You told your husband you had to work late tonight, but he says you never did. It’s not the first time he didn’t seem to hear something you said, and you feel a twinge of concern. He used to be a better listener. He’s only 50, but is it possible that there’s something wrong with his memory?
Early-onset dementia is generally defined as dementia before age 65. Because dementia usually appears in older adults, it’s easy to write off signs and symptoms when they show up in middle-age — especially since occasional forgetfulness is common and expected.
How do you know when forgetfulness is a regular part of life, and when it’s a problem? Richard J. Caselli, M.D., a Mayo Clinic neurologist with expertise in cognitive aging and Alzheimer’s disease, answers our questions about early-onset dementia.
When is a moment of forgetfulness a fluke, and when does it point to something like early-onset dementia?
It’s obviously a gray area, but a good gauge is whether there’s a change in pattern. We all forget things from time to time, whether it’s from distraction, we didn’t hear it in the first place, or we weren’t paying attention. But when people can’t do tasks they used to be able to do, especially when the circumstances really haven’t changed — that’s a concern. For example, the person who suddenly is trying to use a cellphone as the remote control for the TV.
A characteristic experience in younger people with dementia is having trouble at work. In one common scenario, a person has been in a job for a while, and now their performance is declining. Maybe they get a bad report from their supervisor. Now, it’s a question: Is this person just getting flaky on the job or do they have a brain disease leading to their impaired performance?
A second scenario is trouble adapting after changing jobs or being introduced to new technology. These people have never had a problem with a different job, or a new bit of software or spreadsheet program, but now they do. Those concerns would be the not-yet-retired person’s chief complaints that will bring them in to see me at a very early stage.
Sometimes somebody makes an error that’s just so egregious, there’s no way it could have just been a question of “I just didn’t hear.” For instance, they lost their car in the parking lot, and they have to have security come and help them find it. I think we’ve all had trouble in big parking lots where we think, “Where the heck did I park?” But usually, we can figure it out eventually.
Context is also important. The busy parent with four kids trying to get one to soccer who rushes home to drop off the groceries, drops the keys in the refrigerator and can’t find them — that’s not as concerning. But somebody who is putting their wallet and keys in the refrigerator and then sitting down to watch TV would be a problem. Usually, that’s not happening early on in dementia.
If a younger person is experiencing these symptoms, why can it be hard to get a primary care doctor to take them seriously?
If a relatively young person who seems to be fairly well functioning — meaning they’re conversing well and don’t look disheveled — says to the doctor, “I feel like there’s something wrong with me” — well, the most common causes of that are anxiety and depression. Even in my practice, if we’re reviewing appointment requests in our department, and I see a request from a 49-year-old man, I may want more records before seeing him. That’s because 9 times out of 10, that person is going to be somebody with anxiety or bipolar disorder, or some other more common condition.
It can be difficult even for a specialist to diagnose early-onset dementia. If we conduct a mental status, or memory, exam in the office, the person might seem OK. They can probably easily answer questions like “Who is the president?” They might have a little trouble with remembering, say, three or four words, but maybe it’s still not clear that there’s something wrong.
So, I think a major imperative on those of us evaluating these people is to have a low threshold for looking further, not just say, “Well, you pass your mental status exam, you must be fine.” Sometimes we need to say “You pass my office exam, but maybe we should look further and get more detailed neuropsychological testing” — which would be a lot more challenging and a lot more appropriate for a younger person.
It can also be helpful for someone who knows the person well to provide information. A spouse might say, “Yeah, I’ve seen a change. It’s not all the time, but there are times when I’ve said something to him, but he seems like he doesn’t realize I ever said it. I don’t know if he’s just not listening or he’s forgetting.”
There are also some conditions that can cause symptoms that look like dementia. For example, a stroke can lead to someone quickly becoming impaired. Another cause is delirium. Delirium can evolve quickly — for example, from a rapidly spreading infection — and can also evolve more slowly. For example, someone taking a lot of different medications can develop chronic delirium that may resemble dementia.
What are the common causes of dementia in younger people?
Dementia is not the name of a disease, it’s really just a description of a set of symptoms. It describes people who have enough cognitive impairment to the point where they can no longer function independently. It’s become somewhat synonymous with Alzheimer’s disease. But Alzheimer’s disease itself is a specific disease that is only one cause of dementia. There are other degenerative diseases that cause dementia, for example, dementia with Lewy bodies, frontotemporal lobar degeneration, vascular problems, and so on.
When we see somebody with early-onset dementia, say at age 45 or so, we have to be concerned about a genetic cause known as a dominantly inherited mutation, or gene change. Just one copy of a dominantly inherited gene change from either parent will cause early-onset Alzheimer’s. It’s rare. But because of that small percentage of people who have the dominantly inherited gene change, we offer the option of genetic testing. Why? Well, it impacts their first-degree relatives, most notably their children. If their children have a 50% chance of inheriting this gene, they may decide they want to be tested. If you have that gene, maybe you decide you don’t want to have children, maybe you decide you’re not going to spend half your life in medical school becoming a neurosurgeon. There are important life decisions that it would impact.
If you’re diagnosed with early-onset dementia, what treatment options are there?
Two broad treatment categories of treatment in medicine are disease-modifying and symptomatic. The example I give is to imagine somebody with a brain tumor with a headache. I can give them some aspirin for the headache, but it’s not going to take away the brain tumor. That’s symptomatic treatment. Now, I’m going to give them chemotherapy to shrink the tumor. That’s disease-modifying.
For dementia, what we’re really offering people is symptomatic therapy and lifestyle modification. In terms of symptomatic therapy, there are different categories of symptoms in those with Alzheimer’s and related disorders. Intellectual decline is, of course, what everybody thinks about: memory loss, trouble with names, losing your way. But another category is behavioral problems such as paranoia, personality changes, hallucinations, depression, anxiety, and so on. Those are treated directly, not just with the medications we use to try to help people’s memory.
Along with treatment, what other things are there to consider after a diagnosis of early-onset dementia?
So No. 1, there are economic implications. If the person diagnosed is the primary breadwinner, we’ve got a real problem. If they have a partner who was the breadwinner, that partner now also becomes a caregiver. That means both partners are unable to work in the same way they did prior to the diagnosis.
A second aspect is caregiving for the patient while still caring for young children. With young-onset dementia, you may have children in school who have their own needs and a limited capacity to help, as opposed to the retiree who’s got adult children.
Can you offer hope to someone with early-onset dementia?
As doctors, we want to be somebody that our patients can come to for their needs, and sometimes those needs are emotional. One of the really important aspects of the job is to try and be a source of hope and comfort for people with problems even when you can’t fix everything.
To learn more about programs and support available for those with early-onset dementia, check out this resource list by the National Institute on Aging as well as the Alzheimer’s Association website www.alz.org.