Progressive supranuclear palsy (PSP) is an FTD disorder primarily affecting movement. The earliest motor symptoms are stiffness in the axial muscles (the neck and trunk), along with poor balance and more frequent falls. The earliest visual signs are a decrease in upward vertical movement of the eyes and a progressive inability to move the eyes, including opening or closing the eyes. PSP can also affect coordination, and movement of the mouth, tongue, and throat. In addition to motor symptoms, people with PSP may exhibit changes in personality and behavior. PSP may also cause challenges related to language and communication, particularly as the disease progresses.
What is PSP?
Progressive supranuclear palsy (PSP) is an FTD disorder primarily affecting movement. The earliest motor symptoms are stiffness in the axial muscles (the neck and trunk), along with poor balance and more frequent falls. The earliest visual signs are a decrease in upward vertical movement of the eyes and a progressive inability to move the eyes, including opening or closing the eyes. PSP can also affect coordination, and movement of the mouth, tongue, and throat. In addition to motor symptoms, people with PSP may exhibit changes in personality and behavior. PSP may also cause challenges related to language and communication, particularly as the disease progresses.
What Causes PSP?
PSP is a tauopathy, a disease caused by misfolding of the tau protein in the brain. Tau is a normal protein found in brain cells. In PSP, the tau seems to become abnormally folded, which causes it to stick together, creating neurofibrillary tangles which then become stuck inside brain cells. The areas of the brain that have cells with tau inside of them also exhibit impaired neuron function and neuronal death. The symptoms of PSP are caused by a gradual deterioration of brain cells in the base of the brain. Genetic differences and exposure to certain chemicals are being explored as possible causes for PSP, but ultimately, the causes for and certain individuals develop PSP are not fully understood.
MGH FTD Unit PSP Program
The MGH FTD Unit’s PSP program offers a cross-disciplinary collaborative approach to caring for people living with PSP as well as for their families. Our patients receive a highly specialized clinical evaluation, which can diagnose or provide a second opinion of a prior PSP diagnosis. We also facilitate referrals to appropriate specialists and resources designed to optimize quality of life in line with evidence-based treatment guidelines. We are committed to supporting families through their whole journey with this disease and as such, we offer comprehensive support and education about living with PSP and how to plan for the future. Our patients also have the opportunity to participate in research designed to better understand and develop future treatments for those living with PSP. For questions about program, email mghftdunit@mgh.harvard.edu.
Highly Specialized Clinical Evaluation
Our team has over a decade of experience in diagnosing and caring for individuals with PSP. At your initial clinic appointment, you will be evaluated by an interdisciplinary team comprised of neurologists, neuropsychologists, speech-language therapists, and caregiver support specialists. These evaluations will include a detailed clinical interview with patients and family members, as well as neurocognitive testing, with the aim of guiding diagnosis and treatment.
Referrals and Educational Resources
In the event that our patients would benefit from other specialty clinical services, we facilitate these referrals and work closely with these providers to optimize clinical care. Specifically, these specialties include occupational and physical therapists who have familiarity with treating PSP patients, as well as speech and language therapists who can provide cognitive and language therapy to optimize cognitive functioning in the earlier stages of the disease.
We also suggest reviewing informational websites such as Cure PSP, PSPA, the Alzheimer’s Association, the Alzheimer’s Research UK, Rare Dementia Support, the Association for Frontotemporal Degeneration, and this PSP blog.
Support and Education
MGH FTD Unit Caregiver Support Services:
Our team of caregiver support specialists can meet with your family to identify goals of care, establish a person-centered care plan, and connect you to community resources. We can also provide education specifically about the PSP syndrome and what to expect as the disease progresses. Learn more about MGH FTD Unit Caregiver Support Services.
From Care to Cure Newsletter:
The MGH FTD Unit is committed to providing connections with relevant information, helpful resources, and support within our community of patients and care partners. Each issue highlights current research projects and publications, authentic caregiver and patient voices, and spotlights relevant topics to improve quality of life. Email mghftdunit@mgh.harvard.edu to sign up for our online newsletter today.
Participation in Research
Our team is conducting several ongoing research studies aimed at better understanding the clinical, cognitive, and neurological features of PSP. Study participation generally includes detailed neurocognitive testing and comprehensive clinical interviews of the participant and caregiver experience. Neurocognitive testing is currently being administered remotely via Zoom video conferencing technology and may transition to in-person testing at the Charlestown Navy Yard in the future. Additional imaging (MRI or PET scans) or biomarker (cerebral spinal fluid or blood draws) evaluations may also be available for patients where appropriate. All participants will be reimbursed for parking if applicable and will be monetarily compensated for the testing session whether remotely or in person. Participants will have the opportunity to access educational and support resources to better understand how to live with the diagnosis of PSP. To learn more, please email mghftdunit@mgh.harvard.edu.
What have we learned from our research so far?
- Differences in Motor Features of C9orf72, MAPT, or GRN Variant Carriers With Familial Frontotemporal Lobar Degeneration
Philip Wade Tipton, Angela B. Deutschlaender, Rodolfo Savica, Michael G. Heckman, Danielle E. Brushaber, Bradford C. Dickerson, Ralitza H. Gavrilova, Daniel H. Geschwind, Nupur Ghoshal, Jonathan Graff-Radford, Neill R. Graff-Radford, Murray Grossman, Ging-Yuek R. Hsiung, Edward D. Huey, David John Irwin, David T. Jones, David S. Knopman, Scott M. McGinnis, Rosa Rademakers, Eliana Marisa Ramos, Leah K. Forsberg, Hilary W. Heuer, Chiadi Onyike, Carmela Tartaglia, Kimiko Domoto-Reilly, Erik D. Roberson, Mario F. Mendez, Irene Litvan, Brian S. Appleby, Ian Grant, Daniel Kaufer, Adam L. Boxer, Howard J. Rosen, Brad F. Boeve, Zbigniew K. Wszolek; ALLFTD Consortium. Neurology. 2022 Sep 13;99(11):e1154-e1167. doi: 10.1212/WNL.0000000000200860. Epub 2022 Jul 5. - 18 F-flortaucipir tau positron emission tomography distinguishes established progressive supranuclear palsy from controls and Parkinson disease: A multicenter study
Daniel R Schonhaut, Corey T McMillan, Salvatore Spina, Bradford C Dickerson, Andrew Siderowf, Michael D Devous Sr, Richard Tsai, Joseph Winer, David S Russell, Irene Litvan, Erik D Roberson, William W Seeley, Lea T Grinberg, Joel H Kramer, Bruce L Miller, Peter Pressman, Ilya Nasrallah, Suzanne L Baker, Stephen N Gomperts, Keith A Johnson, Murray Grossman, William J Jagust, Adam L Boxer, Gil D Rabinovici. Ann Neurol
. 2017 Oct;82(4):622-634. doi: 10.1002/ana.25060. - Progression of Microstructural Degeneration in Progressive Supranuclear Palsy and Corticobasal Syndrome: A Longitudinal Diffusion Tensor Imaging Study
Yu Zhang, Rudolph Walter, Peter Ng, Phi N Luong, Shubir Dutt, Hilary Heuer, Julio C Rojas-Rodriguez, Richard Tsai, Irene Litvan, Bradford C Dickerson, Maria Carmela Tartaglia, Gil Rabinovici, Bruce L Miller, Howard J Rosen, Norbert Schuff, Adam L Boxer. PLoS One. 2016 Jun 16;11(6):e0157218. doi: 10.1371/journal.pone.0157218. eCollection 2016. - O1-02-01: Imaging tau pathology in vivo in FTLD: Initial experience with [18F] T807 PET.
Dickerson, B., Domoto-Reilly, K., Daisy, S., Brickhouse, M., Stepanovic, M. and Johnson, K. (2014), Alzheimer’s & Dementia, 10: P131-P131. https://doi.org/10.1016/j.jalz.2014.04.062
We are always looking for individuals who would like to participate in our efforts, whether they be patients, family members, interested individuals from the public, potential donors, other health care professionals, or other researchers.