Frontiers in Neurology (Jul 2021)
Best Practices in the Clinical Management of Progressive Supranuclear Palsy and Corticobasal Syndrome: A Consensus Statement of the CurePSP Centers of Care
- Brent Bluett,
- Brent Bluett,
- Alexander Y. Pantelyat,
- Irene Litvan,
- Farwa Ali,
- Diana Apetauerova,
- Danny Bega,
- Lisa Bloom,
- James Bower,
- Adam L. Boxer,
- Marian L. Dale,
- Rohit Dhall,
- Antoine Duquette,
- Hubert H. Fernandez,
- Jori E. Fleisher,
- Murray Grossman,
- Michael Howell,
- Diana R. Kerwin,
- Julie Leegwater-Kim,
- Christiane Lepage,
- Peter Alexander Ljubenkov,
- Martina Mancini,
- Nikolaus R. McFarland,
- Paolo Moretti,
- Erica Myrick,
- Pritika Patel,
- Laura S. Plummer,
- Federico Rodriguez-Porcel,
- Julio Rojas,
- Christos Sidiropoulos,
- Miriam Sklerov,
- Leonard L. Sokol,
- Paul J. Tuite,
- Lawren VandeVrede,
- Jennifer Wilhelm,
- Anne-Marie A. Wills,
- Tao Xie,
- Lawrence I. Golbe
Affiliations
- Brent Bluett
- Neurology, Pacific Central Coast Health Center, Dignity Health, San Luis Obispo, CA, United States
- Brent Bluett
- Neurology, Stanford University, Stanford, CA, United States
- Alexander Y. Pantelyat
- Neurology, The Johns Hopkins Hospital, Johns Hopkins Medicine, Baltimore, MD, United States
- Irene Litvan
- Neurology, University of California, San Diego, San Diego, CA, United States
- Farwa Ali
- Neurology, Mayo Clinic, Rochester, MN, United States
- Diana Apetauerova
- Neurology, Lahey Hospital and Medical Center, Burlington, MA, United States
- Danny Bega
- Ken and Ruth Davee Department of Neurology, Feinberg School of Medicine, Northwestern University, Chicago, IL, United States
- Lisa Bloom
- Neurology, Surgery, University of Chicago, Chicago, IL, United States
- James Bower
- Neurology, Mayo Clinic, Rochester, MN, United States
- Adam L. Boxer
- Neurology, University of California, San Francisco, San Francisco, CA, United States
- Marian L. Dale
- 0Neurology, Oregon Health and Science University, Portland, OR, United States
- Rohit Dhall
- 1Neurology, University of Arkansas for Medical Sciences, Little Rock, AK, United States
- Antoine Duquette
- 2Service de Neurologie, Département de Médecine, Unité de Troubles du Mouvement André-Barbeau, Centre Hospitalier de l'Université de Service de Neurologie, Centre Hospitalier de l'Université de Montréal (CHUM), Montreal, QC, Canada
- Hubert H. Fernandez
- 3Center for Neurological Restoration, Neurological Institute, Cleveland Clinic, Cleveland, OH, United States
- Jori E. Fleisher
- 4Neurological Sciences, Rush Medical College, Rush University, Chicago, IL, United States
- Murray Grossman
- 5Neurology, University of Pennsylvania, Philadelphia, PA, United States
- Michael Howell
- 6Neurology, Medical School, University of Minnesota, Minneapolis, MN, United States
- Diana R. Kerwin
- 7Geriatrics, Presbyterian Hospital of Dallas, Dallas, TX, United States
- Julie Leegwater-Kim
- Neurology, Lahey Hospital and Medical Center, Burlington, MA, United States
- Christiane Lepage
- 2Service de Neurologie, Département de Médecine, Unité de Troubles du Mouvement André-Barbeau, Centre Hospitalier de l'Université de Service de Neurologie, Centre Hospitalier de l'Université de Montréal (CHUM), Montreal, QC, Canada
- Peter Alexander Ljubenkov
- Neurology, University of California, San Francisco, San Francisco, CA, United States
- Martina Mancini
- 0Neurology, Oregon Health and Science University, Portland, OR, United States
- Nikolaus R. McFarland
- 8Neurology, College of Medicine, University of Florida, Gainesville, FL, United States
- Paolo Moretti
- 9Neurology, The University of Utah, Salt Lake City, UT, United States
- Erica Myrick
- 4Neurological Sciences, Rush Medical College, Rush University, Chicago, IL, United States
- Pritika Patel
- Neurology, Lahey Hospital and Medical Center, Burlington, MA, United States
- Laura S. Plummer
- 0Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, United States
- Federico Rodriguez-Porcel
- 1Neurology, Medical University of South Carolina, Charleston, SC, United States
- Julio Rojas
- Neurology, University of California, San Francisco, San Francisco, CA, United States
- Christos Sidiropoulos
- 2Neurology, Michigan State University, East Lansing, MI, United States
- Miriam Sklerov
- 3Neurology, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, United States
- Leonard L. Sokol
- Ken and Ruth Davee Department of Neurology, Feinberg School of Medicine, Northwestern University, Chicago, IL, United States
- Paul J. Tuite
- 6Neurology, Medical School, University of Minnesota, Minneapolis, MN, United States
- Lawren VandeVrede
- 4Neurology, School of Medicine, University of California, San Francisco, San Francisco, CA, United States
- Jennifer Wilhelm
- 0Neurology, Oregon Health and Science University, Portland, OR, United States
- Anne-Marie A. Wills
- 0Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, MA, United States
- Tao Xie
- Neurology, Surgery, University of Chicago, Chicago, IL, United States
- Lawrence I. Golbe
- 5Neurology, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, United States
- DOI
- https://doi.org/10.3389/fneur.2021.694872
- Journal volume & issue
-
Vol. 12
Abstract
Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS; the most common phenotype of corticobasal degeneration) are tauopathies with a relentless course, usually starting in the mid-60s and leading to death after an average of 7 years. There is as yet no specific or disease-modifying treatment. Clinical deficits in PSP are numerous, involve the entire neuraxis, and present as several discrete phenotypes. They center on rigidity, bradykinesia, postural instability, gait freezing, supranuclear ocular motor impairment, dysarthria, dysphagia, incontinence, sleep disorders, frontal cognitive dysfunction, and a variety of behavioral changes. CBS presents with prominent and usually asymmetric dystonia, apraxia, myoclonus, pyramidal signs, and cortical sensory loss. The symptoms and deficits of PSP and CBS are amenable to a variety of treatment strategies but most physicians, including many neurologists, are reluctant to care for patients with these conditions because of unfamiliarity with their multiplicity of interacting symptoms and deficits. CurePSP, the organization devoted to support, research, and education for PSP and CBS, created its CurePSP Centers of Care network in North America in 2017 to improve patient access to clinical expertise and develop collaborations. The directors of the 25 centers have created this consensus document outlining best practices in the management of PSP and CBS. They formed a writing committee for each of 12 sub-topics. A 4-member Steering Committee collated and edited the contributions. The result was returned to the entire cohort of authors for further comments, which were considered for incorporation by the Steering Committee. The authors hope that this publication will serve as a convenient guide for all clinicians caring for patients with PSP and CBS and that it will improve care for patients with these devastating but manageable disorders.
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