What Is Frontotemporal Dementia and Is There a Cure?

Frontotemporal dementia is a type of brain disease that worsens over time and causes the brain to shrink. It belongs to a group of conditions where abnormal proteins form inside brain cells leading to damage and eventual loss of those cells.

Frontotemporal dementia contains several diseases that selectively affect the brain’s frontal and temporal lobes. This type of dementia often occurs at a younger age. Around 60% of people with frontotemporal dementia are 45 to 65. People with a family history of certain forms of the disease may be more likely to develop it. However, most people with frontotemporal dementia have no relatives with the disease.

The frontal and temporal lobes, responsible for attention, problem-solving, and language skills, are affected first. The frontal lobe also controls aspects of personality and behavior, such as social etiquette, empathy, judgment, and self-control. Over time, other brain areas become affected. Consistent with most neurodegenerative diseases, symptoms gradually worsen over several years.

On average, individuals with frontotemporal dementia live for 6 to 8 years from diagnosis. The rate of decline varies from person to person. It is important to note that frontotemporal dementia is a highly variable disease, and some individuals may experience more rapid decline while others may have slower progression.

How is frontotemporal dementia diagnosed?

Frontotemporal dementia can be hard to diagnose because it is an uncommon type of dementia that initially causes changes in behavior. In early stages, it can be misdiagnosed as a mood disorder such as depression.

To help diagnose frontotemporal dementia, physicians and other healthcare providers may:

Conduct a neurological exam to assess mental status, reflexes and movements
Order laboratory tests to rule out other conditions that can mimic dementia
Conduct a neuropsychological evaluation to assess memory, language skills, problem-solving ability and other thinking skills
Order imaging of the brain to examine areas of the brain where shrinkage has occurred
Order genetic testing

What are the main types of frontotemporal disorders?

Behavioral variant frontotemporal dementia: This is the most common type of frontotemporal disorder. As the name suggests, changes in a person’s behavior are the hallmark feature and are observed early in the disease. In behavioral variant frontotemporal dementia, nerve cell loss is most noticeable in areas that control conduct, judgment, empathy and foresight.

People with behavioral variant frontotemporal dementia may show the following symptoms:

Socially inappropriate behavior: An example may be standing too close to strangers or making offensive remarks. Caregivers might describe them as “having no filter.”
Apathy or loss of interest in activities they used to enjoy. They might become more passive
Loss of empathy: They may be less sensitive or unconcerned by the feelings of others
Compulsive behavior: This can appear as movements or behaviors that are repetitive, obsessive interest in a special and new hobby, or ritualistic behaviors like checking or hoarding
Change in their dietary preferences: They may continue to eat after they have had a full meal. Some people may try to consume inedible objects.

As the disease progresses, complex cognitive skills such as working memory, planning and problem-solving worsen.

Primary progressive aphasia: This disorder is associated with changes in the ability to communicate. People with primary progressive aphasia may have one or both symptoms and become mute or unable to speak.

The two forms of primary progressive aphasia have different symptoms:

Semantic variant of primary progressive aphasia: Persons with this type of aphasia experience a loss of word meanings and knowledge of concepts. They may substitute specific words for vague terms, accompanied by the misreading and misspelling of words. Over time, they may struggle with recognizing objects and people.
Nonfluent/agrammatic variant of primary progressive aphasia: These people have difficulty getting words out and putting sentences together and may have problems pronouncing simple words. As the disease progresses, they have issues using correct grammar and understanding complex sentences.

Movement disorders: Movement problems like those seen in Parkinson’s disease are also associated with frontotemporal disorders. Examples include difficulty with balancing, walking, and swallowing. People can experience movement disorders along with behavioral variant frontotemporal dementia or primary progressive aphasia, though that is not always the case.

A rare movement disorder is ALS or amyotrophic lateral sclerosis. This is a motor neuron disease that causes muscle weakness or wasting. Frontotemporal dementia and ALS are two distinct disorders that have common protein abnormalities. Some people with ALS develop cognitive and behavioral symptoms associated with frontotemporal conditions.

Is there a cure for frontotemporal disorders?

There is no cure for frontotemporal disorders. For patients with language problems as part of the disease, speech therapy can be helpful for learning strategies to assist with communication. A doctor can prescribe medication to help manage challenging behavior, but the first line of intervention should involve making environmental changes to meet the needs of the individual. The goal is to adapt to the environment rather than trying to adapt to the individual. Prescriptions cannot stop frontotemporal dementia from worsening, but they can help reduce some symptoms.

Individuals with frontotemporal dementia must receive appropriate medical care and support at each stage of the disease. Working closely with a healthcare specialist knowledgeable about frontotemporal dementia can help support the specific needs of individuals and provide the best possible quality of life.