|Frontotemporal dementia (FTD) is a group of disorders caused by progressive cell degeneration in the brain's frontal lobes (the areas behind your forehead) or its temporal lobes (the regions behind your ears).|
The cell damage caused by frontotemporal dementia leads to tissue shrinkage and reduced function in the brain's frontal and temporal lobes, which control planning and judgment; emotions, speaking and understanding speech; and certain types of movement.
FTD includes a range of specific disorders with different core symptoms. But there's significant symptom overlap, especially as these disorders progress. The disorders grouped under FTD fall into three broad categories (discussed below). Scientists have identified a range of microscopic brain abnormalities implicated in FTD. The overall term for the brain cell damage and tissue shrinkage associated with FTD is frontotemporal lobar degeneration.
FTD used to be called Pick's disease after Arnold Pick, a physician who in 1892 first described a patient with distinct symptoms affecting language. Some doctors still use the term "Pick's disease." Other terms you may see used to describe FTD include frontotemporal disorders, frontotemporal degeneration and frontal lobe disorders.
FTD was once considered rare, but it's now thought to account for up to 10 to 15 percent of all dementia cases. It's still believed to be less common than Alzheimer's disease, vascular dementia and Lewy body dementia.
In those younger than age 65, FTD may account for up to 20 to 50 percent of dementia cases. People usually develop FTD in their 50s or early 60s, making the disorder relatively more common in this younger age group. Sign up for our enews to receive updates about Alzheimer’s and dementia care and research.
Experts group frontotemporal dementia into three main categories. Initially, these groups tend to cause different core symptoms based on the first parts of the brain's frontal or temporal lobes they affect. But there's increasing overlap in symptoms as these disorders progress.
Symptoms related tothe three types of FTD include:
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There is no single test — or any combination of tests — that can conclusively diagnose frontotemporal dementia. FTD is a "clinical" diagnosis representing a doctor's best professional judgment about the reason for a person's symptoms. Magnetic resonance imaging (MRI) often plays a key role in diagnosis because it can detect shrinkage in the brain's frontal and temporal lobes, which is a hallmark of FTD.
In some cases, it may be hard to distinguish FTD from Alzheimer's disease. In the future, tests to detect specific protein abnormalities linked to Alzheimer's and FTD may help clarify the diagnosis in difficult cases.
Researchers have identified abnormal deposits of several proteins inside the brain cells of those who died with frontotemporal dementia. Scientists have not yet learned what causes these protein abnormalities or solved the mystery of why the damage associated with FTD targets the brain's frontal and temporal lobes.
The only known risk factor for FTD is a family history of the disease. Scientists have found several genes linked to FTD.
Recent research suggests a possible connection between FTD and Lou Gehrig's disease, also known as amyotrophic lateral sclerosis (ALS) and motor neuron disease. Although ALS primarily affects nerves controlling voluntary movement, many people with ALS also develop symptoms affecting their behavior or language, and some people with FTD develop ALS.
Current frontotemporal dementia treatment focuses on managing symptoms, primarily those affecting behavior. Emerging insights into specific protein abnormalities associated with FTD may identify targets for new treatments aimed at underlying disease processes.
Antidepressants and antipsychotic drugs are the chief medications used to treat behavioral FTD symptoms. None of these drugs have been approved by the U.S. Food and Drug Administration (FDA) for use in FTD.
FTD inevitably gets worse, usually over several years. In advanced FTD, people typically become mute and bedbound. Like other types of dementia, FTD shortens lifespan. Studies suggest that most people with FTD survive an average of six to eight years, but survival can range from two to 20 years.