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Digging into Dementia

When someone hears the word ‘dementia’, they automatically think Alzheimer’s disease. But there are dozens of conditions that can cause dementia, and it doesn’t just affect the elderly.

Hear more about dementia from Dr. Erin Foff, an expert at UVA’s Memory and Aging Care Clinic.
Digging into Dementia
Featured Speaker:
Erin Foff, MD, PhD
Erin Foff is an Assistant Professor of Neurology. She received her Ph.D. in Molecular and Human Genetics from Baylor College of Medicine (Houston, TX) and her medical degree from the Jefferson Medical College in Philadelphia, PA. Following neurology residency at the University of Virginia, she completed a research fellowship in nucleotide repeat disorder biology at the University of Virginia. She has a basic science laboratory where her team conducts research on several nucleotide repeat disorders, including frontotemporal dementia/ALS and myotonic muscular dystrophy. Dr. Foff sees patients with neurodegenerative diseases causing cognitive complaints, and she specializes clinically in early onset dementias, frontotemporal dementia and the primary progressive aphasias. She lives in Charlottesville with her husband and two children.

Learn more about Erin Foff, MD, PhD

Learn more about UVA Health System
Transcription:

Melanie Cole (Host): When someone hears the word dementia, they automatically think Alzheimer’s disease but there are dozens of conditions that can cause dementia, and it doesn’t just affect the elderly. My guest today is Dr. Erin Foff. She’s a neurologist and a memory disorder specialist at UVA Health Systems. Welcome to the show, Dr. Foff. As I said in the intro, when someone hears that word, they automatically think Alzheimer’s but tell us about some of the other conditions that can cause dementia.

Dr. Erin Foff (Guest): Absolutely. You know, that’s one of the most common misconceptions that patients and their families come into our clinic harboring. So, dementia is actually just a description of the severity of a cognitive problem. And many, in fact dozens, of disorders can cause dementia. Alzheimer’s is really only one of them. Other disorders--other neurodegenerative disorders that can cause dementia include such diseases as dementia with Lewy bodies which many of the listeners may be familiar with because it is the disorder that Robin Williams recently passed away from, as well as frontotemporal dementia which we’ll discuss a little bit today; vascular dementia which is even more of a problem in our society with many vascular risk factors that our patients suffer with; and then, other non-neurodegenerative diseases like autoimmune diseases that lead to cognitive complaints, and also mood disturbances.

Melanie: So, people also usually think that dementia only happens to older people but some of these conditions, as you mentioned Robin Williams, happen to younger people. So that’s true, correct? And what are some of the conditions that might happen to a younger person?

Dr. Foff: That’s exactly right. So, oftentimes in our clinic, we are stratifying people in terms of their risk of a certain type of dementia based on their age. So, Alzheimer’s dementia tends to strike mostly folks who are above age 65, most commonly in 70s and 80s, and, in fact, by the time people reach their mid-80s, they’re approaching about 50% risk of developing Alzheimer’s disease. But, some of the disorders that can strike young folks in their 40s, 50s and 60s, include diseases like the frontotemporal dementias which is a class of diseases that tends to strike young. There is a form of Alzheimer’s called “early onset Alzheimer’s disease” that strikes, again, before age 65. And, many of the non-neurodegenerative dementias, those associated with severe depression or autoimmune disease, can also strike folks that are much younger than you would typically expect for a dementing illness.

Melanie: Dr. Foff, what are some of the red flags that people--they hear about leaving their keys somewhere or not knowing where they’re going, but what are some of the ones for loved ones that they might notice in somebody that would signal the onset of one of these dementias?

Dr. Foff: That’s a really great question. So, typically, it’s actually not the patient that recognizes there may be a problem, but oftentimes their family or another one of their healthcare providers like their primary care physician. So, it’s true that we do lose some mental flexibility as we age but when family members start to indicate that somebody is repeating their sentences or questions over numerous times, if there are severe or unusual personality or mood changes, if there is any evidence of weakness or changes in the sensory system of the body, those can all be red flags--headaches, etc. So, we typically encourage people, if anybody has expressed concern that perhaps their slight memory problems seem out of proportion that they should at least have a quick screening exam with their primary care doctor, and if it looks like there may be more severe problems, having a referral to a neurologist.

Melanie: Is there a screening? I’m glad that you mentioned that because, is there a way to diagnose it for sure? Is it based just on history or would something like a CAT scan or an MRI show you anything that would tell you that that person is in cognitive decline?

Dr. Foff: So that’s great question, and that really is dependent on the reason for the cognitive decline. So, certainly, a primary care physician or a general neurologist can do the initial workup that can include some screening measures that are usually validated mostly for older patients. But, if there is a concern, we can do more in-depth cognitive testing. This is something called “neuropsychological testing” which is looking at all of the cognitive domains for evidence of abnormalities. And, in addition to standard tests like structural imaging or MRIs, looking for shrinkage of the brain or lesions in the brain, we can also do some very advanced testing, actually looking specifically for the presence of the Alzheimer’s protein or other patterns that indicate the brain is not functioning normally, and that can help a lot with diagnosis. There are now also more sophisticated biomarker tests which is where we analyze spinal fluid on patients to look for evidence that there is breakdown of brain cells and, in a particular pattern, consistent with certain diseases. So, we are much more accurate in our diagnosis now than we ever were before.

Melanie: You’re among a select group of researchers worldwide trying to find the genetic defects behind frontotemporal dementia. Tell us about the work that you’re doing in the lab, and how it might one day benefit these families.

Dr. Foff: That’s right. So, I work on a particular form of frontotemporal dementia that is associated with one underlying genetic defect, and it’s the most common cause of inherited frontotemporal dementia. It is also linked and causes many cases of familial or inherited ALS which many people know by the other name, Lou Gehrig’s disease. And so, typically in the past, people have studied mouse models or animal models in an effort to understand how these diseases progress and to develop therapies targeting those processes. We take a different approach in our lab using stem cells created directly from patients, where we are able to take patients skin cells from an adult living patient, turn them into a stem cell which is a cell that can become anything in the body, and then further make that stem cell into brain cells in a dish, and we have created very complex models of the brain in three dimensions from those patient cells so that we can study them and understand what’s going wrong, and hopefully develop drugs that can target that process.

Melanie: That’s absolutely fascinating and so well spoken. You explained that just perfectly for the listeners, Dr. Foff. Now, tell us what you tell families when they do get diagnosed--someone that they love. What do you tell them about available treatments or lack thereof or lifestyle things that they can do to at least help this person while they’re going through this?

Dr. Foff: That’s such a critical part of the process. So, many people are aware of the fact that we just don’t have a cure for any of these neurodegenerative diseases although we really hope, as a community, that we’re headed there soon. But, that doesn’t mean that our process stops at the diagnosis. So, we are very interested in pursuing what we call a “multidisciplinary approach” for every single patient. So we try to provide support along every line of the patient’s process and journey, and this includes symptom management--since many of these diseases come with lots of symptoms in terms of mood or physical symptoms--as well as providing support for the family, and access to community resources, palliative services when necessary, and then, importantly, clinical trials when patients are interested in pursuing that. For many of these disorders, we have national clinical trial networks and after we identify patients that are interested in participating in those trials, we can help direct them to the appropriate trial, which oftentimes is testing a new drug therapy that we hope will be that future wonderful drug that can slow or prevent these diseases from getting worse.

Melanie: So, tell families that are facing dementia what they should think about if they want to consider a clinical trial.

Dr. Foff: That’s right. So, the first thing is to be referred to a center that does clinical trials. That’s very, very important. And then, once there, the neurologist or the care team can help decide the appropriate diagnosis because that determines the clinical trial, and then can lead the patient and the family through their available options. Clinical trials are labor intensive for both the providers and the patients and their families and understanding fully exactly what being in a trial entails is really important. We also make a big push to make sure that patients and their families know that, in addition to the clinical trials, there are things that the patient should be doing at home to slow decline and that includes regular, vigorous exercise, a healthy diet, social engagement and good sleep. All of these things have been proven to slow decline and should be done in concert with a clinical trial as well.

Melanie: And now, to wrap up, what do you tell families, Dr. Foff, because this is, as we said, such a difficult time, not only for the person going through it, but for their loved ones, about that support for them as a caregiver, and how difficult it can be to watch somebody in cognitive decline.

Dr. Foff: That is such a crucial piece of the puzzle. So many of these caregivers take on just an incredible burden in terms of emotional output, time away from work--all of those things that. So, what I tell families is this is a marathon and not a sprint. And that keeping them healthy, well-supported emotionally and from community resources, etc, and from the clinic resources, is really critical to keeping them healthy through the entire process. And making sure that every step of the way, they understand that there’s a team that can help answer their questions, making sure they get rested when it’s needed—all of those are just so crucial to making sure that the entire family unit is supported, not just the patient alone.

Melanie: Great information. Thank you so much, Dr. Foff, for being with us today. You’re listening to UVA Health Systems Radio. And for more information, you can go to www.uvahealth.com. That’s www.uvahealth.com. This is Melanie Cole. Thanks so much for listening.