Understanding Early Onset Alzheimer's Disease

Article Highlights

• Early-onset Alzheimer’s disease is Alzheimer’s disease diagnosed before the age of 65.
• Early-onset Alzheimer’s is more often genetically related and includes a more rapid onset of symptoms than late-onset Alzheimer’s disease.
• Compared to late-onset individuals, those diagnosed with early-onset Alzheimer’s are more likely to experience depression or other behavioral symptoms such as anxiety before memory issues.
• Early-onset Alzheimer’s disease can devastate financial planning as people who develop the disease at such an early age are generally enrolled in an insurance not intended for elder care.
• There is a cultural stigma around early-onset Alzheimer’s, therefore spreading awareness about the disease is vital.
• The average life expectancy after diagnosis is 8 to 10 years.
• Early-onset Alzheimer’s has no cure but researchers are exploring potential treatments such as gene therapy and lifestyle therapy.

Overview

There is a misconception that Alzheimer’s disease only affects older adults (age 65 and over). However, there are currently about 200,000 people in the U.S. who have developed Alzheimer’s disease at a much younger age, many in their 40’s and 50’s. When people develop Alzheimer’s before age 65, the disease is referred to as early-onset Alzheimer’s disease (EOAD) or younger onset Alzheimer’s disease. While late-onset Alzheimer’s disease (LOAD) is devastating to millions of families, there are unique challenges for those who develop the early-onset form of the disease.

How Early-Onset Alzheimer’s Disease is Different from Late-Onset Alzheimer’s Disease

The obvious reason that early-onset differs from late-onset is the age when individuals first show signs of the disease. Not so obvious is the fact that this age of onset can be determined by one’s genes, or rather mutations of particular genes. While there are some genes that can put someone at a higher risk for late-onset Alzheimer’s disease, most cases of Alzheimer’s disease that occur in the later years seem to be due to a gradual accumulation of age-related malfunctions (plaques and tangles in the brain). However, early-onset Alzheimer’s is more likely to be genetically related and begins at a younger age.

Additionally, while there is some disagreement about the onset of symptoms, some people with early-onset tend to exhibit more a rapid onset of symptoms than those with late-onset Alzheimer’s. Researchers feel that with both types of Alzheimer’s the disease has been developing for years – even decades – prior to symptoms.

Some studies have noted that the pathological hallmarks (protein deposits called plaques and tangles) are more severe in early-onset AD than in late-onset AD and may be concentrated in different regions of the brain. These differences in underlying brain structure suggest that treatments may overlap with, but not always be identical to, treatments being developed for late-onset AD.

Causes of Early-Onset Alzheimer’s Disease

Some people who develop early-onset Alzheimer’s carry a genetic mutation that puts them at high risk for the disease. Those with familial Alzheimer’s Disease (eFAD) are likely to have a parent or grandparent who also had the disease at a younger age. Early-onset Alzheimer’s that runs in families is associated with 3 genes that can have mutations: amyloid precursor protein (APP), presenilin-1 (PSEN 1) and presenilin-2 (PSEN 2). This is different from late onset Alzheimer’s, for which the APOE gene is the primary gene known to increase risk of the disease. People with familial Alzheimer’s disease genes in their family have about a 50 percent chance of developing the disease. There are estimated to be approximately 500 families worldwide with these genetic mutations, and they seem to account for less than 1% of all Alzheimer’s cases.

It is thought that the most common causes of early-onset Alzheimer’s are the same progressive diseases that cause most cases of dementia in older adults. While the disease does not always discriminate, research suggests that healthy diet, physical activity, and social engagement may help reduce risk of developing of dementia. See our article on Alzheimer’s risks, causes, and prevention for more information.

Who is at Risk for Early-Onset Alzheimer’s Disease?

Age and lifestyle are considered risk factors for developing Alzheimer’s, and lifestyle may contribute to early-onset Alzheimer’s, as well. However, genetic influences are more likely to play a role in early-onset than late-onset Alzheimer’s. As mentioned, familial Alzheimer’s (eFAD) is almost entirely gene related.

Early-onset Alzheimer’s has been seen in people under 30, but is more common as people enter middle age. People with familial Alzheimer’s may begin to see changes in their 40s and 50s, though occasionally symptoms can even appear in their 30s. The earlier the symptoms of dementia start, the more likely the disease is genetic.

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What are the Symptoms of Early-Onset & Are They Different from Late-Onset?

As with most Alzheimer’s information, opinions change almost daily as new research surfaces. However, in one interesting study, Researchers at University College London (UCL) studied 7,815 people who have been diagnosed with Alzheimer’s. They wanted to determine if early symptoms of Alzheimer’s differed according to the age of onset.

The average age of people in the studied group was 75, with the youngest person being age 36, and the oldest 110. The researchers kept records of symptoms starting with the first probable symptom that made them wonder about the possibility of dementia.

The scientists found that, when they looked at both thinking skills and behavioral problems, the younger individuals were more likely to experience depression or other behavioral symptoms such as anxiety before they experienced memory issues. Older individuals with Alzheimer’s more often showed memory loss as their first symptom.

Alzheimer’s affects everyone differently, so there is no absolute when it comes to early symptoms. However, below are common symptoms seen in early onset Alzheimer’s:

• Forgetting important information, especially newly learned information
• Repeatedly asking for the same information
• Difficulty following instructions or solving problems
• Losing track of the date or time of the year
• Forgetting where they are and how they got there
• Difficulties with depth perception and other visual problems
• Trouble with finding the right words
• Exhibiting poor judgment
• Misplacing things and being unable to find them later
• Withdrawing from work and social situations
• Mood and personality changes

As the disease progresses, they may also experience more severe mood swings and behavior changes, worsening confusion, suspicions or paranoia, trouble speaking, swallowing, or walking, and severe memory loss. It is important to remember that everyone’s trajectory will be slightly different. There is a saying that when you know one person with Alzheimer’s you know one person with Alzheimer’s. This statement is true, regardless of age at onset.

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What it’s like for a family when a spouse is diagnosed:

Carlen Maddux, author of “A Path Revealed: How Hope, Love and Joy Found Us Deep in a Maze Called Alzheimer’s,” explains how his family’s now 17-year-old journey with EOAD began:

“September 23, 1997. The date is seared in my mind. That’s the day for our follow-up visit with the doctor who had run some tests on my wife Martha. “The previous 18 months had been tough on her. Martha had run for the Florida State Legislature as a favored candidate and lost by 20 votes. Then there was the car accident, in which she bumped her head. And other stressful issues related to her health. I don’t remember looking into Martha’s eyes during those two long weeks. I was afraid of what I might find.

“Finally, the follow-up day arrived. We were surprised by the nurse, who said, “I’m sorry, but the doctor had an emergency and was called away. One of his associates will be meeting with you.”

“This associate, stiff and formal, invited us in and sat behind his big desk, waving us to seats opposite him. I don’t remember if we even shook hands. Social skills apparently weren’t a priority for him. He could have been a perfect stand-in for Mr. Spock on Star Trek. “Could this be starting off any worse?” I wondered. I found out soon enough.

“With no introduction, the doctor looked straight at Martha and said in a calm, ice-cold voice, “I’m sorry to tell you this, but it appears likely that you have early onset Alzheimer’s disease.” “We looked at each other in pained bewilderment. Martha’s bright blue eyes instantly dulled. She seemed to have retreated into her shadow. Our world wasn’t turned upside down. It was imploding before our eyes.

“This can’t be happening to Martha,” I thought. “Isn’t this disease something you worry about when you get old?” Martha had turned 50 only twenty days earlier. “We felt trapped in a dark place with no way out.

A career journalist, Carlen Maddux’s blog is at www.carlenmaddux.com.

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Early-Onset Alzheimer’s Disease Can Devastate Financial Planning

All types of Alzheimer’s disease are expensive. Unlike many diseases which are expensive because of treatments and drugs that are at least partially covered for most people’s insurance, Alzheimer’s disease can severely impact the ability to complete daily tasks. People need increasing amounts of support and care as the disease progresses. This type of care is rarely covered by any insurance.

A hallmark of evaluating Alzheimer’s care needs is the determination of how people can complete their activities of daily living (ADLs). There are six basic categories of ADLs: eating, bathing, dressing, toileting, transferring/walking and continence. These have been argued to be vital abilities for someone to care for oneself and live independently. When these abilities are lost, through either a rapid increase in symptoms or a slow but steady decline, the person must have increased care. Ultimately, the final stages of Alzheimer’s typically require around-the-clock care.

People with good long-term care (LTC) insurance can often receive reimbursement from the policy for in-home caregivers, but most other families are left to their own devices. Since people with early-onset Alzheimer’s are generally enrolled in an employer’s insurance, or have policies not intended for “elder care,” they rarely have a rider that would cover this type of long-term care.

Additionally, the person affected by the disease must eventually stop working because the disease makes it impossible to perform his or her job. The spouse often has to cut down to part-time or quit working entirely to provide care. These changes can take a significant financial toll on families and individuals affected by Alzheimer’s disease. Additionally, in the case of early-onset, individuals may be caregivers themselves for older family members or for children who are still living at home.

Our Financial Future Is Horrifying!

One man’s thoughts on the financial devastation his family experienced:

Alzheimer’s disease can devastate the most well thought out financial plans. After my diagnosis, I had to retire early, which reduced my income to almost nothing. My wife had to sell off most of our assets, including our home, and use our life savings just to make ends meet. She’d had a part-time job but had to quit that, although she has been able to make some money working from home. Our financial future – and eventually hers alone – looks scary. So there’s a devastating financial cost that comes with EOAD besides what happens emotionally.

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Cultural Influences on How Dementia is Viewed by the Public

There is a cultural stigma around Alzheimer’s that likely stems from fear. Historically, any disease that affects the brain seems harder to understand and digest for those not affected by it.

What people can’t understand, they often fear. With early-onset dementia, there is added fear when the reality that this aging-related disease actually affects younger people becomes apparent. People don’t know how to treat their co-worker who can no longer perform the fundamentals of a job that he or she did well for a decade. Worse, the individual who has developed early-onset Alzheimer’s may not be able to recall the names of good friends and even family members. These changes are frightening to comprehend so, to many, it seems easier to ignore them, which is why dementia diagnoses are so often associated with social isolation and loneliness. Due to their younger age, people may not believe they have the disease or question their diagnosis. These reactions can be hurtful and damaging.

Thankfully, there have been some breakthroughs in educating the public. Smart television and movie producers are seeing that if they present a dramatic story in an interesting way, the story will draw a crowd. Perhaps the most well known in this area is the movie “Still Alice”. The book came first, as is often the case, but the movie brought early-onset Alzheimer’s into mainstream consciousness.

Additionally, sports and media elites who’ve developed dementia have gone public with their disease in the interest of education. When Pat Summitt, famed University of Tennessee basketball coach, developed Alzheimer’s at age 59, she became an important voice in the field of AD. People watched her courageous battle with heartbreak but admiration. Summitt died at age 64 but her legacy lives on through the Pat Summitt Foundation.

There are some caregivers who feel that movies like “Still Alice,” as well as wealthy celebrities, can harm the cause as much or more than they help. These people feel that the reality of a struggling family who can’t pay the bills and must try to provide care 24-hours a day with no respite is not represented. While these are valid criticisms, increasing public awareness and education about Alzheimer’s disease is critical. Thus, bringing Alzheimer’s into mainstream is an important first step.

Ask an Expert

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Can I inherit EOAD from my parent who had it?

How It Feels to Live with Early Onset Alzheimer’s Disease

The only people who can say what it’s like to live with early onset dementia are those who live with it. Harry Urban is one of those people. With his permission, here are two of his recent blog posts to provide insight into his experiences:

December 2016

Does it hurt, I’m asked? Does what hurt, I answer back; living with Alzheimer’s, they say. No, there is no pain unless you factor in the emotional pain that is caused to the people that watched me during my decline.

I suffer through the pain in what they feel. I see the grief in their eyes and can’t help feeling the pain because I am causing it. I don’t think living with dementia would be so hard if it did not affect our caregivers the way it does.

I have control over it, I wish they would learn how to also.

January 2017

When you are caring for someone living with dementia, are you bringing HOPE back into their life or just taking care of their daily needs? Do you talk to them and get to know this new person? You may know the old person very well but unfortunately that person is being left behind and someone new is being born.

We often take for granted we know and understand the person, only to find out that this person we are caring for is so different. The person we knew may have been kind and caring but this person is not.

Something happened and are you trying to figure out what it is. Finding out what the problem is may return a part of the old person you love.

Life expectancy with EOAD

Life expectancy is different for everyone who develops any type of Alzheimer’s. The average life expectancy after diagnosis is eight to 10 years. In some cases, it can be as short as three years or as long as 20 years.

In general, individuals with early-onset Alzheimer’s lose 15 to 18 years of average life expectancy.

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The Future of EOAD

There is currently no cure for early or late onset Alzheimer’s disease. Drugs have so far failed to provide much hope, but they may provide temporary help with some symptoms. These include medications such as Donepezil, Rivastigmine, Galantamine, and Memantine. Results are mixed, but these medications seem to help alleviate symptoms in some individuals for some time, from a few months to a few years. While there is currently no cure, scientists are working hard to discover methods to prevent or reverse the disease. Some researchers are investigating the role of gut bacteria in the development of the disease. Some are digging into how the body uses nutrients. Others are looking at inflammation processes which seem to play an important role.

Two promising areas of research exploration in coming years:

Gene Therapy

This is a treatment that delivers a modified virus to a gene in the brain that could theoretically erase the damage being done by developing Alzheimer’s before any symptoms become evident. The virus, which is called a lentivirus vector, is already used in gene therapy for other illnesses. Researchers from Imperial College London have shown how using this modified virus to deliver a gene, known as PGC1-alpha, to the brain cells of mice destroys the progression of AD. However, studies using mice may not always translate to humans, so more research and clinical trials are needed.

Lifestyle Therapy

Through the combined efforts of the Buck Institute for Research on Aging and UCLA Easton Laboratories for Neurodegenerative Disease Research a lifestyle therapy is being tested for the long-term. The program is called metabolic enhancement for neurodegeneration (MEND) and involves a combination of lifestyle changes, brain stimulation, and medication. Their pilot study showed some reversal of memory loss in people with mild cognitive impairment. While their findings are promising, they were based on a small group of 10 patients with memory loss and further clinical studies are needed.

Final Thoughts

There is still much work to do before people who are genetically at risk of developing early-onset Alzheimer’s can feel at peace with their future. Funding for Alzheimer’s research is lagging behind that of cancer and other diseases, yet with the aging of our population, increasing numbers of individuals and families will be affected. Funding for caregiver support and policies to help caregivers like paid leave from work are also needed. Fortunately, some big companies like Deloitte are beginning to recognize this important need. Hopefully, increasing numbers of companies and workplaces will follow their lead. Alzheimer’s is hard, and it can be even harder to deal with the unique challenges and timeline of early-onset. It is important to remember you are not alone. Many resources, such as support groups for caregivers and/or for people diagnosed with early-onset, are available, online and in your communities. Be sure to check with your doctor and your local branch of the Alzheimer’s Association or similar foundations.

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