Progressive Supranuclear Palsy & Corticobasal Degeneration

Definition

According to the PSP Association, “PSP is a neurological condition caused by the premature loss of nerve cells in certain parts of the brain. A protein called tau builds up in certain areas of the brain and forms into clumps (neurofibrillary tangles), which are believed to damage the nerve cells.” Please find more information on the PSP Association’s website.

Relevant Guidelines

Revised diagnostic criteria for PSP, Movement Disorders Society-endorsed PSP Study group, International Consensus Meeting in Munich, March 2016.

Consensus Papers

  • Höglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, Lang AE, Mollenhauer B, Müller U, Nilsson C, Whitwell JL, Arzberger T, Englund E, Gelpi E, Giese A, Irwin DJ, Meissner WG, Pantelyat A, Rajput A, van Swieten JC, Troakes C, Antonini A, Bhatia KP, Bordelon Y, Compta Y, Corvol JC, Colosimo C, Dickson DW, Dodel R, Ferguson L, Grossman M, Kassubek J, Krismer F, Levin J, Lorenzl S, Morris HR, Nestor P, Oertel WH, Poewe W, Rabinovici G, Rowe JB, Schellenberg GD, Seppi K, van Eimeren T, Wenning GK, Boxer AL, Golbe LI, Litvan I; Movement Disorder Society-endorsed PSP Study Group. Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria. Mov Disord. 2017;32(6):853-864. doi: 10.1002/mds.26987.
  • Respondek G, Kurz C, Arzberger T, Compta Y, Englund E, Ferguson LW, Gelpi E, Giese A, Irwin DJ, Meissner WG, Nilsson C, Pantelyat A, Rajput A, van Swieten JC, Troakes C, Josephs KA, Lang AE, Mollenhauer B, Müller U, Whitwell JL, Antonini A, Bhatia KP, Bordelon Y, Corvol JC, Colosimo C, Dodel R, Grossman M, Kassubek J, Krismer F, Levin J, Lorenzl S, Morris H, Nestor P, Oertel WH, Rabinovici GD, Rowe JB, van Eimeren T, Wenning GK, Boxer A, Golbe LI, Litvan I, Stamelou M, Höglinger GU; Movement Disorder Society-Endorsed PSP Study Group. Which ante mortem clinical features predict progressive supranuclear palsy pathology? Mov Disord. 2017;32(7):995-1005. doi: 10.1002/mds.27034.
  • Whitwell JL, Höglinger GU Antonini A, Bordelon Y, Boxer AL, Colosimo C, van Eimeren T, Golbe LI, Kassubek J, Kurz C, Litvan I, Pantelyat A, Rabinovici G, Respondek G, Rominger A, Rowe JB, Stamelou M, Josephs KA; Movement Disorder Society-endorsed PSP Study Group. Radiological biomarkers for diagnosis in PSP: Where are we and where do we need to be? Mov Disord. 2017;32(7):955-971. doi: 10.1002/mds.27038.
  • Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, Boxer AL, Dickson DW, Grossman M, Hallett M, Josephs KA, Kertesz A, Lee SE, Miller BL, Reich SG, Riley DE, Tolosa E, Tröster AI, Vidailhet M, Weiner WJ. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496-503. doi: 10.1212/WNL.0b013e31827f0fd1.

Diagnostic Algorithms

  • Höglinger GU, Respondek G, Stamelou M, Kurz C, Josephs KA, Lang AE, Mollenhauer B, Müller U, Nilsson C, Whitwell JL, Arzberger T, Englund E, Gelpi E, Giese A, Irwin DJ, Meissner WG, Pantelyat A, Rajput A, van Swieten JC, Troakes C, Antonini A, Bhatia KP, Bordelon Y, Compta Y, Corvol JC, Colosimo C, Dickson DW, Dodel R, Ferguson L, Grossman M, Kassubek J, Krismer F, Levin J, Lorenzl S, Morris HR, Nestor P, Oertel WH, Poewe W, Rabinovici G, Rowe JB, Schellenberg GD, Seppi K, van Eimeren T, Wenning GK, Boxer AL, Golbe LI, Litvan I; Movement Disorder Society-endorsed PSP Study Group. Clinical diagnosis of progressive supranuclear palsy: The movement disorder society criteria. Mov Disord. 2017;32(6):853-864. doi: 10.1002/mds.26987.
  • Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, Boxer AL, Dickson DW, Grossman M, Hallett M, Josephs KA, Kertesz A, Lee SE, Miller BL, Reich SG, Riley DE, Tolosa E, Tröster AI, Vidailhet M, Weiner WJ. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80(5):496-503. doi: 10.1212/WNL.0b013e31827f0fd1.

Registries

Germany
  • DescribePSP: a prospective cohort study of DZNE clinical centers
  • ProPSP: a prospective cohort study of German and Luxembourg academic hospitals
UK
Europe
  • CBD (European CBD registry)

Scales

  • PSP Rating Scale (PSP-RS): the PSP-RS is a disease-specific scale capturing the diverse clinical dimensions of PSP. It has been validated in several prospective mono- and multicentric studies. It is sensitive to measure change over time with a reproducible and stable progression rate. Golbe LI, Ohman-Strickland PA. A clinical rating scale for progressive supranuclear palsy. Brain 2007;130:1552–65
  • PSP Quality of Live (PSP-QoL): the PSP-QoL is a disease-specific scale capturing diverse aspects affecting the quality of life of PSP patients. It has not been used so far in a multi-centric setting. Schrag A, Selai C, Quinn N, et al. Measuring quality of life in PSP: the PSP-QoL. Neurology 2006;67:39–44
  • PQoL Carers: Pillas M, Selai C, Quinn NP, Lees A, Litvan I, Lang A, Bower J, Burn D, Low P, Schrag A. Development and validation of a carers quality-of-life questionnaire for parkinsonism (PQoL Carers). Qual Life Res. 2016 Jan;25(1):81-8. doi: 10.1007/s11136-015-1071-y.

Networks and Study Groups

Patient Organisations

Recent Review Papers

  • Boxer AL, Yu JT, Golbe LI, Litvan I, Lang AE, Höglinger GU. Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches. Lancet Neurol. 2017;16(7):552-563. doi: 10.1016/S1474-4422(17)30157-6.
  • Ali F, Josephs KA. Corticobasal degeneration: key emerging issues. J Neurol. 2017. doi: 10.1007/s00415-017-8644-3.
  • Lamb R, Rohrer JD, Lees AJ, Morris HR. Progressive Supranuclear Palsy and Corticobasal Degeneration: Pathophysiology and Treatment Options. Curr Treat Options Neurol. 2016 Sep;18(9):42. doi: 10.1007/s11940-016-0422-5.
  • Grijalvo-Perez AM, Litvan I. Corticobasal degeneration. Semin Neurol. 2014;34(2):160-73. doi: 10.1055/s-0034-1381734.