When keen walker Beth Shepheard-Walwyn fell over on stepping stones while crossing a river, she feared it was yet another worrying sign about her health.
“Crossing rivers shouldn’t have been an issue for me as I’d done it many times before, but I lost my concentration and landed on my bottom,” recalls Beth, 66, a retired primary school teacher and tutor about her fall six years ago. “Although it caused no real harm, it felt unusual.
“Around this time I’d also noticed that I was now struggling to play the piano – my right hand was fine, but my left didn’t know what to do.
“I struggled with everyday tasks like putting on a coat or unhooking clips too. Despite then only being 60, I thought I was just getting old, but felt something wasn’t quite right.”
Beth also experienced dramatic weight loss and occasional involuntary limb movements, so eventually saw her GP in early 2020.
He suggested it might be caused by a stroke or even ataxia, a condition where people struggle with speech, balance or coordination. Her doctor immediately referred her to a neurologist for further investigation.
“When I saw the neurologist, it was a case of ruling out conditions such as Parkinson’s by going through all of my symptoms before making a diagnosis,” says Beth, who lives with her husband Hugh in Stourbridge, West Midlands.
“I also told him of an odd experience I’d had one day where I looked at my hand but felt it belonged to someone else instead of me.”
This symptom, which is known as alien hand syndrome, is defined when one hand appears to act independently of the person’s mind.
This, along with her other symptoms, led to Beth being diagnosed with corticobasal degeneration (CBD).
It’s a form of dementia that affects movement by causing shaking, jerky spasms and difficulty controlling a limb on one side. Balance and swallowing can also be impaired along with slurred speech and memory issues. It shares many of the symptoms of Parkinson’s.
CBD is caused by parts of the brain becoming damaged by a build-up of a protein called tau, which in most of us is broken down before causing any harm. The condition usually develops in those aged between 50 and 70, with symptoms gradually worsening over time.
Sadly there’s currently no cure and treatment tends to be limited to physiotherapy to help with balance and drugs (usually the same ones taken by those living with Parkinson’s) to help with movement.
Sharing many similarities with another rare neurological disorder called progressive supranuclear palsy (PSP), it’s believed about 10,000 people in the UK live with either one of these conditions. Despite her shattering diagnosis, Beth and Hugh were adamant it wouldn’t stop their long-distance walking challenges.
Now they believe determination to keep going is helping to slow Beth’s progression of the disease, with recent studies showing that regular exercise might help delay the symptoms of other brain-related issues like Parkinson’s.
“We began walking the 630-mile long South West Coast Path in 2013, seven years before I went to the GP with my symptoms. It was something I’d always wanted to do,” says Beth.
“We completed sections of it during our holidays and the odd weekend and it was while working towards completing the whole route that I first noticed I had begun to walk at a slower pace. Before I would have been striding out ahead of Hugh. It was another sign something was amiss.”
The couple finally completed the epic walk in late 2021, 18 months after Beth’s diagnosis.
Keen gardener Beth, who also attends weekly exercise classes to help manage her symptoms, is now working on walking the length of the 870-mile Wales Coast Path.
“We haven’t set a date to complete it and will do it at our own pace, enjoying it as we go,” says Hugh.
Beth takes medicines to dampen down her jerky limb twitches and wears a splint on her left arm to stop her “alien” arm making involuntary movements.
She is enrolled in the PROSPECT-M study, which has recruited more than 1,000 people living with CBD or PSP. It has created an international research network using samples and brain images to shed more light on the possible causes and progression of these rare disorders. Beth undergoes tests and observations every 12 months to assess the disease’s advances.
James Rowe, a Cambridge University professor of cognitive neurology who heads up a specialist clinic into these conditions, is hopeful more can be done to help patients. “It’s an exciting time to be a doctor and scientist in the PSP and CBD field,” he says.
“For many years, they were the Cinderella topics, seen as too hard, too rare, too uncertain to be able to get funding and attention.
“A small group of enthusiasts persisted, and now PSP especially has moved centre stage.
“There are research papers on the biology of the illness and there have been multiple clinical trials of new drugs.”
The PSP Association, the national charity supporting those with PSP and CBD, is calling for people to have faster referrals to see a neurologist to avoid an incorrect diagnosis – as sometimes happens.
Despite now struggling to read, write and having poor fine motor skills, Beth is determined to remainactive.
“You have to look forward and be relaxed about everything,” she says.
“If I’m uptight about anything, everything gets worse with my arm jerking about more.
“I’ve tried to learn to accept it and live with it as best I can.”
* PSPA is the only UK charity dedicated to creating a better future for people living with PSP and CBD. To learn more, visit pspassociation.org.uk
