergent CSF tau alterations in two common tauopathies: Alzheimer’s disease and progressive supranuclear palsy. J Neurol Neurosurg Psychiatry. 2015;86(3):244–50. https://doi.org/10.1136/jnnp-2014-308004.CrossRefPubMed

34.

Hall S, Öhrfelt A, Constantinescu R, Andreasson U, Surova Y, Bostrom F, et al. Accuracy of a panel of 5 cerebrospinal fluid biomarkers in the differential diagnosis of patients with dementia and/or parkinsonian disorders. Arch Neurol. 2012;69(11):1445–52. https://doi.org/10.1001/archneurol.2012.1654.CrossRefPubMed

35.

Scherling CS, Hall T, Berisha F, Klepac K, Karydas A, Coppola G, et al. Cerebrospinal fluid neurofilament concentration reflects disease severity in frontotemporal degeneration. Ann Neurol. 2014;75(1):116–26. https://doi.org/10.1002/ana.24052.CrossRefPubMedPubMedCentral

36.

Boxer AL, Lang AE, Grossman M, Knopman DS, Miller BL, Schneider LS, et al. Davunetide in patients with progressive supranuclear palsy: a randomised, double-blind, placebo-controlled phase 2/3 trial. Lancet Neurol. 2014;13(7):676–85. https://doi.org/10.1016/S1474-4422(14)70088-2.CrossRefPubMedPubMedCentral

37.

Bacioglu M, Maia LF, Preische O, Schelle J, Apel A, Kaeser SA, et al. Neurofilament light chain in blood and CSF as marker of disease progression in mouse models and in neurodegenerative diseases. Neuron. 2016;91(2):494–6. https://doi.org/10.1016/j.neuron.2016.07.007.CrossRefPubMed

38.

Boman A, Svensson S, Boxer A, Rojas JC, Seeley WW, Karydas A, et al. Distinct lysosomal network protein profiles in parkinsonian syndrome cerebrospinal fluid. J Parkinsons Dis. 2016;6(2):307–15. https://doi.org/10.3233/JPD-150759.CrossRefPubMedPubMedCentral

39.

Hansson O, Janelidze S, Hall S, Magdalinou N, Lees AJ, Andreasson U, et al. Blood-based NfL: a biomarker for differential diagnosis of parkinsonian disorder. Neurology. 2017;88(10):930–7. https://doi.org/10.1212/WNL.0000000000003680.CrossRefPubMedPubMedCentral

40.

Rojas JC, Karydas A, Bang J, Tsai RM, Blennow K, Liman V, et al. Plasma neurofilament light chain predicts progression in progressive supranuclear palsy. Ann Clin Transl Neurol. 2016;3(3):216–25. https://doi.org/10.1002/acn3.290.CrossRefPubMedPubMedCentral

41.

Höglinger GU, Melhem NM, Dickson DW, Sleiman PM, Wang LS, Klei L, et al. Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy. Nat Genet. 2011;43(7):699–705. https://doi.org/10.1038/ng.859.CrossRefPubMedPubMedCentral

42.

Kouri N, Ross OA, Dombroski B, Younkin CS, Serie DJ, Soto-Ortolaza A, et al. Genome-wide association study of corticobasal degeneration identifies risk variants shared with progressive supranuclear palsy. Nat Commun. 2015;6(1):7247. https://doi.org/10.1038/ncomms8247.CrossRefPubMedPubMedCentral

43.

Williams DR, Holton JL, Strand K, Revesz T, Lees AJ. Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy. Mov Disord. 2007;22(15):2235–41. https://doi.org/10.1002/mds.21698.CrossRefPubMed

44.

Compta Y, Valldeoriola F, Tolosa E, Rey MJ, Martí MJ, Valls-Solé J. Long lasting pure freezing of gait preceding progressive supranuclear palsy: a clinicopathological study. Mov Disord. 2007;22(13):1954–8. https://doi.org/10.1002/mds.21612.CrossRefPubMed

45.

Facheris MF, Maniak S, Scaravilli F, Schüle B, Klein C, Pramstaller PP. Pure akinesia as initial presentation of PSP: a clinicopathological study. Parkinsonism Relat Disord. 2008;14(6):517–9. https://doi.org/10.1016/j.parkreldis.2007.11.004.CrossRefPubMed

46.

Glasmacher SA, Leigh PN, Saha RA. Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry. 2017;88(5):402–11. https://doi.org/10.1136/jnnp-2016-314956.CrossRefPubMed

47.

Litvan I, Mangone CA, McKee A, Verny M, Parsa A, Jellinger K, et al. Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study. J Neurol Neurosurg Psychiatry. 1996;60(6):615–20. https://doi.org/10.1136/jnnp.60.6.615.CrossRefPubMedPubMedCentral

48.

Moscovich M, Boschetti G, Moro A, Teive HAG, Hassan A, Munhoz RP. Death certificate data and causes of death in patients with parkinsonism. Parkinsonism Relat Disord. 2017;41:99–103. https://doi.org/10.1016/j.parkreldis.2017.05.022.CrossRefPubMed

49.

Stamelou M, Höglinger G. A review of treatment options for progressive supranuclear palsy. CNS Drugs. 2016;30(7):629–36. https://doi.org/10.1007/s40263-016-0347-2.CrossRefPubMed

50.

Golbe LI, Ohman-Strickland PA. A clinical rating scale for progressive supranuclear palsy. Brain. 2007;30:1552–65.CrossRef

51.

Clerici I, Ferrazzoli D, Maestri R, Bossio F, Zivi I, Canesi M, et al. Rehabilitation in progressive supranuclear palsy: effectiveness of two multidisciplinary treatments. PLoS One. 2017;12(2):e0170927. https://doi.org/10.1371/journal.pone.0170927.CrossRefPubMedPubMedCentral

52.

Sale P, Castiglioni D, De Pandis MF, Torti M, Dall'armi V, Radicati FG, et al. The Lee Silverman Voice Treatment (LSVT ®) speech therapy in progressive supranuclear palsy. Eur J Phys Rehabil Med. 2015;51(5):569–74.PubMed

53.

Scelzo E, Lozano AM, Hamani C, Poon YY, Aldakheel A, Zadikoff C, et al. Peduncolopontine nucleus stimulation in progressive supranuclear palsy: a randomised trial. J Neurol Neurosurg Psychiatry. 2017;88(7):613–6. https://doi.org/10.1136/jnnp-2016-315192.CrossRefPubMed

54.

Bensimon G, Ludolph A, Agid Y, Vidailhet M, Payan C, Leigh PN, et al. Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: the NNIPPS study. Brain. 2009;132(1):156–71. https://doi.org/10.1093/brain/awn291.CrossRefPubMed

55.

Tolosa E, Litvan I, Höglinger GU, Burn D, Lees A, Andrés MV, et al. A phase 2 trial of the GSK-3 inhibitor tideglusib in progressive supranuclear palsy. Mov Disord. 2014;29(4):470–8. https://doi.org/10.1002/mds.25824.CrossRefPubMed

56.

Apetauerova D, Scala SA, Hamill RW, Simon DK, Pathak S, Ruthazer R, et al. CoQ10 in progressive supranuclear palsy: a randomized, placebo-controlled, double-blind trial. Neurol Neuroimmunol Neuroinflamm. 2016;3(5):e266. https://doi.org/10.1212/NXI.0000000000000266.CrossRefPubMedPubMedCentral

57.

Leclair-Visonneau L, Rouaud T, Debilly B, Durif F, Houeto JL, Kreisler A, et al. Randomized placebo-controlled trial of sodium valproate in progressive supranuclear palsy. Clin Neurol Neurosurg. 2016;146:35–9. https://doi.org/10.1016/j.clineuro.2016.04.021.CrossRefPubMed

58.

Nuebling G, Hensler M, Paul S, Zwergal A, Crispin A, Lorenzl S. PROSPERA: a randomized, controlled trial evaluating rasagiline in progressive supranuclear palsy. J Neurol. 2016;263(8):1565–74. https://doi.org/10.1007/s00415-016-8169-1.CrossRefPubMed

59.

Wiblin L, Lee M, Burn D. Palliative care and its emerging role in multiple system atrophy and progressive supranuclear palsy. Parkinsonism Relat Disord. 2017;34:7–14. https://doi.org/10.1016/j.parkreldis.2016.10.013.CrossRefPubMed

Titel: Progressive Supranuclear Palsy: an Update
verfasst von: Melissa J. Armstrong
Publikationsdatum: 01.03.2018
Verlag: Springer US
Erschienen in: Current Neurology and Neuroscience Reports / Ausgabe 3/2018
Print ISSN: 1528-4042
Elektronische ISSN: 1534-6293
DOI: https://doi.org/10.1007/s11910-018-0819-5

Leitlinien kompakt für die Neurologie

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Neu im Fachgebiet Neurologie

Sozialer Aufstieg verringert Demenzgefahr

24.05.2024 Demenz Nachrichten

Ein hohes soziales Niveau ist mit die beste Versicherung gegen eine Demenz. Noch geringer ist das Demenzrisiko für Menschen, die sozial aufsteigen: Sie gewinnen fast zwei demenzfreie Lebensjahre. Umgekehrt steigt die Demenzgefahr beim sozialen Abstieg.

Hirnblutung unter DOAK und VKA ähnlich bedrohlich

17.05.2024 Direkte orale Antikoagulanzien Nachrichten

Kommt es zu einer nichttraumatischen Hirnblutung, spielt es keine große Rolle, ob die Betroffenen zuvor direkt wirksame orale Antikoagulanzien oder Marcumar bekommen haben: Die Prognose ist ähnlich schlecht.

Was nützt die Kraniektomie bei schwerer tiefer Hirnblutung?

17.05.2024 Hirnblutung Nachrichten

Eine Studie zum Nutzen der druckentlastenden Kraniektomie nach schwerer tiefer supratentorieller Hirnblutung deutet einen Nutzen der Operation an. Für überlebende Patienten ist das dennoch nur eine bedingt gute Nachricht.

Thrombektomie auch bei großen Infarkten von Vorteil

16.05.2024 Ischämischer Schlaganfall Nachrichten

Auch ein sehr ausgedehnter ischämischer Schlaganfall scheint an sich kein Grund zu sein, von einer mechanischen Thrombektomie abzusehen. Dafür spricht die LASTE-Studie, an der Patienten und Patientinnen mit einem ASPECTS von maximal 5 beteiligt waren.

Update Neurologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Live-Webinar "Urologie und Sexualmedizin in der Praxis"

Springer Medizin

Abstract

Purpose of review

Recent findings

Summary

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 3/2018

Liquid Biopsy in Primary Brain Tumors: Looking for Stardust!

The Neuropsychological Consequences of Armed Conflicts and Torture

Neurological Complications of Acute and Chronic Otitis Media