Bruce Willis and Frontotemporal Dementia

Bruce Willis and Frontotemporal Dementia
Bruce Willis. Photo/Gage Skidmore

By Dr. Vivek Sinha

Dementia is a condition that affects more than 55 million people worldwide. The financial strain of this progressive condition is matched by the emotional toll it places on family members and care- givers. Last week we learned the unfortunate news that actor Bruce Willis was diagnosed with a type of dementia, called Frontotemporal Dementia.

The diagnosis of dementia is a difficult burden for anyone to bear, and given the actor’s fame and popularity, there have been many questions about FTD. What is it? How does it differ from other types of dementia? How is it diagnosed? And is there treatment?

Dementia is defined as a decline in cognition. Cognition determines how our brain obtains and processes information. In dementia, we find a decline in one or more aspects of cognitions – language, learning and memory, executive function, complex attention, perceptual-motor or social cognition. This decline must be severe enough to interfere with day-to-day functioning and independence.

There are multiple types of dementia. The five most common are: Alzheimer’s Dementia, Dementia with Lewy Bodies, Parkinson Disease Dementia, Frontotemporal Dementia and Vascular Dementia. These have broadly overlapped symptoms, but there are some specific features associated with the varying types.

Approximately 60% to 80% of older individuals with dementia have Alzheimer’s Dementia. It is important to note that it is also possible to have more than one type of dementia, also known as Mixed Dementia.

How do patients with dementia present clinically? There are common features that we see in all patients and then there are features that are more common depending on the types of dementia the person may have. The most common symptom that can be found across the board is usually related to difficulty with memory such as remembering events or retaining new information.

Other common features are difficulty with handling complex tasks such as managing finances, getting lost in familiar places, trouble finding the proper words or difficulty in coping with unexpected events. Family members are usually the first to notice the change but since these changes can be subtle and are often chalked up to “normal aging changes,” it may be months or years before the symptoms are truly addressed and medical care is sought.

In addition to the common signs noted above, certain features that may be more specific to the particular type of dementia:

Alzheimer’s Dementia – This type of dementia typically occurs in people older than 65, although there is an early onset variant that can affect people in their 40s and 50s. Short term memory impairment is the most common initial symptom as well as impaired executive functions, and reduced insight. As the condition progresses, sleep disturbances, anxiety and emotional lability become more common.

Dementia with Lewy Bodies, Parkinson Disease Dementia – Both of these types of dementia present with a gradual decline in cognition associated with specific movement disorders. Specifically, for Parkinson’s Dementia, patients present with movement problems approximately five to eight years prior to showing cognitive decline. Some of the movement changes seen are an abnormal leaning-forward gait when walking, small quick shuffling steps, reduced swinging of arms while walking and difficulty in initiating or continuing movement. Patients diagnosed with Lewy Bodies usually exhibit cognitive decline and movement disorders as well as sleep behavior changes, hallucinations and fluctuations in levels of alertness.

Frontotemporal Dementia – Different parts of the brain are responsible for various body functions. For example, the frontal lobe in the brain is responsible for emotional expressions, reasoning, thinking and problem solving. The side of the brain, temporal lobe, is responsible for speech and hearing. In FTD, both lobes are affected. The typical onset usually occurs in patients in the 60s. There are several types of FTD. The two main subtypes that we will mention here are: (a) Primary Progressive Aphasia FTD and (b) Behavioral Variant FTD.

In Primary Progressive Aphasia FTD, symptoms early on are usually a slow and insidious onset of speech and language impairment. This includes word finding difficulty, word usage difficulty, word comprehension difficulty, and sentence construction difficulty. Per media reports, this is the subtype that Willis was diagnosed with.

In the Behavioral Variant FTD, we see symptoms of the frontal lobe being impaired. Since the frontal lobe is responsible for the emotional aspect of our personality, we start to see a loss of control of emotions.

Vascular Dementia – This type of dementia is usually associated with small blood-vessel disease caused by cardiovascular problems. Patients with long-standing uncontrolled high blood pressure, patients with a history of strokes or mini-strokes, heart disease or anything else that causes decreased blood flow to the blood vessels in the brain are at risk for this type of dementia. Symptoms are initially associated with cognitive impairment and a slowing of overall processing speed.

How do doctors diagnose dementia? Unfortunately, there is not a specific blood test or imaging study that can be used to diagnose dementia. The first step is a visit with your primary care provider.

Since most patients with dementia do not complain about memory loss, a family member or a close contact of the patient must be available to give a history and talk about the behavioral changes. A thorough medical history should be taken, including a review of every prescription and non-prescription medication that the patient is taking. A complete past medical history and family history should also be taken.

An assessment of someone’s Activities of Daily Living and Instrumental Activities of Daily Living should be taken. The ADLs consist of basic self-care activities such as walking, feeding, dressing, toileting, bathing and transferring and the IADLs are the self-care tasks that include more executive function tasks. Any decline or change in ADLs or IADLs should be carefully evaluated and monitored.

A careful mental status examination should also be performed. There are various screening tests that can be utilized by the primary care physician. Checking for depression is also important. While people with dementia can have depression as well, it is important to know that in some cases depression in the older patient can mimic dementia.

Afterward, a thorough physical examination should take place followed by lab work. The lab work is performed to ensure that other conditions that can mimic signs and symptoms of dementia are not present. For example, untreated thyroid problems and vitamin B-12 deficiency can present with symptoms like dementia.

A consideration for brain imaging should also be considered at this point. Often the imaging is performed to ensure that any anatomical abnormalities or any other conditions are not causing the patient’s symptoms.

Different findings on the imaging and lab work may help guide the clinician towards a specific diagnosis. However, it is important to note that a presumptive diagnosis of dementia can only be made by taking all factors into account. For certain patients, further neuropsychological testing may be indicated. Often, specialists such as neurologists can help primary care physicians decide if this is the best step to take.

Unfortunately, dementia is a progressive condition, which means that it will always progress and worsen. Currently, a cure for dementia does not exist. However, there are certain treatments that can help either slow the progression down or can help with some of the symptoms of dementia, such as anxiety, aggression and insomnia. Medications can be helpful for some but may be potentially harmful for others, so a detailed conversation about risks versus benefits should take place prior to starting any new treatment.

Dementia is a daunting condition but luckily there are many resources available for caregivers. The Alzheimer’s Foundation of America, the Alzheimer’s Association, and the Centers for Disease Control and Prevention all have valuable online resources and guides.

As a primary care and house call physician who has had both personal and professional experience with this disease process, it is important to know that you are not alone. Find a good support team, find out about medical and community resources and most importantly, go to your doctor to talk about your concerns and situation.

The writer is chief medical officer of Belleview Medical Partners, an office and house call practice located in Old Town.