Frontotemporal Degeneration (FTD)

Frontotemporal Degeneration Overview

Frontotemporal degeneration (FTD) is a progressive neurological disease in which the neurons in the temporal anterior and frontal lobes of the brain decrease in size or become damaged. This syndrome can also be referred to as Pick’s disease, frontotemporal dementia, and frontotemporal lobar degeneration.

Frontotemporal degeneration has several variants and related syndromes, including:

• Behavioral variant
• Primary progressive aphasia
  • Nonfluent/agrammatic variant
  • Semantic variant
  • Logopenic variant
• ALS and frontotemporal degeneration
• Corticobasal degeneration
• Progressive supranuclear palsy

Frontotemporal degeneration is rare, and it is estimated that about 60% of individuals who are diagnosed with frontotemporal degeneration are between the ages 45 and 64. Out of all cases of dementia, frontotemporal degeneration accounts for 2-5% of cases. Frontotemporal degeneration is equally common in both women and men and among different ethnicities and races. However, a family history of dementia or memory disorders is present in about 50% of cases.

Genetic testing and counseling are available to help families identify a predisposition for frontotemporal degeneration earlier.

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There are some symptoms that are specific to the different variants of frontotemporal degeneration.

For instance, the symptoms of behavioral variant frontotemporal degeneration are more concentrated on social interactions, emotional responses, and judgement. The memory of people with the behavioral variant may be mostly unaffected.

In primary progressive aphasia FTD, symptoms are focused on the language processes in the brain. People with primary progressive aphasia may have difficulty speaking, understanding what is being said to them, or comprehending written language.

In corticobasal syndrome and progressive supranuclear palsy, people experience difficulty moving their body. They may also experience unwanted movements, difficulty with balance, or muscle rigidity.

Finally, in the ALS and Parkinson’s disease variants, people experience a combination of the symptoms of both diseases.

Prognosis and Progression of Frontotemporal Degeneration

Frontotemporal degeneration and its variants progress differently for each individual. Some people experience a longer life expectancy, while others can face a life expectancy of about seven-to-thirteen years. Sometimes it is possible for people to have a shorter life expectancy.

There aren’t specific stages of frontotemporal degeneration, however, the presence of multiple symptoms that occur at once usually means that the disease is progressing to a more severe stage. Problems with behavior and language can be indicative of more severe disease, and loss of motor skills indicates an individual is in a later stage. Scans and neuroimaging can be used to also see the progression of this syndrome.

General Measures

It is important for people with frontotemporal degeneration to maintain optimal general health. Anything that worsens your medical condition can also worsen FTD.

Maintaining physical and intellectual activity is also important. However, it is important to not stress someone beyond their capacities. Your doctor can discuss with you which activities are appropriate and may be helpful.