Young-onset dementia: Teen's diagnosis shows disease can strike at any time

Millions around the world live with it, but treatment can often be delayed because it is more common among older people

An illustration from the US National Institute on Ageing depicts cells in an Alzheimer’s-affected brain. The brown areas are clumps of protein that disrupt cell functions. Photo: AP
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The recent diagnosis of a 19-year-old from China with Alzheimer's disease has shattered preconceptions about a debilitating brain disorder that does not discriminate based on age.

Thought to be the youngest person ever found to have the condition, the teenager had experienced memory loss since he was 17.

He is one of the estimated 3.9 million people worldwide who, according to a 2021 study, are living with young-onset dementia, which is defined as when symptoms emerge before the age of 65.

Each year there are around 370,000 new cases of the condition.

Not just an 'old person's disease'

When Dan Jaworski was diagnosed with Alzheimer’s disease in 2019 at the age of only 49, it was “a bit of a shock”.

His family thought something was wrong because Mr Jaworski was repeating himself during a family holiday, while he later realised things were not right when he became lost travelling to a college he knew well.

Receiving a diagnosis of Alzheimer’s disease was not easy, but Mr Jaworski, who lives with his wife, Julie, in Maitland, Florida, was determined not to dwell on his circumstances.

“I didn’t stew too long, ‘OK, this is fatal to everybody who’s ever had it,’” he said. “I said, ‘This is what I have, these are my options, what can I do about this thing?’”

Dan Jaworski was diagnosed with Alzheimer's disease at 49. Photo: Alzheimer's Association

Mr Jaworski, a national early-stage adviser for the Alzheimer’s Association in the US, which supports research, early detection, risk reduction and support related to the condition, has symptoms such as struggling to remember dates. But he tries to live in the moment and to continue to enjoy life.

“What can I do to make today a great day?” said the father of two, who used to work in international investment management.

“From a practical standpoint, it’s trying to be grateful for what I have and to make the best of the day.”

Such is his enthusiasm for life that last year he took part in an Ironman competition in Hawaii, dedicating each of the 140 miles he swam, ran or cycled to someone affected by Alzheimer’s. He continues to run and hike and can drive himself.

Shock over teen diagnosis

Most cases of young-onset dementia are in people in middle age, but the condition hit the headlines recently when a young man in China was diagnosed with Alzheimer’s disease at only 19.

People diagnosed before the age of 65 face a potentially greater impact on their life expectancy than those whose symptoms emerge later. They may also experience particular difficulties in receiving the support they need.

According to Jules Knight, a consultant admiral nurse — a nurse who specialises in dementia — at Dementia UK, research suggests there are at least 70,800 adults in the UK with the condition, but the real figure is “likely to be far higher”.

“There is limited awareness of young onset dementia and how it affects people under the age of 65,” she added. “This has resulted in low service provision and a lack of specialist advice and support.

“It is vital that we develop specialist support for younger families with dementia, to enable them to live as well as possible.”

Younger people with dementia often have to give up work, which may cause financial problems, said Raymond Koopmans, a professor and elderly care physician at Radboud University Medical Centre in the Netherlands who has looked after many young-onset dementia patients.

“It is a great burden on the relationship. A great burden with, for instance, children. There can be grown-up children if you’re in your fifties,” he said.

“There’s a lot of research regarding the specific burden and it can be totally different [than it is] for people who are in their eighties.”

There are between about 14,000 and 17,000 people in The Netherlands living with young-onset dementia, and the place where Prof Koopmans works has a facility, Joachim en Anna, dedicated to their care.

Crucial delays in treatment

Prof Raymond Koopmans, a professor and elderly care physician at Radboud University Medical Centre in the Netherlands. Photo: Prof Koopmans.

Because doctors are not expecting dementia in younger patients, and because symptoms in younger people often do not involve memory loss, diagnosing people aged under 65 can be delayed.

“We know from GPs that it’s very rare for them,” Prof Koopmans said. “That’s one of the reasons why there’s a huge diagnostic delay, because these people with cognitive symptoms, they live at home, they’re still working.

“Maybe the first expression are often changes in personality, changes in behaviour, which often leads to what we call misdiagnosis.

“The GP is not specifically to be blamed because they don’t think of dementia. This diagnostic delay is a very important topic for research and improving the care and recognition.”

Research in The Netherlands, Australia and Norway indicates, he said, that the diagnostic delay can be around 5.5 years or longer, depending in part on the type of dementia.

“That’s two to three years longer than for people with later-onset dementia,” he said. “They think it’s depression, so they send them to a psychiatrist, or [GPs think it is] burnout when there are problems at work.

“We know from research that as there’s a long diagnostic delay, sometimes receiving the diagnosis is a relief. It’s, 'Oh, now I know what’s wrong with me or what’s wrong with my husband or my wife'.”

The diagnosis can, he said, be the starting point for specially tailored support, which may include assistance for family members if they become caregivers.

“Ultimately there has to be professional help,” he said, with some people ultimately moving to a specialised facility because their spouse cannot cope any more, while in other instances the support available allows the patient to remain at home.

Among elderly people with dementia, about two in three have Alzheimer’s disease, but Alzheimer’s accounts for a lower proportion of dementia cases that develop before the age of 65.

With young-onset Alzheimer’s, memory loss is less likely to be the first symptom, according to the Alzheimer’s Society in the UK.

These “atypical” forms of Alzheimer’s may instead first emerge as difficulties in understanding visual information, which may cause problems reading, or there could be speech issues, or difficulties in planning and making decisions.

A rare form of Alzheimer’s, accounting for about 1 per cent of cases, is familial, which typically develops between the ages of 30 and 60.

This is associated with mutations of three genes, presenelin 1, presenelin 2 and amyloid precursor protein.

Another form of dementia that accounts for some of the cases in younger people is vascular dementia, where there is damage to blood vessels supplying the brain.

Among numerous other types, there is also frontotemporal dementia, which, unusually, is most often diagnosed in patients aged 45 to 65. The family of the American actor Bruce Willis, who is 67, recently announced that he had the condition.

There are many reasons why some people develop dementia at a younger age. Indeed Prof Koopmans said that a review that he was involved with found as many as 250 potential causes, some existing at birth, such as metabolic disorders.

“In general you can say there’s a larger chance of a genetic cause in young-onset dementia than in late-onset dementia,” Prof Koopmans said.

“The research community is still searching for more specific genetic causes and genes that are affected.”

Genetic testing is sometimes available for people who, because of their family history, are at high risk of inheriting dementia, although organisations warn that it can be difficult if a person finds out that they are very likely to develop dementia. Testing may also be carried out with people who already have a diagnosis.

Mr Jaworski is trying to keep his condition at bay for as long as possible by following a healthy diet and exercising.

He has check-ups each year — less often than he thought would be the case — and so far has been told that if the disease is progressing, it is doing so slowly. This means he should have many more years of life to enjoy and he is determined to make the best of them.

“There’s a gift that comes from knowing the sand is running out of the hour glass,” he said. “You celebrate and value things more.”

Updated: March 13, 2023, 7:57 AM