Our grateful thanks to the PSP Association for their input to our on-air report and for the use of the information below. To find out more about PSP and to access help and support click here to visit their website.
A Brief Guide to PSP
PSP is caused by the progressive death of nerve cells in the brain, leading to difficulty with balance, movement, vision, speech and swallowing. It is so called because it’s:
PSP is associated with an over-production of a protein called tau in certain areas of the brain. In PSP, it forms into clumps – or neurofibrillary tangles – which are believed to damage nerve cells.
There is nothing to suggest that the disease is inherited but research indicates that some people may have a genetic susceptibility that puts them more at risk of developing the condition than others.
Prevalence PSP is a rare condition. Research into the prevalence of the disease suggests that there are around 4,000 people in the UK living with the condition at any one time, though neurologists believe the figure could be as high as 10,000.
Signs and Symptoms Early symptoms may include loss of balance and unexpected falls (usually backwards), stiffness and eye problems. These can include difficulties in looking up or down, focusing, double or tunnel vision and dislike of bright lights.
Some people can experience behavioural and cognitive changes – depression, apathy, clumsiness, or tiny, cramped handwriting. Early on, symptoms may resemble those of other neurodegenerative diseases such as Parkinson’s disease, Alzheimer’s disease, Motor Neurone disease or Multiple System Atrophy. As a result, many people are initially misdiagnosed.
As PSP progresses, symptoms increase. There may be problems with swallowing, slurred speech, recurrent falls, irritability and apathy, slowness of response and severe difficulties walking. Sometimes the eyelids close involuntarily and it becomes increasingly difficult to look up or down. Emotional lability, such as laughing or crying inappropriately, maybe another feature and there can be incontinence.
The average life expectancy is around seven years from onset. However, every case is different – there is considerable variation in the symptoms and rate of progression in individuals.
Diagnosis There are no simple tests to diagnose PSP. Brain scans are often used by neurologists for diagnosis as they help by excluding other conditions.
Due to the slowness of movement and balance problems, PSP is often initially diagnosed as Parkinson’s disease, a stroke or a brain tumour. Sometimes it is misdiagnosed as Alzheimer’s because of changes in mood, intellect and personality that can occur.
PSP often goes undiagnosed in the elderly, especially for those living in care homes.
Data held by the PSP Association indicates that three out of every four people with PSP are initially misdiagnosed and often repeatedly misdiagnosed with other conditions.
There are no effective treatments for PSP but there are many therapies that can help manage symptoms. People with PSP should see their neurologist and palliative care team on a regular basis.
Hopefully, in the future, research will lead to new treatments to stop or at least slow down the progression of the condition.
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