Frontotemporal Dementia

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Frontotemporal dementia (frontotemporal lobar degeneration) is an umbrella term for a diverse group of uncommon disorders that primarily affect the frontal and temporal lobes of the brain — the areas generally associated with personality, behavior and language.

In frontotemporal dementia, portions of these lobes atrophy or shrink. Signs and symptoms vary, depending upon the portion of the brain affected. Some people with frontotemporal dementia undergo dramatic changes in their personality and become socially inappropriate, impulsive or emotionally indifferent, while others lose the ability to use and understand language.

Frontotemporal dementia is often misdiagnosed as a psychiatric problem or as Alzheimer's disease. But frontotemporal dementia tends to occur at a younger age than does Alzheimer's disease, typically between the ages of 40 and 70.

Frontotemporal Dementia Symptoms

Identifying precisely which diseases fall into the category of frontotemporal dementia presents a particular challenge to scientists. The signs and symptoms may vary greatly from one individual to the next. Researchers have identified several clusters of symptoms that tend to occur together and are dominant in subgroups of people with the disorder. More than one symptom cluster may be apparent in the same person. The signs and symptoms of frontotemporal dementia progressively worsen with time, almost always over years, eventually requiring 24-hour care.

Behavioral changes
The most common signs and symptoms of frontotemporal dementia involve extreme changes in behavior and personality. These include:

Increasingly inappropriate actions
Loss of empathy and other interpersonal skills
Lack of judgment and inhibition
Apathy
Repetitive compulsive behavior
A decline in personal hygiene
Changes in eating habits, predominantly overeating
Lack of awareness of thinking or behavioral changes
Speech and language problems
Some subtypes of frontotemporal dementia are marked by the impairment or loss of speech and language difficulties. For example, primary progressive aphasia is characterized by an increasing difficulty in using and understanding written and spoken language. People with another subtype, semantic dementia, utter grammatically correct speech that has no relevance to the conversation at hand or may have difficulty recalling the words for common objects.

Movement disorders
Rarer subtypes of frontotemporal dementia are characterized by problems with movement, similar to those associated with Parkinson's disease or amyotrophic lateral sclerosis (ALS), which is also often called Lou Gehrig's disease.

Movement-related signs and symptoms may include:

Tremor
Rigidity
Muscle spasms
Poor coordination
Difficulty swallowing
Muscle weakness

Frontotemporal Dementia Causes

A variety of mutations on several different genes have been linked to specific subtypes of frontotemporal dementia. But more than half the people who develop frontotemporal dementia have no family history of dementia.

In some cases of frontotemporal dementia, the affected parts of the brain contain microscopic Pick bodies — abnormal protein-filled structures that develop within brain cells. Frontotemporal dementia was once known as Pick's disease, but now that terminology is reserved for the subtype that actually features these abnormal structures.