Below are some of the commonly asked questions about PSP. If you have any further questions about PSP or CBD, contact a member of our Care and Support team.
What causes PSP/CBD?
The cause is unclear. We do know that a protein called ‘tau' builds up in tangles in the brain. These neuro fibrillary tangles interfere with its working - a similar process to that occurring in Alzheimer's Disease, but in a different part of the brain. This causes a variety of symptoms to appear.
There is now known to be a complex genetic component to PSP. Around twenty percent of the UK population carry the gene which provides susceptibility to PSP (though with a very low level of risk), but the disease itself appears to be triggered environmentally and selectively, perhaps by any one of a variety of neurotoxins or injury to the head.
Is PSP inherited?
The role of heredity in PSP is under current investigation, but the likelihood of transmitting PSP to one's children through genetic mutations is very small. If there is a family history of dementia, Parkinson's disease or PSP, the risks increase a small degree, but are still not high. To date, there have only been seven reported families with autopsy-proven PSP spanning more than one generation in the world.
More about PSP and genetics.
How many people have it?
PSP is much more common than previously believed. Research has confirmed it is a world wide illness and has an incidence [number of people diagnosed annually] for PSP of 5.3:100,000 over the age of 50, and a prevalence[number of people diagnosed and living] of 6.4 per 100,000 of the population. This means that there are at least some 4,000 patients across the UK diagnosed and living with PSP. However, as the incidence tells us about 1250 people are diagnosed each year and the illness lasts over seven years (i.e. 8 as a minimum), this means over 10,000 actually have PSP. Comparing these figures it is clear to see that as there are at least 10,000 with PSP in the UK, but only 4,000 diagnosed, clearly 6,000 people remain un or misdiagnosed and are having to contend with the abject misery that that causes.
PSP and CBD may be difficult to diagnose, particularly in the early stages and often mimic Parkinson's disease or other related neurodegenerative diseases. They may be labelled as other conditions or ‘Parkinson's plus' or atypical parkinsonism when an element of doubt exists about the precise diagnosis. (About three quarters of the people registered with The PSP Association were initially given another diagnosis).
Taking this into account, neurologists estimate the true figure across the UK to be up to 15 per 100,000, (equivalent to 10,000 living patients). This means PSP is at least as common as, for example, Motor Neurone Disease.
CBD is thought to be half as common as PSP.
Can PSP be cured?
There is currently no effective treatment to halt the progression of the disease which is why The PSP Association is supporting research into prevention and a cure. However, many of the symptoms can be managed, to improve the quality of life for individuals and families.
Although average life expectancy is seven years from the onset of symptoms, PSP affects people in many different ways, some living much longer with the condition.