Let’s demystify progressive supranuclear palsy
The physical and cognitive struggles of patients with progressive supranuclear palsy (PSP) are not often visible. Like centaurs, they are constantly seeking harmony between two worlds. Their balance is thrown off from within as tau protein accumulates in their brains.
Together, we can make their experiences seen and help patients with PSP regain their natural equilibrium.
What is PSP?
PSP is a primary tauopathy characterised by abnormal accumulation of tau protein in brain areas that control body movements and thinking. This predominantly occurs in the subcortical and brainstem regions, involving both neuronal and glial components.1,2
Tau accumulation leads to nerve cell damage and disrupts normal brain function, leading to the appearance of many different symptoms, including loss of balance, difficulty swallowing or problems with eye movement.3
As tau protein builds up, I can feel my balance slipping away. Even swallowing and moving my eyes is challenging.3
PSP can be expressed in different forms called variants. Each variant has key distinctive symptoms and is named according to the most predominant of them:10
PSP
Richardson’s Syndrome (PSP-RS)
Ocular motor problems & early postural instability and falls
PSP with parkinsonism (PSP-P)
Tremors, slowness of movement
Pure akinesia with gait freezing (PSP-PGF)
Inability to voluntarily move one’s muscles or limbs
Corticobasal syndrome (PSP-CBS)
Neurodegeneration of cerebral cortex and basal ganglia
Primary progressive apraxia of speech (PSP-SL)
Speech/language disorder
Behavioural frontotemporal dementia (PSP-F)
Neurodegeneration of the frontal lobe and sometimes temporal lobe
PSP with cerebellar ataxia (PSP-C)
Sudden, uncoordinated muscle movement due to injury to the cerebellum
PSP centaurs are more than myth
100,000.4
100,000.4
Cases of familial PSP due to inherited mutations in genes are very rare.7
PSP is considered a sporadic disease with an unknown cause.5,6
Risk factors
Advanced age
Genetic mutations
>10 genes
are linked to risk8
MAPT mutations are the strongest genetic risk factor.8
Gender
Environmental factors
Know the symptoms
PSP symptoms can vary and evolve over time. These might include alterations in movement, speech, thought, sleep or mood.3,4
Movement
- Eye and eyelid movement
- Difficulty swallowing and breathing
- Imbalance, walking difficulty and frequent falls
Speech
- Difficulty finding the proper words
- Slowed speech
Cognition and sleep
- Forgetfulness
- Changes in judgment
- Sleep problems and insomnia
Mood
- Depression and lack of motivation
- Personality changes
How does PSP progress?
PSP progression occurs rapidly and gradually interferes with independence and daily activities.
Although we have the same condition, PSP progresses at different rates for everyone. Our individual experiences vary significantly.4,9,11,12
Providing personalised and multidisciplinary care
Although there are no approved therapies for PSP, managing the symptoms can improve the quality of life of patients.
An early diagnosis can help avoid delays in symptom management.13
These are some non-pharmacological treatment options available to manage PSP symptoms:3, 6, 13
Mobility and falls management
Occupational therapy to promote independence
Physiotherapy to improve mobility and balance, and manage falls (e.g. using weighted equipment to prevent backward falls)
Special glasses for eye movement problems
Dysphagia
Dietitian therapy to manage difficulty swallowing (e.g. modified diets and fluid thickeners) and prevent associated vitamin D deficiency (e.g. Calcium-D3 supplements)
Speech
Speech therapy to alleviate communication difficulties
Emotional care and support
Support through patient organisations (e.g. ERN-RND) and associations (e.g. PSPA and CurePSP)
Hear from PSP centaurs
Person living with PSP:
“No one can help us more than we can help ourselves. The support and understanding of those around us – especially the people we live with daily – is very important.”14
Caregiver of a person living with PSP:
“Yes, I as a carer and husband have the odd “rough day”, but then I remember she has many more rough days and nights. My ongoing care, with love and purpose, will keep us living happily and independently together for as long as possible.”14
A multidisciplinary care plan not only improves our quality of life, but also helps our families and caregivers.4, 11
PSP frequently asked questions
PSP is a rare disorder that affects between 5.8-6.5 people per 100,000.5
PSP is classified as a tauopathy. Tauopathies are diseases where tau protein accumulates in the brain, leading to neuronal failure and death. Tau protein helps support the structure of neurons by attaching to structures called microtubules. In tauopathies, tau detaches from the microtubules and aggregate with each other creating tangles. The accumulation of tau tangles damage neurons and lead to the PSP symptoms.8
Parkinson’s disease (PD) and PSP are distinct neurodegenerative disorders despite sharing some symptoms.15
These are some of the main differences:
| PSP | PD | |
|---|---|---|
| Protein pathology16, 17 | Primary tauopathy (Tau protein accumulation) |
Synucleinopathy (alfa-synuclein accumulation) |
| Age of onset and disease progression18, 19 | 60s Faster progression |
Early 60s to 70s Slower progression |
| Symptoms | Slowness, stiffness, and balance problem | |
| Falls20, 21 | Early in disease progression, often backwards (due to postural instability), without warning or loss of consciousness | Later in disease progression, often forward (due to freezing of gait) |
| Tremors22, 23 | Rare or mild(for PSP-RS) Can occur and mimic early PD stages (for PSP-P) |
Classic and common (resting tremor) |
| Eye problems15, 19 | Classic and common (difficulty looking up and down) | Not a characteristic symptom, usually preserved |
| Speech and swallowing difficulties19 | More frequent Earlier appearance |
Less frequent Later appearance |
Although most cases are sporadic with unknown cause, familial PSP cases have also been detected.7 They are very rare and occur due to a mutation in specific genes such as MAPT — the gene producing tau protein.3
There are 3 stages in PSP disease:10
- Presymptomatic PSP. The disease is beginning to develop, but patients do not yet present symptoms.
- Suggestive PSP. Patients develop mild or isolated symptoms (e.g. unexplained falls or slight changes in speech), but do not meet the full PSP criteria.
- Symptomatic PSP. Patients exhibit noticeable and clear symptoms (e.g. severe postural instability and swallowing and speech difficulties), meeting the criteria for Richardson’s syndrome (PSP-RS) or another clinical PSP variants.
References
1. Murray ME, Kouri N, Lin WL, Jack CR, Jr., Dickson DW, Vemuri P. Clinicopathologic assessment and imaging of tauopathies in neurodegenerative dementias. Alzheimers Res Ther. 2014;6(1):1. doi: 10.1186/alzrt231. 2. Planche V, Mansencal B, Manjon JV, Meissner WG, Tourdias T, Coupé P. Staging of progressive supranuclear palsy-Richardson syndrome using MRI brain charts for the human lifespan. Brain Commun. 2024;6(2):fcae055. doi: 10.1093/braincomms/fcae055. 3. National Institute of Neurological Disorders and Stroke (NINDS) [Internet]. Bethesda: National Institutes of Health; start date unknown [last updated date unknown; cited 23 Apr 2025]. Available at: https://www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp. 4. Agarwal S, Gilbert R. Progressive Supranuclear Palsy. StatPearls. Treasure Island (FL): StatPearls Publishing Copyright © 2025, StatPearls Publishing LLC.; 2025. 5. Lyons S, Trépel D, Lynch T, Walsh R, O'Dowd S. The prevalence and incidence of progressive supranuclear palsy and corticobasal syndrome: a systematic review and meta-analysis. J Neurol. 2023;270(9):4451-65. doi: 10.1007/s00415-023-11791-2. 6. Ichikawa-Escamilla E, Velasco-Martínez RA, Adalid-Peralta L. Progressive Supranuclear Palsy syndrome: an overview. IBRO Neurosci Rep. 2024;16:598-608. doi: 10.1016/j.ibneur.2024.04.008. 7. Donker Kaat L, Boon AJ, Azmani A, Kamphorst W, Breteler MM, Anar B, et al. Familial aggregation of parkinsonism in progressive supranuclear palsy. Neurology. 2009;73(2):98-105. doi: 10.1212/WNL.0b013e3181a92bcc. 8. Zhang Y, Wu KM, Yang L, Dong Q, Yu JT. Tauopathies: new perspectives and challenges. Mol Neurodegener. 2022;17(1):28. doi: 10.1186/s13024-022-00533-z. 9. Mahale RR, Krishnan S, Divya KP, Jisha VT, Kishore A. Gender differences in progressive supranuclear palsy. Acta Neurol Belg. 2022;122(2):357-62. doi: 10.1007/s13760-021-01599-0. 10. 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):552-63. doi: 10.1016/s1474-4422(17)30157-6. 11. Markiewicz M, Madetko-Alster N, Alster P. Quality of life in patients with progressive supranuclear palsy: a review of literature and implications for practice. Front Neurol. 2024;15:1476488. doi: 10.3389/fneur.2024.1476488. 12. dell'Aquila C, Zoccolella S, Cardinali V, de Mari M, Iliceto G, Tartaglione B, et al. Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients. Parkinsonism Relat Disord. 2013;19(11):980-5. doi: 10.1016/j.parkreldis.2013.06.014. 13. Rowe JB, Holland N, Rittman T. Progressive supranuclear palsy: diagnosis and management. Pract Neurol. 2021;21(5):376-83. doi: 10.1136/practneurol-2020-002794. 14. CCF for PSP Awareness [Internet]. United States: PSP Awareness; start date unknown [last updated date unknown; cited 23 Apr 2025]. Available at: https://pspawareness.com/pages/faces-voices-of-atypical-parkinsonism. 15. 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. doi: 10.1002/mds.26987. 16. Dickson DW, Rademakers R, Hutton ML. Progressive supranuclear palsy: pathology and genetics. Brain Pathol. 2007;17(1):74-82. doi: 10.1111/j.1750-3639.2007.00054.x. 17. Spillantini MG, Schmidt ML, Lee VM, Trojanowski JQ, Jakes R, Goedert M. Alpha-synuclein in Lewy bodies. Nature. 1997;388(6645):839-40. doi: 10.1038/42166. 18. Golbe LI, Davis PH. Progressive supranuclear palsy. N Engl J Med. 2006;355(18):1874–83. doi:10.1056/NEJMra052731. 19. Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Lancet Neurol. 2009;8(3):270-9. doi: 10.1016/s1474-4422(09)70042-0. 20. 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. doi: 10.1212/wnl.47.1.1. 21. 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. doi: 10.1002/mds.25327. 22. 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(Pt 6):1247-58. doi: 10.1093/brain/awh488. 23. Dickson DW. Parkinson's disease and parkinsonism: neuropathology. Cold Spring Harb Perspect Med. 2012;2(8). doi: 10.1101/cshperspect.a009258.
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