Christine Bycroft has a rare neurological disease. Having spent years waiting for an accurate diagnosis, she is now working to raise the profile of progressive supranuclear palsy, a condition many doctors would fail to recognise

IT was the tremor in her hands that made Christine Bycroft aware that something wasn't right. She had a nagging suspicion it might be something serious but it took years for specialists to diagnose the rare condition which will eventually take her life.

Christine has progressive supranuclear palsy (PSP) - a disease most people have never heard of. It is the condition that actor Dudley Moore had, and is caused by increasing numbers of brain cells becoming damaged over time, causing difficulty with movement, vision, speech and swallowing.

Only known to affect 4000 people in the UK, PSP is often misdiagnosed as other neuro-degenerative conditions such as such as Parkinson’s disease, Alzheimer’s disease, Stroke or Multiple System Atrophy.

Christine was initially diagnosed with Parkinson's disease.

But then came new symptoms: a weakening of her voice and problems with her sight as her eyelids started to shut involuntarily. Most shocking of all was when Christine began to fall backwards, causing her to break her back in one serious fall at her home in Badger Hill, York.

As time went on Christine - who used to work in the medical and nursing library at York Hospital - and her family, became increasingly more aware that the new developments were not symptoms of Parkinson's disease. "We had suspicions something else was wrong but there wasn't much information," Christine's husband Ray said.

It was only when Christine, 72, was referred to York Hospital neurologist Dr Phil Duffy, that he recognised the symptoms and diagnosed her with PSP.

"He looked at her and stood her up and just touched her shoulders and she fell backwards," Ray said, "Obviously he caught her. He knew straight away it was PSP. That's when we got more information."

The information they received must have been tough to process as the deterioration with PSP is more rapid than with Parkinson's. Life expectancy from when the symptoms start is six or seven years. But Christine and her family say they are glad to finally know and understand what is happening as it has allowed them to plan for the future. "There's a natural progression with PSP," Ray said, "We know what's going to happen and approximately when it's going to happen. The next thing is her sight goes, she can't open her eyes. Then her swallowing will be affected. We know these things are going to happen.

"With Parkinson's you can go on forever, for 20 years. With this we know there will be a finality in four to six years.

"Once you know about PSP you can calculate what's going to happen and be prepared for all sorts. We have been referred to the day care centre at the hospice for respite every Wednesday. Then they talk to you about different things such as end of life care."

Christine said she's grateful to have been able to plan ahead, with the couple adapting their home for when she won't be able to climb the stairs.

But in other ways life carries on as normal - they plan to go to the races next week, and Christine continues her hobby of making greeting cards. They attend a PSP Association (PSPA) support group in Pocklington every month. When the grandmother-of-three and her family was diagnosed with Parkinson's she and her family threw themselves into fundraising - raising over £2000 for charity.

Now they're starting again, using PSP Awareness Week next week to raise awareness of the condition. Christine's daughter Rachel said: "People could be like mum, thinking they have Parkinson's when they have PSP and struggling on and they don't know about it."

The PSP Association is encouraging supporters to take action during the awareness week by delivering copy of its PSP Red Flags for GPs document to their local GP surgery. The poster lists ten key symptoms that may help professionals spot warning signs that raise clinical suspicion of PSP.

Paula McGrath, PSPA’s director of communications, said: “Sadly time after time we hear stories from people who have waited far too long for a diagnosis, sometimes a number of years since they first spotted symptoms.

“This year our awareness week campaign aims to see more people living with PSP receive a timely referral to neurology, which in turn should speed up an accurate diagnosis.

“An accurate diagnosis removes uncertainty and a sense of isolation for families. It also means they are able to access relevant health and social care services, receive information and support from PSPA and take part in our research projects if they wish.

“We are very grateful to Ray and Christine for raising funds and awareness to support our work.”

To find out more about PSP and PSPA visit www.pspassociation.org.uk or find the charity on Facebook and Twitter.

- The family is holding a coffee morning on Saturday, May 21 from 10am to 12pm at Osbaldwick Sports, The Leyes, Osbaldwick, York.

FACT FILE

- Progressive supranuclear palsy (PSP) is a rare and progressive condition that can cause problems with balance, movement, vision, speech and swallowing.

- It's caused by increasing numbers of brain cells becoming damaged over time.

- The PSP Association estimates there are around 4,000 people with PSP living in the UK. However, it's thought that the true figure could be much higher, because many cases may be misdiagnosed.

- Most cases of PSP develop in people who are over 60 years of age.

- PSP occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau.

Tau occurs naturally in the brain and is usually broken down before it reaches high levels. In people with PSP, it isn't broken down properly and forms harmful clumps in brain cells. Where these clumps form and the amount of abnormal tau in the brain can vary in people with PSP, which means the condition can have a wide range of symptoms.

- The condition has been linked to changes in certain genes, but these genetic faults are not inherited and the risk to other family members, including children or siblings of someone with PSP, is very low.

- The symptoms of PSP usually get gradually worse over time. At first, the symptoms can be similar to some other conditions, which makes it difficult to diagnose early on.

- Some of the main symptoms of PSP include: problems with balance and mobility, including frequent falls, changes in behaviour, such as irritability or apathy (lack of interest) muscle stiffness , an inability to control eye and eyelid movement, including focusing on specific objects or looking up or down at something, slow, quiet or slurred speech, difficulty swallowing (dysphagia), slowness of thought and some memory problems - The rate at which the symptoms progress can vary widely from person to person.