Frontotemporal dementia

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Definition

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

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 blunted, 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. And the memory problems associated with Alzheimer's disease are not as prominent in the early stages of frontotemporal dementia.

Researchers estimate that at least 2 percent of all dementia cases are the frontotemporal variety. The disease appears to run in families — between 20 percent and 50 percent of people who have frontotemporal dementia have a family history of some type of dementia. After diagnosis, the course of the disease may run anywhere from two to 10 years before resulting in death.

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Symptoms