When the family of actor Bruce Willis announced that he has frontotemporal dementia (FTD), many Americans learned for the first time about a condition that isn’t nearly as well-known as Alzheimer’s disease or other brain disorders.

Up until the 1990s, even doctors were largely in the dark about FTD, which is more precisely called frontotemporal lobar degeneration (FTLD), because it describes a group of conditions that affect the frontal and temporal lobes of the brain.

What doctors and scientists do know today isn’t enough to offer cures or slow down the damage caused by these conditions. But progress is being made. And greater public awareness may help more patients get help sooner for the life-altering symptoms that often begin in midlife and go years without appropriate diagnosis and treatment.

So, here are the answers to a few common questions.

What happens in frontotemporal lobar degeneration?

Conditions under the FTLD umbrella can cause problems with behavior, language, decision-making, and movement. The common thread is that they all involve progressive damage to brain cells in the frontal and temporal lobes of the brain. Those are the areas behind your forehead and ears. The underlying cause is likely a buildup of abnormal proteins. As the damage progresses, different symptoms may emerge, most often between ages 45 and 64. About 60,000 Americans have some form of FTLD, according to the Association for Frontotemporal Degeneration.

While some people with FTLD are initially misdiagnosed with Alzheimer’s disease, the conditions differ in  important ways. Memory loss is the most prominent early symptom of Alzheimer’s disease, but not FTLD. Most, though not all, Alzheimer’s cases are diagnosed in older people. 

Doctors divide FTLD into several subtypes, based on which symptoms are most pronounced when patients are first diagnosed. These are:

  • Behavioral-variant frontotemporal dementia. This is the most common type and often starts with socially inappropriate behaviors, such as making rude comments, shoplifting, neglecting personal hygiene, and touching strangers. A loss of judgment, which makes people vulnerable to scams and financial mistakes, is common, as are emotional changes, including a loss of warmth and concern for others. Other symptoms can include binge-eating sweets and obsessive-compulsive habits, such repeating words and phrases.
  • Primary progressive aphasia. This type starts with problems using language to speak, read, write, or understand others. The person may struggle to produce speech, talking in a way that is increasingly hesitant and labored or, in another variation, may seem to speak almost normally while gradually losing the ability to use and understand specific words. Willis’s family said he was initially diagnosed with language problems.
  • Movement and muscle disorders that may or may not involve behavior or speech changes. These include a condition called corticobasal syndrome, which can start with problems using the hands and arms in a purposeful way, and another called progressive supranuclear palsy, which causes muscle stiffness and changes in walking, posture, and eye movements.

How are these conditions diagnosed?

These conditions often are misdiagnosed as Alzheimer’s disease, other forms of dementia or, in the case of the behavioral variant, as anything from depression to bipolar disorder to a midlife crisis. Some people with movement and muscle symptoms are misdiagnosed with Parkinson’s disease. It’s common for symptoms to persist for more than two years before a correct diagnosis is made.

Getting a correct diagnosis will typically start with a visit to a neurologist, who will take a thorough history, relying not just on the patient, but on the people closest to them, to describe changes in behavior, speech, movement, or other possible clues. The patient also will take tests that can show language and thinking problems and undergo blood tests to rule out other conditions. The doctor will perform a physical exam, looking for altered reflexes and other possible signs of neurological damage.

Finally, the patient will undergo brain scans that look for telltale shrinkage in the frontal and temporal lobes and sometimes additional scans that show how the brain is functioning. Right now, there’s no test for the underlying buildup of abnormal proteins, but that’s likely coming in the future.

What’s the treatment?

While there’s no cure, treatment can help patients manage their symptoms and function better. For people with the behavioral variant, the mainstays are drugs that might reduce abnormal behavior and improve psychological well-being, including antidepressants, antipsychotics, and certain antiseizure drugs that have mood stabilizing effects. Medications used primarily for Alzheimer’s disease haven’t been found to be effective and can make symptoms worse in some people.

People with language problems can undergo speech and language therapy to work on ways to communicate more effectively. For example, someone having trouble speaking might point to pictures, words, or letters on a communication board to make themselves better understood.

People with motor problems might benefit from physical therapy and medication. Counseling for patients and families can be an important part of treatment, as well.

And there’s hope for better treatments in the future. Promising research on medications that directly target genetic causes is underway.

What happens as these conditions progress?

FTLD gets worse over time and eventually leads to death. But there’s a lot of variation in how quickly patients decline and how long they live. The typical life span after symptoms start is seven to 10 years, but the range is two to 20 years, studies suggest.

As these disorders progress, people with behavioral symptoms may also develop speech and motor problems, and people primarily affected with speech or motor problems may develop behavioral symptoms, as well. That’s because larger areas of the brain may become damaged over time.

During milder stages, patients may be able to continue some normal activities and spend some time alone. Later on, people typically need close, constant supervision and help with most daily activities. Families may need to consider in-home help, adult day programs, and other services. At some point, it may make sense for the person to move to a facility that offers extra support. Memory care facilities designed for people with Alzheimer’s and other more common kinds of dementia may not always be a good fit, because the needs of generally younger, more active people with FTLD can be quite different. But individual needs vary widely.

Eventually, the brain damage associated with these conditions causes physical changes such as swallowing problems, reduced appetite, and losses in strength, mobility and control of the bowels and bladder. These changes can make people vulnerable to urinary tract and skin infections, as well as pneumonia. Many people die from one of those infections.

Who’s at risk?

About 10 percent of people with FTLD carry a known gene strongly associated with their condition. In roughly 40 percent of remaining cases, some family history suggests there might be a genetic link. Other possible risk factors, including autoimmune diseases, childhood learning disabilities, toxin exposures, and head injuries, have been studied but not proven to increase risks.

Patients who have family histories suggesting a genetic link can undergo counseling to decide if they want genetic testing. If they test positive, their families also can consider testing.

Where can families find help?

Caring for a loved with FTLD is challenging, so many families find it helpful to talk to others in the same situation. The Association for Frontotemporal Degeneration has a variety of support options:

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