Frontotemporal Dementia

Symptoms

The most common symptoms are related to language, behavior, and personality. Studies show that half of people with the disease have behavior and personality changes, and half have language and memory changes. But all eventually experience significant declines in their cognitive abilities and higher executive functions.

Personality changes, impulsive behavior, and impaired judgment

• They may wash themselves less often.
• They may engage in risky behaviors.
• They may start making poor decisions with their money and savings.
• They may hoard food or other items, and may compulsively overeat.
• Sometimes they are misdiagnosed with a psychotic break.

Language problems

• Someone may understand language but have difficulty speaking actual words.
• They may have difficulty processing language and use words that don’t have any meaning.

Apathy

• Someone may become passive or “blank.”
• Trouble initiating activities

Lack of empathy

• A person may not care how their actions are affecting others.

Trouble concentrating or paying attention

Memory loss

• Losing track of the date, time, or year
• Frequently losing things and being unable to find them
• Forgetting names of family members or close friends

Mood changes

• Depression
• Irritability
• Anger out of proportion to the situation

Increased dependence on others in activities of daily living

Causes

Scientists do not know exactly what causes frontotemporal dementia. The specific changes to the brain may be similar to changes in other brain diseases such as amyotrophic lateral sclerosis (ALS or Lou Gherig’s disease), Alzheimer’s disease, and Parkinson’s disease. There may be a genetic component to some types of frontotemporal dementia.

Frontotemporal Degeneration and Lobar Degeneration

Frontotemporal dementia is part of a larger group of brain disorders called frontotemporal degeneration (FTD). FTD refers to a process where the frontal and temporal lobes of the brain gradually lose nerve cells and shrink over time. This degeneration leads to the various symptoms seen in frontotemporal dementia.

The term "frontotemporal lobar degeneration" (FTLD) is often used interchangeably with FTD. FTLD specifically describes the physical changes in the brain that occur as the disease progresses. In FTLD, the frontal and temporal lobes become smaller and show distinctive patterns of damage when examined under a microscope.

Both frontotemporal degeneration and frontotemporal lobar degeneration are progressive conditions. This means that over time, more areas of the brain become affected, and symptoms typically worsen. The rate of progression can vary from person to person, but on average, symptoms tend to worsen over a period of 8 to 10 years after they first appear.

Understanding these terms helps explain why frontotemporal dementia causes such a wide range of symptoms affecting behavior, language, and movement. As different areas within the frontal and temporal lobes are affected by the degeneration process, various abilities controlled by these brain regions begin to decline.

Next steps

Symptoms of frontotemporal dementia progress over years. You and your family may start to notice subtle changes in behavior and language skills. For example, a person may mix up words or have difficulty remembering what objects are called. Your family may notice personality changes or unusual behavior that you do not notice.

It is important to take note of these and not brush them off or ignore them. Contact your doctor if you start to notice any symptoms. It is important to have a thorough evaluation for other causes of memory loss or behavior changes.

There is not one single test or biomarker that diagnoses the disease. Your doctor will do a full examination that includes lab tests, cognitive tests, and brain imaging. The most important part of the evaluation is the careful review of symptoms. A brain MRI might show shrinkage of the frontal and temporal lobes, but the brain may also look normal because structural changes often lag behind your symptoms. Your doctor might recommend functional imaging like a PET scan.

Treatment

There is no cure for frontotemporal dementia. However, treatments can help control symptoms, and make living with the condition easier.

Therapies

A combination of therapies can help people maintain as much independence as possible.

• Physical therapy helps improve strength and balance, and exercise has also been shown to help with brain function and memory.
• Occupational therapy can help adapt and manage activities that now may seem difficult, such as toileting, feeding, and dressing.
• Speech therapy can help people adapt to problems with communication and swallowing.
• Nutritional therapy can help with weight gain that is caused by compulsive overeating, or with weight loss that can occur later in the illness.

Medications

Different medications can help reduce symptoms. Any medication has to be used with caution with careful attention to the possible side effects. Each person needs a personalized approach to medications based on their symptoms, their goals, and their other medical problems.

• Antipsychotic medications can help with behavior problems.
• Antidepressants can help control depression, anxiety, compulsions, and other mood symptoms.
• Stimulants may help with apathy.