Discover more aboutProgressive supranuclear palsy (PSP) is a rare and severe neurodegenerative brain disorder for which there is no cure.1

People with PSP experience issues with balance and walking, speech, swallowing, vision, mood, behaviour and thinking.2

The symptoms of PSP are not widely understood. Because of this, PSP is often misdiagnosed, or diagnosis is delayed by an average of nearly 2.5 years.3

PSP progresses rapidly – life expectancy from symptom onset is approximately 6–8 years.4

Achieving earlier diagnosis of PSP is therefore a key goal for people a�ected by the disease – for patients, neurologists, families and carers.

In the brains of people with PSP, tau is misfolded and sticky, and clumps of misfolded tau protein are responsible for the degeneration of brain cells.5

The exact causes of PSP are unknown. However, the disease is defined by the loss of cells in certain regions of the brain, brought about by an accumulation of a protein called “tau” inside the brain cell.5

What causes PSP?

What are the symptoms of PSP?

PSPProgressiveSupranuclear

Palsy

Symptoms of PSP vary from person to person, as such, the experience of living with PSP also varies from person to person.1,2

Some of the main symptoms include:1,2

Problems with balance and mobility, including falls (mainly

backwards)

Lack of eye movements (up and down and left and right); reduced eyesight,

including inability to control eye and eyelid

movement

Forgetfulness and personality/behavioural changes (e.g. loss of interest in ordinary pleasurable activities, increased

irritability, and inappropriate behaviour)

Musclesti�ness

Slow or slurred speech

Di�culty swallowing

Slowness of thought and

some memory problems

Symptoms are dependent on the area of the brain a�ected and some may only appear in the latter stages of the disease.5

Who is a�ectedby PSP?

5–6 people in every 100,000 worldwide are thought to live with PSP.6

Each year, an average of 1.1 people per 100,000 are newly diagnosed with PSP.6

On average, symptoms of PSP begin after age 63, but can occur earlier.2,6

Over a third (41%) of people with PSP are misdiagnosed. People with PSP are often misdiagnosed with other movement disorders such as Parkinson’s disease because they share similar symptoms.2,7

It takes usually 2–3 years to receive a clinical diagnosis of PSP. Specialists can make the diagnosis with over 90% accuracy but a definitive diagnosis can only be made post-mortem.8

Treatment Is PSP inherited?

There are currently no treatments to slow down, halt, or reverse the progression of PSP; only treatments that manage the symptoms.1,9

No, PSP is thought to be a sporadic disease (not inherited).10

There are a few rare cases in which PSP has been reported in families but the genetic causes are unknown.10

Socioeconomicburden

It is estimated that annual healthcare costs in the EU associated with PSP are between €1.2–2.7 billion.11

The average healthcare costs for someone with PSP living in the US is estimated to be $38,975 per year.12

The economic burden of disease for PSP is higher than other movement disorders, such as Parkinson's disease.7

Challenges regularly faced by people with PSP and their families include:7,13–15

Significant direct and indirect costs resulting in a

substantial reduction in household income

Reduced qualityof life

Inadequate management of symptoms

Increased caregiver burden, including burnout,

psychological strain and depression, especially

among caregivers of those with late stage PSP

Lack of knowledge

Limited service access and interactions

Inadequate research and research dissemination

References1. NHS. Progressive supranuclear palsy. https://www.nhs.uk/conditions/ progressive-supranuclear-palsy-psp/. Last accessed September 2019.

2. National Institute of Neurological Disorders and Stroke. Progressive Supranuclear Palsy Fact Sheet. https://www.ninds.nih.gov/Disorders/ Patient-Caregiver-Education/ Fact-Sheets/Progressive-Supranuclear-Palsy-Fact-Sheet.Last accessed September 2019.

3. Mamarabadi et al. (2018) Is the Latency from Progressive Supranuclear Palsy Onset to Diagnosis Improving? Mov Disord Clin Pract. 5(6):603-606.

4. Cosseddu et al. (2017) Natural history and predictors of survival in progressive supranuclear palsy. J Neurol Sci 382:105-7.

5. European Parkinson's Disease Association. Progressive Supranuclear Palsy (PSP). https://www.epda.eu.com/about-parkinsons/types/progressive-supranuclear-palsy-psp/ Last accessed September 2019.

6. CurePSP. The CurePSP Guidebook. https://www.psp.org/wp-content/uploads/2018/08/August-2018-GUIDEBOOK.pdf. Last accessed September 2019.

7. Tilley et al. (2014) The e�ectiveness of allied health therapy in the symptomatic management of progressive supranuclear palsy: a systematic review protocol. JBI Database of Systematic Reviews and Implementation Reports. 12(7):119–137. https://insights.ovid.com/jbi-database-systematic-reviews-implementation/jbisris/2014/12/070/e�ectiveness-allied-health-therapy-symptomatic/12/01938924. Last accessed September 2019.

8. PSP Association. A Guide to PSP and CBD for General Practitioners and the Primary Healthcare Team. https://pspassociation.org.uk/app/uploads/2018/07/A-Guide-to-PSP-CBD-for-GPs-and-the-Primary-Healthcare-Team-1.pdf. Last accessed September 2019.

9. CurePSP. About CurePSP. https://www.psp.org/about-curepsp/. Last accessed September 2019.

10. Genetic and Rare Diseases Information Center. Progressive supranuclear palsy. https://rarediseases.info.nih.gov/diseases/7471/progressive-supranuclear-palsy. Last accessed September 2019.

11. McCrone P. et al. (2011). The Economic Costs of Progressive Supranuclear Palsy and Multiple System Atrophy in France, Germany and the United Kingdom. PLoS ONE, 6 (9). e24369. ISSN 1932-6203 https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0024369. Last accessed September 2019.

12. T. Xie, X. Ye, L. Kandukuri, Y. Bao. Burden among Patients with Progressive Supranuclear Palsy [abstract]. Mov Disord. 2018; 33 (suppl 2). https://www.mdsabstracts.org/abstract/burden-among-patients-with-progressive-supranuclear-palsy/. Accessed September 2019.

13. Galvin JE et al. (2017). The social and economic burden of frontotemporal degeneration. Neurology. 89 (20) 2049-2056.

14. Moore T and Guttman M. (2004). Challenges Faced by Patients With Progressive Supranuclear Palsy and their Families. Movement Disorders Clinical Practice. 1(3):188-193.

15. Schmotz C et al. (2017). High Burden and Depression Among Late-Stage Idiopathic Parkinson Disease and Progressive Supranuclear Palsy Caregivers. J Geriatr Psychiatry Neurol. 30(5):267-272.

GL-N-OT—1900032 Date of preparation: 09/19