The stereotypical image of someone with Parkinson’s is of an old man but, as our stories show, it isn’t just a disease of later life and can affect both sexes. Around 127,000 people in the UK have Parkinson’s and 4,500 of them are under 45. One in 28 people diagnosed is under 45, and one in 100 below 40. What’s more, although men are 60 per cent more likely to develop it, women are affected too.
What is Parkinson’s?
‘It’s a progressive, neurological condition caused by the death of the nerve cells (neurons) that produce dopamine, a messenger chemical that controls movement, in a brain region called the substantia nigra,’ explains neurologist Professor David Burn, clinical director of Parkinson’s UK. It’s this loss of dopamine that leads to the classic ‘motor’ (movement) symptoms.
But that’s not all. There’s growing evidence that other systems and messenger chemicals are involved, including the so-called ‘happiness hormone’, serotonin. ‘It’s thought that an abnormal protein, a-synuclein, builds up in dopamine-producing cells in parts of the body, such as the bowel, and is passed like a relay baton from one nerve cell to the next,’ says Professor Burn.
Unfortunately, by the time symptoms become noticeable, 50-60 per cent of affected brain cells and 70-80 per cent of dopamine is lost. Which is why there’s a massive drive to discover early biological ‘markers’ – as well as ‘non-motor’ symptoms that could flag up Parkinson’s much earlier.
How is young onset Parkinson’s different?
Progression can be slower in younger sufferers and symptoms such as tremor and problems with walking and balance tend to be less severe, says Parkinson’s UK researcher, Professor Huw Morris, consultant neurologist at University College London’s Institute of Neurology, who is studying young onset Parkinson’s. ‘This may be because the Parkinson’s we’re seeing in younger people is actually a different disease, or because younger people are less likely to have other complicating health problems,’ he explains. However, genes are slightly more likely to be a factor, and involuntary muscle movements (dyskinesias) linked to long-term use of the main anti-Parkinson’s drug, Levodopa (L-Dopa) can be more problematic.
Why is young onset Parkinson’s often missed?
‘GPs usually aren’t on the lookout for Parkinson’s in a younger person, as the average age of onset is 55-60 and it’s often missed or misdiagnosed as frozen shoulder or a joint problem,’ observes consultant neurologist, Dr Nicola Pavese of Imperial College London. Dr Pavese is leading the British arm of a landmark study (funded by the Michael J Fox Foundation), hunting for biomarkers – biological signatures that, like high cholesterol for heart disease, point to underlying problem.
What causes Parkinson’s?
Experts agree that it’s a mixture of nature and nurture, although the exact mix varies.
1. Genes load the gun…
We all have a one in 40 chance of developing Parkinson’s but if a parent or sibling was affected, this increases to one in 25. Five major causal genes have been identified, plus around another 25, which, together with environmental factors, increase risk. Two genes currently causing a stir are LRRK2, which causes overactivity of an enzyme in nerve cells and which has been pinpointed as the chief genetic culprit, and Parkin, the gene most often implicated in early onset forms, which controls energy production in nerve cells. Experts are currently investigating whether these could be potential biomarkers.
2. Environment pulls the trigger…
Factors linked to a higher risk:
Age (60+), gender (male), head injury, pesticide exposure, exposure to toxic chemicals, metals, solvents and certain pollutants.
Factors linked to a lower risk:
Caffeine, uric acid (found in meat), anti-inflammatory drugs, statin use and vitamin D and high levels of physical activity.
Could it be Parkinson’s?
Early diagnosis is vital and trouble moving or walking, not swinging your arm when walking, shoulder stiffness or pain, feeling frozen or rooted to the spot, small handwriting, a frozen or ‘masked’ expression that can look severe and poor posture can all point to the four motor symptoms doctors look for. These are tremor, often in the hands, legs or jaw, usually worse at rest or under stress, muscle stiffness, balance problems and slow movement (bradykinesia). However, many experts now believe that Parkinson’s begins long before these symptoms and have identified a slew of ‘non-motor’ symptoms mostly due to changes in the relevant brain centres. These include:
Loss of smell (anosmia) and taste. More than nine out of ten sufferers complain of this.
Sleep problems. Disturbed rapid eye movement (REM) or dreaming sleep is a particular problem, causing sufferers to thrash around, shout, talk or fall out of bed in their sleep.
Constipation. Thought to be a result of protein accumulation in gut nerve cells, which affects function.
A quiet, breathy, monotonous voice. 70-90 per cent of people with Parkinson’s have speech and voice changes. Altered mood, behaviour and thinking (cognition) – anxiety and depression are among early symptoms. Later, memory problems can appear.Impulsivity, obsessive compulsive behaviour – including compulsions for gambling, sex, shopping, food, eating or even hobbies are usually related to anti-Parkinson’s medications.
‘I had no idea someone as young as me could be affected by Parkinson’s’
Former sports PR, Sarah Webb, 44, runs the South London Younger Parkinson’s Network, which has already raised £130,000 to fund research.
‘When my arm began to shake as I fed my second son, Connor, now five, I put it down to tiredness. I’d just had a baby and also had a toddler Jasper, now seven, so it wasn’t surprising. It was only my mum asking, ‘What’s wrong with your arm?’ that prompted me to see the GP.
‘At first he thought I had essential tremor (ET), a benign condition also known as shaky hand syndrome. But my tremor was only in one arm, whereas ET usually affects both sides. So, after some googling I went back to the GP who referred me to a neurologist.
‘I was expecting to be told there was nothing wrong that a holiday wouldn’t cure. Neither my husband, Tim, 48, nor I, were prepared for the news that I had Parkinson’s. I burst into tears. I was just 40 and had no idea it could affect someone so young. In retrospect, there were clues back in my twenties: a shaky hand that made holding a glass of wine and a canapé difficult, stiff, achy muscles and later, when I was pregnant, dreadful fatigue. But it was easy to attribute these to other things.
‘I take a cocktail of medications, together with daily ginkgo biloba and co-enzyme Q10. I’m thinking about Deep Brain Stimulation (DBS) too, which can have excellent results, especially in younger people. There are everyday difficulties – doing up Connor and Jasper’s buttons is a real challenge and some days I really struggle to walk. I also have terrible sleep problems. Walking helps ease stiffness and I stretch every day. And I’ve also learnt to pace myself and take things a day at a time.
‘At first I had no one apart from Tim to confide in. Since helping found the South London Younger Parkinson’s Network (SLYPN) with others in the same boat, however, I’ve felt much better. There’s always someone to talk to about medication, consultants and the future – all things that have helped me get my life back on track.’
Visit slypn.org.uk to find out more.
‘I was lucky to get diagnosed quickly but it was a big shock’
Conductor James Morgan, 44, and his wife, mezzo-soprano, Juliette Pochin, 43, tell it how it is…
The weird thing is that, looking back, James had had problems for years – things like back and neck pain, which we put down to muscle strain, and vivid, almost hallucinatory, dreams that made him shout out in his sleep. We now know these were due to Parkinson’s, but at the time we had no idea.
James finally went to the doctor when he developed a tremor in his left hand. Our GP immediately referred him to a neurologist at London’s University College Hospital. Even though James suspected it was Parkinson’s, it was still a huge shock to discover that it was. We both thought it was something only old people got and had no idea it could strike someone as young as James.
As a busy singer and conductor with a busy music production company, and parents of three children, Anna, 12, and twins, Seth and Arthur, five, we didn’t know how we would manage and we did go through a grieving process. Parkinson’s makes things like turning the pages of a musical score difficult. And it also affects family life. Fiddly things, like doing up the kids’ coats, tying their laces, putting the key in the door or getting his credit card out of his wallet are difficult, and Lego and Playmobil are virtually impossible. These days it’s always me, too, who gets the roast out of the oven in case James drops it!
It took us some time to get used to James having Parkinson’s but we try not to dwell on it and get on with enjoying life.
I first noticed the tremor when I was being filmed for a live concert on Danish TV and the camera panned over my left hand, which started to shake. I’d been having pins and needles in my left arm for months but I thought it was due to sleeping awkwardly. I’d done some research and at the back of my mind I suspected Parkinson’s but I was in complete denial and it was a big shock to discover that it was.
Since being diagnosed, I’ve been prescribed a drug called rasagiline, designed for people with early Parkinson’s, which helps my movement problems. I take beta blockers to help calm me before concerts, as stress increases the tremor. And I also drink green tea, which is thought to be therapeutic, and take ginkgo biloba as well as cycling and swimming regularly, which I find extremely helpful. Cold weather and stress seem to make my symptoms worse, and being warm and relaxed improve them. However it is an odd condition and things change every day.
Luckily our GP was quick off the mark and my consultant is great but not everyone is so fortunate and it would be great to raise more awareness of young onset Parkinson’s. Last year we did a concert to raise funds for Parkinson’s UK research and show that it’s possible to continue to work and live life to the full after diagnosis – which I do.
The good news is there are tried and tested treatments. They include:
Levodopa (L-dopa), a compound the brain uses to make dopamine. Doctors are now much better at calibrating the dose and dealing with side effects.
Dopamine agonists trick the brain into thinking it’s receiving the dopamine it needs.
Deep Brain Stimulation (DBS), a device that’s implanted under the collarbone where it sends electrical impulses to the part of the brain that controls movement. ‘It improves tremor and overall mobility, especially after several years of treatment, when drugs no longer provide steady control,’says Dr Pavese.
Coping strategies include:
Exercise which can help manage symptoms and may even slow progression by encouraging new nerve cells.
Singing can help improve voice and speech problems. Lee Silverman Voice Treatment (LSVT), an intensive voice therapy programme, may also be beneficial.
Supplements are scientifically unproven yet but possibles include ginkgo biloba, creatine, a compound that boosts energy in the muscles and brain, vitamins C and E, and plant chemicals found in blueberries and other fruit and veg.
Dedicated to the cause
Actor Michael J Fox is the most famous example of young onset Parkinson’s and his optimism and the work of The Michael J Fox Foundation for Parkinson’s Research give inspiration and hope. Michael, 53, was diagnosed at the age of 30, went public seven years later and launched The Michael J Fox Foundation for Parkinson’s Research in 2000. In 2012, he spoke about finding a drug cocktail that helped control symptoms well enough to play a character with Parkinson’s in an NBC series.
Could you help research? Parkinson’s UK lists trials it funds taking part in clinical trials at parkinsons.org.uk/researchstudies.
Fox Trial Finder is a new web-based clinical trial matching tool developed by the Michael J Fox Foundation. Visit foxtrialfinder.michaeljfox.org
Parkinson’s Awareness Week: 20-26 April
Visit parkinsons.org.uk or call the Parkinson’s UK helpline 0808 800 0303 for information and support if you or a loved one are affected
For advice on living with Parkinson’s, visit our article here.