Dementia

Understanding Dementia

As a writer and journalist who has lost loved ones to dementia, my primary objectives in sharing personal accounts — mine, with my husband and father, and those of others — are to help strengthen awareness and sensitivity for people living with and around dementia, reduce stigma and isolation, and change the way we understand and manage dementia in both daily life and the practice of medicine.

The World Health Organization declared dementia a public health priority — in 2012. And it’s only becoming more acute. Neurological illnesses know no geographic, racial, or socio-economic barriers. Nor are they limited to genetic predisposition. Not one of us is immune.

In my ongoing efforts to illuminate the hidden ways that dementia-related illnesses impact lives, I’m writing a narrative nonfiction book, which expands on articles I’ve researched and written for New York Magazine / The Cut, New York Times Magazine, San Francisco Chronicle Magazine, Canadian Journal of Neurological Sciences, and other publications.

Alzheimer’s disease is the leading cause of dementia, so it’s a vital part of the discussion. Comparatively, much less has been written about non-Alzheimer’s dementias — some of which strike people when they’re younger, at the peak of their careers. Wider recognition is essential for many reasons, especially because it can decrease the shame that also prevents people from finding help. I hope this book clarifies non-Alzheimer’s dementias for those less familiar, while also elucidating possible ramifications that some experts might not have considered.

Another purpose is to inspire conversations with family and friends about what we want in life and death, and everything in between — should we ever lose the capacity to make those decisions for ourselves. As noted above, there are no guarantees for any of us. So we would do well to share our truth, lose the shame, articulate what we want for our own lives long before we think we should, advocate for each other, and become better listeners. Because these experiences are not as unique as we’d like to think, and nobody should have to go through them alone.

Brief Overview of Dementia

Dementia is defined as a constellation of symptoms that degenerates over time. Different causes affect various parts of the brain, which result in some notable distinctions.

Illnesses that may cause dementia include Alzheimer’s disease, Lewy body dementia, frontotemporal dementia, vascular dementia, progressive supranuclear palsy, and corticobasal degeneration, among others.

What are the sources these diseases? A combination of genetic, environmental, and lifestyle (diet, exercise, stress) factors, along with each person’s unique predisposition for a particular illness. We know a lot about what we can do to improve our chances of staying healthy, but much remains a mystery about why one family member develops a disease and another, who lives a similar lifestyle, may not.

Here’s an overview of the most common illnesses that cause the cognitive decline of dementia:

Lewy body dementia (LBD) or Dementia with Lewy bodies (DLB)

Personal observations: When my father first began showing signs of dementia, nobody could pinpoint the source of the problems. Issues with attention and memory resembled Alzheimer’s disease. Vivid recollections from his football days in high school and college were readily available, yet he couldn’t remember new material for volunteer work. Yet the forgetfulness was inconsistent. As a collegiate (and lifelong) athlete and former Navy SEAL, Dad’s trouble with balance, coordination, and handwriting raised suspicions of Parkinson’s disease. But there was no obvious tremor. Besides, that explanation  seemed incomplete. He struggled to sleep, so a neurologist prescribed a C-PAP machine. Conversations shifted to sleep apnea, Post-Traumatic Stress Disorder (he fought in the Vietnam war), and mild cognitive impairment, which also seemed inadequate, given the disturbing hallucinations that upset him (and everyone else) more frequently.

I started calling the primary care physician to make sure he had all the information about what had been happening. But he didn’t, because reports to him had been incomplete or well-burnished. We suspected Lewy body dementia. Pink blobs deposited in the brain are the Lewy bodies that give the disease its name. These are different from the tumbleweeds that are the abnormal amyloid of Alzheimer’s disease.

We finally confirmed the illness — in the autopsy after he died. 

From the textbooks: Lewy body dementia (LBD) is a disease associated with abnormal deposits in the brain of a protein called alpha-synuclein. These deposits, also called Lewy bodies, affect chemicals whose changes, in turn, can lead to problems with thinking, movement, behavior, and mood. This is one of the reasons why it can be misdiagnosed for a psychiatric illness such as schizophrenia. LBD is one of the most common causes of dementia, after Alzheimer’s disease and vascular disease. 

(Source: National Institute on Aging at the National Institutes of Health)

In our video, behavioral neurologist Tiffany Chow, M.D. calls LBD (or DLB – don’t let the acronyms get you down) a terrible mash-up of Alzheimer’s and Parkinson’s’ diseases.

Key parts of the brain involved:

• Cerebral cortex (information processing, perception, thought, language)
• Limbic cortex (emotions and behavior)
• Hippocampus (forming new memories)
• Mid-brain and basal ganglia (movement)
• Brain stem (regulating sleep and alertness)

(Source: National Institute on Aging, National Institutes of Health)

More information and help:

Th Lewy Body Dementia Association website contains videos of lectures and webinars with experts. Because LBD differs from other forms of dementia, often quite dramatically, there’s helpful information about the unique symptoms — which include hallucinations, delusions, and sleep issues — along with links to virtual support groups.

Excerpts:

• Regardless of the initial symptom, over time all three presentations of LBD will develop very similar cognitive, physical, sleep and behavioral features.
• Some individuals will start out with a movement disorder leading to the diagnosis of Parkinson’s disease, and later develop dementia. This is diagnosed as Parkinson’s disease dementia.
• Another group of individuals will start out with a cognitive/memory disorder that may be mistaken for AD (Alzheimer’s disease), but over time, two or more distinctive features become apparent, leading to the diagnosis of ‘dementia with Lewy bodies.
• Lastly, a small group will first present with neuropsychiatric symptoms, which can include hallucinations, behavioral problems, and difficulty with complex mental activities, also leading to an initial diagnosis of DLB. (Source: 10 Things You Should Know about LBD)

Now you know why it’s so confusing!

Alzheimer’s disease (AD)

Personal observations: Thanks to enhanced public education and courageous people like the Reagans and the Shrivers who have shared their experiences, most people are familiar with Alzheimer’s disease. Even so, almost everyone I’ve interviewed has described a bewildering and protracted struggle to arrive at a diagnosis. Susan Disney Lord, daughter of Roy E. Disney, talked with me about her mother’s years-long battle with confusion and disorientation before the family understood what was happening. You can hear her account in the Podcast section of this website. 

Maria Shriver is someone I deeply admire as a woman, mother, and journalist. She’s also a tireless advocate for people battling Alzheimer’s. Check out Maria’s website here. 

From the textbooks: Alzheimer’s is a degenerative disease that destroys memory and other important mental functions. At first, someone with Alzheimer’s may notice mild confusion and difficulty remembering details. Eventually, people  may even forget loved ones and undergo dramatic personality changes. (Source: Mayo Clinic)

• Hippocampus (memory center of the brain)
• Parietal lobes (processing sensory information, such as taste, temperature, and touch)
• Temporal lobes (understanding speech/language)

Learn about the Ten Early Signs and Symptoms of Alzheimer’s disease

Learn more about genetic factors, APOE, testing, epigenetics:  Alzheimer’s Disease Genetic Fact Sheet (Source: National Institute on Aging at the National Institutes of Health)

 More information and help:

Alzheimer’s Association

While the Alzheimer’s Association has numerous local chapters under the national umbrella, the following cities have disassociated with the national organization to better control funds raised and programs implemented:

Los Angeles

Orange County

San Diego

Texas

New Jersey

New York

Frontotemporal dementia (FTD)

Personal observations: At the time my husband began showing symptoms, he was an accomplished physician. He began having trouble speaking, reading, writing, multi-tasking — doing his job as dean of a medical school. But most disturbing to me was the change in his behavior. Paranoia crept in. He began exhibiting anomalous behaviors: lying, spending money recklessly, endangering our family. I didn’t know who he was anymore. My constant requests for him to see a doctor were met with accusations: I was the problem. Not until his colleagues from Yale confronted him — and discussed with me — the dramatic cognitive changes they’d noticed, did we see  a doctor. Even then, a definitive diagnosis was elusive because it was so easy for him to appear perfectly normal and cover for himself (others joined this effort, believing they were helping). 

From the textbooks: As opposed to the previous diseases, this one is named for its location in the brain. FTD refers to several conditions resulting from the progressive degeneration of the temporal and frontal lobes of the brain. Because these areas play a significant role in decision-making, behavioral control, emotion, and language, any of these can be disabled over time. It can strike when patients are in their thirties, forties, or fifties, when people are at the peak of their careers and raising families, often leading others to believe they’re suffering from a mid-life crisis. People with FTD are often misdiagnosed with psychiatric problems such as depression, manic-depression, obsessive-compulsive disease, or schizophrenia, and occasionally vascular dementia, Parkinson’s disease, or Alzheimer’s. (Source: UCSF Memory & Aging Center)

The Memory and Aging Center at the University of California, San Francisco is a leader in many fields, but is known for its expertise in FTD.

Frontotemporal dementia is often misdiagnosed as a psychiatric problem or, less frequently, as Alzheimer’s disease. But frontotemporal dementia tends to occur at a younger age than does Alzheimer’s disease, sometimes between the ages of 40 and 45. (Source: Mayo Clinic)

The Association of Frontotemporal Dementias changed its name to The Association of Frontotemporal Degeneration. Sometimes the illness is called Frontotemporal Lobar Degeneration, and the series of disorders associated with deterioration of the frontal lobes (decision-making and insight) and the temporal lobes (speech center of the brain, especially on the left side) are often referred to as Frontotemporal Disorders. To add another layer, the disease used to be called Pick’s for the doctor who first identified Pick bodies in the brain. (Personal note: The name changing makes me crazy.)

Vascular dementia

Personal observations: When I first heard that someone I knew who had been struggling with cognitive decline was diagnosed with Alzheimer’s disease, I thought, that’s not right. I’d spent enough time with her to know that her memory problems weren’t significant enough, though other abilities had been compromised. She had difficulty tracking a conversation, and she’d offered/agreed to stop driving after a couple of fender benders.

I kept saying it wasn’t Alzheimer’s, but my personal experience told me it wasn’t Lewy body dementia or FTD either. Her relatives gave permission to share the brain scans with a different neurologist, who quickly identified vascular dementia.

Important point: Reading scans (MRI, CT) is a complex mix of science, art, experience, and diagnostic abilities. Just because it’s medicine doesn’t mean that all physicians possess the same skills. Make sure you have electronic copies of everything, and don’t hesitate to obtain a second or third opinion.

This person’s family hired a part-time caregiver to help with daily necessities. But I urged them to consider moving her out of the single-family home with a steep staircase before an accident occurred, and researched viable possibilities. Nothing changed. After a shopping excursion one evening, she fell backwards down the stairs and ended up in the hospital. It took a while for me to understand that people will make adjustments in their families at their own pace, and there’s nothing you can do to alter it. Much of the time, family members disagree on these matters. In fact, I’ve never heard about — or experienced — a loved one’s dementia without a huge dose of family conflict. Try not to take it personally.

Thanks to her remarkable and resilient friend, she was able to make the most of her few remaining years with excursions and road trips and lots of laughter.

From the textbooks:  Vascular dementia refers to progressive loss of cognitive functions caused by vascular injury (i.e. small strokes) within the brain. Symptoms of vascular dementia may be difficult to distinguish from Alzheimer’s disease. Problems with organization, attention, slowed thinking, and problem solving are present, while memory loss is more prominent in Alzheimer’s.

Not everyone who has had a major stroke will develop vascular dementia, but the risk for dementia is significantly higher in someone who has had a stroke. (Source: National Institute on Aging at the National Institutes of Health)

Key parts of the brain involved:

Wherever the strokes occur. Vascular dementia is caused by conditions that damage the blood vessels in the brain, depriving the brain of oxygen. This oxygen shortage inhibits the brain’s ability to work as well as it should. For example, stroke blocks blood flow to the brain, decreasing oxygen. However, high blood pressure, high cholesterol, and smoking also increase the risk of vascular dementia, which can occur  alone or with Alzheimer’s disease. (Source: National Heart, Lung, and Blood Institute at the National Institutes of Health

Other illnesses that can cause dementia:

ALS, or Lou Gehrig’s disease

Corticobasal Syndrome (CBS) or Corticobasal Degeneration (CBD)

Progressive Supranuclear Palsy

Chronic Traumatic Encephalopathy (CTE)

A more comprehensive list of neurodegenerative diseases and other disorders  that can cause dementia: (Source: UCSF Memory and Aging Center)