When Dementia Strikes at an Early Age

People aren’t too worried when an octogenarian sometimes forgets the best route to his favorite store, can’t remember a friend’s name, or dents his car trying to park on a busy city street. Over the years, even healthy brains function less efficiently and memory, sensory perceptions and physical abilities become less reliable.

But what if the person is not in their 80s, but in their 30s, 40s or 50s and forgets the way home even though they are on the corner? There the situation is much more worrying. Even though most of the 5.3 million Americans living with Alzheimer’s or other forms of dementia are over age 65, about 200,000 are younger and develop serious memory and thinking problems much sooner than expected. .

“Early-onset dementia is a particularly bleak diagnosis because it affects individuals who are in their prime,” wrote Dr. David S. Knopman, a neurologist at the Mayo Clinic in Rochester, Minnesota, in the July 2021 editorial of the scientific journal JAMA Neurology. Many of those affected are in their 40s and 50s, mid-career, ill-prepared for retirement and perhaps still raising children.

Dementia in young adults is especially traumatic and challenging for families to recognize, and many doctors fail to identify it.

“Complaints about brain fog in young patients are very common and generally harmless,” Knopman said. “It’s hard to know when they’re not attributable to stress, depression or anxiety, or a result of normal aging. It is rare that even neurologists identify early dementia early on.”

However, recent studies indicate that the problem is much more common than most doctors realize. Worldwide, up to 3.9 million people under the age of 65 could be affected, a Dutch analysis of 74 surveys indicated. The study, published in JAMA Neurology in September, revealed that for every 100,000 people aged between 30 and 64, 119 had early-onset dementia.

Knopman’s editorial called early-onset dementia “an underappreciated problem.” His diagnosis, he wrote, is often late, and knowledge about his management is also “scarce.”

The various causes of early dementia

The Dutch study found that, in general, the Alzheimer’s disease was the most common cause of dementia praecox. But when symptoms developed before age 50, early-onset Alzheimer’s disease was a less likely explanation than two other causes: vascular dementia and frontotemporal dementia.

Vascular dementia results from a blockage or damage to blood vessels in the brain, interfering with circulation and depriving the brain of oxygen and nutrients. In addition to memory problems, its most common symptoms are confusion, difficulty concentrating, slow thinking, and difficulty organizing thoughts or tasks.

In frontotemporal dementia, the parts of the brain behind the forehead and ears shrink, resulting in dramatic personality changes, socially inappropriate or impulsive behavior, and emotional indifference. Movement and memory problems often develop later in the course of the disease.

According to the Mayo Clinic, frontotemporal dementia usually starts between the ages of 40 and 65 and can be misdiagnosed as a psychiatric problem.

Dementia with Lewy bodies is another cause of dementia in younger adults. It is associated with abnormal deposits of a protein called alpha-synuclein, which affects brain chemistry and leads to behavioral, thinking and movement problems. Most symptoms are similar to those seen in other dementias, and additional symptoms, such as hallucinations, may resemble schizophrenia, but the decline in brain function occurs significantly faster. A hallmark symptom of dementia with Lewy bodies is having violent dreams and trying to fulfill them, Knopman said.

Alzheimer’s remains the most common cause of dementia in both younger and older adults. There is an inherited form of Alzheimer’s that typically appears at younger ages, but these cases account for less than 10% of early disease. Most cases of Alzheimer’s occur sporadically, for unknown reasons, although genetic factors may increase the risk.

People with Alzheimer’s typically have a buildup of abnormal substances — tau proteins and beta-amyloid — in the brain. Early symptoms include impaired memory, language problems, difficulty concentrating and completing tasks, blurred judgment, and visual or spatial deficits that result in errors at the wheel and loss of sense of location. Brain scans can show a loss of brain cells and an impaired ability to metabolize glucose, which is indicative of degenerative brain disease.

Probably the factor best known for increasing the risk of early dementia is repeated head injuries, such as those experienced by professional boxers, football and football players, and sometimes military veterans.

Once brain cells are damaged or killed, there is no turning back. So for now the best protection possible is to prevent head injuries.

Many parents these days try to discourage young people from playing sports like football, where repeated head injuries are common. However, proper and consistent wearing of helmets and not heading the ball in soccer can limit the risk of head injuries. Knopman said he is less concerned about elementary school children playing these sports: The risk of developing early-onset dementia from repeated head trauma is much higher among those who played college football or became professional boxers.

Among the elderly in general, the same inflammatory factors associated with atherosclerosis, the clogging and hardening of the arteries that nourish the heart, can also affect the blood vessels that supply the brain. Body-wide inflammation linked to diabetes and heart disease can also cause brain changes that lead to dementia.

Early diagnosis of dementia

Accurately diagnosing early-onset dementia can be difficult and time-consuming and should start with a detailed medical history, Knopman said. “If doctors don’t ask the right questions, families may fail to mention a telltale symptom, such as violent dreams.”

A thorough cognitive assessment of the person’s memory and language difficulties is also critical, he said. Does the person tangle with words or does he say “white” when he means “black”? Neuropsychological tests can detect subtle difficulties with memory, visual and cognitive functions.

Tests are needed to rule out the possibility that a brain tumor is causing the person’s cognitive symptoms. A spinal tap and spinal fluid analysis can reveal elevated levels of tau and beta-amyloid proteins in the brain. An MRI can identify the shrinkage of specific parts of the brain. And a glucose PET scan can find abnormal patterns of sugar uptake in various parts of the brain, which can help distinguish between Alzheimer’s disease, dementia with Lewy bodies and frontotemporal dementia. “Different degenerative brain diseases have specific patterns of glucose uptake,” Knopman said.

As with older dementia patients, keeping young adults with dementia safe is essential. They must no longer drive, operate hazardous equipment, including the stove, or be left unattended. Everyone must wear an identification tag day and night alerting others to their condition.

/ TRANSLATION BY RENATO PRELORENTZOU

About Abhishek Pratap

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