Frontotemporal Dementia

Overview

What is frontotemporal dementia (FTD)?

Frontotemporal dementia (FTD) is a group of disorders that result from damage to the frontal and temporal lobes of the brain. Depending on the location of the damage, the disorder causes changes in social behavior, personality, and/or loss of language skills. In addition, some people (between 10% and 20%) with FTD also develop neuromuscular and movement disorders, such as parkinsonism or motor neuron disease (also known as amyotrophic lateral sclerosis or Lou Gehrig’s disease).

FTD is a common cause of early dementia. Symptoms worsen over time as more parts of the brain are affected. Unlike typical Alzheimer’s disease, however, memory usually remains intact in the early stages of FTD.

FTD consists of three subtypes:

  • Behavioral variant FTD (bvFTDO). This is also called frontal variant FTD). This subtype accounts for about 50% of all cases of FTD. Persons with bvFTD experience progressive changes in personality, behavior, emotions, and planning and problem-solving skills.
  • Nonfluent primary progressive aphasia (PPA). This is also called agrammatic PPA). People with this subtype have difficulty forming and speaking words because the brain has trouble planning and performing the facial muscle movements needed to produce speech. They often seem to have problems with grammar, such as leaving out words or mixing up words, effortful speech, and communicate with shorter, simpler, sometimes incomplete phrases.
  • Semantic variant PPA. This is also called temporal variant FTD or semantic dementia. People with this subtype slowly lose their ability to understand the meanings of words, recognize everyday objects and familiar faces, and/or use common items.

Who gets frontotemporal dementia (FTD)?

Approximately 60% of people with frontotemporal dementia (FTD) are between the ages of 45 and 64. It is estimated that about 10% of dementia cases are the result of FTD. Men and women are affected about equally.

Symptoms and Causes

What causes frontotemporal dementia (FTD)?

Frontotemporal dementia (FTD) occurs when abnormal proteins (there are many kinds) build up in the brain. This leads to death of brain cells and deterioration and shrinkage (atrophy) of the frontal and temporal lobes of the brain. Sometimes the disease is genetic, and passed down through families, but in most cases no specific cause can be found.

What functions are controlled by the frontal and temporal lobe of the brain?

The frontal lobes control executive functioning, meaning a person’s ability to plan in a step-by-step process, to choose priorities, to keep track of several tasks at one time, and make adjustments to one’s behavior. The frontal lobe also controls social and emotional responses. In addition, the frontal lobe helps in linking words to form sentences and is crucial making involuntary muscle movements.

Lobes of the brain, including the frontal, temporal, parietal, and occipital lobes

The temporal lobes are the major language center, involved with speaking, reading, writing, understanding words and their meanings, and recognizing objects. The temporal lobe also plays a role in recognizing and responding to emotions and is a major memory center.

What are the symptoms of frontotemporal dementia (FTD)?

Early behavioral changes may include:

  • Inappropriate social behavior and lack of social tact/manners. Examples include touching or kissing strangers, urinating in public, making rude or offensive comments, arguing, rashly overspending, and/or doing or saying things that others would find embarrassing or disgusting.
  • Lack of empathy (interest in, or understanding of, what others feel), loss of interest in other people or activities, reduced affection, neglect of personal grooming and hygiene. People with FTD are not aware of the changes that are happening and do not know how hurtful they are to close family members.
  • Changes in food preferences, overstuffing mouth with food, binge eating, eating food quickly, attempting to eat non-food items.
  • Becoming very obsessive or developing rituals, repeating things, collecting/hoarding items.

Language problems may include:

  • Difficulty with the ability to form words.
  • Problems with grammar, leaving words in a sentence out, causing speech to become effortful and shortened to simple, partial phrases.
  • Lack of understanding of the meanings of words.
  • Inability to recognize objects or familiar faces.

Symptoms of neuromuscular disease or Parkinsonism (most often seen in patients with behavioral FTD) can include:

  • Weakness in the arms and legs.
  • Weakness in the muscles of speech, such as lips and tongue.
  • Difficulty swallowing.
  • Muscle jerks or spasms.
  • Stiffness of muscles and slowness of movement.
  • Tremor, or shaking.
  • Problems with balance and walking.

The types of symptoms and the order in which they appear can vary.

Diagnosis and Tests

How is frontotemporal dementia (FTD) diagnosed?

Diagnosis is typically based on the clinical judgment of the doctor. Often, people with behavioral variant FTD don't believe they need to see a doctor. And when they do, they may ace the standard memory tests for dementia. Therefore, interviewing loved ones about the patients’ symptoms is often key to making the diagnosis, as patients typically cannot recognize changes in their behavior. Lab tests and brain scans will also be done to exclude other causes of impairment. Brain scans may be normal early in the course of disease. As the disease worsens, up to 65% of patients will show signs of brain damage in the frontal and temporal lobes.

Management and Treatment

How is frontotemporal dementia (FTD) treated?

There is no cure for frontotemporal dementia (FTD). No treatment has been shown to slow the progression of the disease. However, medications may reduce some of the behavioral symptoms. Antidepressants, particularly selective serotonin reuptake inhibitors, have been shown to improve some behavioral symptoms. Antipsychotic drugs may also be used to treat behavioral symptoms, however, they are associated with significant side effects that can further aggravate the symptoms of FTD. People with motor neuron disease can be treated with riluzole (Rilutek®).

Speech and language therapy may be helpful for some people with early-stage primary progressive aphasia. A speech-language pathologist may help patients learn new communication strategies, such as non-verbal techniques (for example, gesturing or pointing to cards with words, pictures or drawings). Electronic and computer devices and artificial voice synthesizers can also aid or replace speech in some people with PPA.

Genetic counselling may be appropriate for family members with a family history of FTD.

Outlook / Prognosis

What is the prognosis (outcome) for someone with frontotemporal dementia (FTD)?

Frontotemporal dementia worsens over time. Eventually people with FTD will need help with their activities of everyday living. Some people may need 24-hour care at home or at living facilities or nursing homes. The speed of decline varies from person to person, but the disease course typically ranges from two to 10 years.

Resources

What resources are available for frontotemporal dementia (FTD)?

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy