Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD) Rare, complex and often misunderstood neurological conditions
As part of Rare Disease Day, this week on Word on Health we’re speaking to consultant neurologist Ed Jabari about two rare but devastating neurodegenerative conditions: Progressive Supranuclear Palsy (PSP) and Corticobasal Degeneration (CBD). (You can hear our short radio feature at the bottom of this page - a more detailed extended interview is available our podcast which you can access via out 'in profile' page.
Both PSP & CBD affect movement, thinking and behaviour. They are uncommon, frequently misdiagnosed, and can present in ways that overlap with more familiar disorders such as Parkinson’s disease. Below you’ll find a clear overview of each condition, their symptoms, diagnosis and management.
What is Progressive Supranuclear Palsy (PSP)? It is a rare brain disorder that gradually damages specific areas of the brain, particularly those controlling balance, eye movements, speech and swallowing.It is sometimes initially mistaken for Parkinson’s disease, but PSP behaves differently and does not typically respond well to standard Parkinson’s medications.
Common symptoms of PSP:
One of the hallmark signs of PSP is difficulty with vertical eye movement — particularly looking down — which can make everyday activities such as walking downstairs extremely challenging.
What causes PSP? PSP is caused by an abnormal build-up of a protein called tau in brain cells. This protein accumulation damages and eventually destroys nerve cells. PSP is therefore classified as a tauopathy.It is not contagious and, in most cases, is not directly inherited.
Who is affected? PSP usually develops after the age of 60. It is considered rare, affecting only a few people per 100,000 of the population.
What is Corticobasal Degeneration (CBD). It is another rare neurodegenerative disorder and, like PSP, is also a tauopathy.CBD primarily affects the cerebral cortex (responsible for thinking and voluntary movement) and the basal ganglia (involved in movement control).
Common symptoms of CBD. CBD often begins asymmetrically — affecting one side of the body more than the other.Symptoms may include:
Because symptoms vary significantly between individuals, diagnosis can be challenging.
How are PSP and CBD Diagnosed? There is currently no single definitive test for either condition.Diagnosis is based on:
Both PSP and CBD can initially resemble:
Accurate diagnosis often requires specialist neurological expertise.
Is There Treatment? There is currently no cure for PSP or CBD, and no treatment that can stop disease progression.However, supportive management can significantly improve quality of life. This may include:
Care is typically coordinated through a multidisciplinary team.
Living with a Rare Neurodegenerative Condition. Receiving a diagnosis of PSP or CBD can be overwhelming. Because these conditions are rare, patients and families often feel isolated or struggle to find clear information.
Awareness matters - Rare Disease Day aims to shine a light on conditions that individually affect small numbers of people, but collectively impact millions worldwide.
If you or a loved one are experiencing unexplained balance problems, unusual eye movement difficulties, asymmetric stiffness, or unexplained cognitive changes, seek medical advice from your GP and request referral to a neurologist if appropriate.
Further Support: Patients and families may benefit from specialist neurology services and help and support from the PSP Association (see our links page to connect through to the charity)
Listen to this weeks radio report
All material on this website is provided for your information only and may not be construed as medical advice or instruction. No action or inaction should be taken based solely on the contents of this information; instead, readers should consult appropriate health professionals on any matter relating to their health and well-being.
