Frontotemporal dementia (FTD) is a group of degenerative diseases that gradually damage or shrink the front of the brain — the frontal and anterior temporal lobes. These two areas are the center of many important brain functions, including language skills and the ability to focus attention, make plans and decisions and control impulses.
Symptoms often relate to personality or mood changes, compulsive or repetitive behavior and lack of emotion, inhibition or social tact.
The UCSF Memory and Aging Center is one of the few centers in the country specializing in the diagnosis and research of FTD, which includes frontotemporal lobar degeneration, Pick's Disease, progressive aphasia and semantic dementia.
FTD affects an estimated 250,000 Americans. The average duration of the disease is about eight years. In the past, the disease was confused with Alzheimer's disease or other forms of dementia, but unlike Alzheimer's disease, frontotemporal dementia appears earlier in life. As researchers have learned more about the disease, they now believe it is the second leading cause of dementia in middle age. Most patients are diagnosed during their 50s and 60s with men and women equally affected.
About 40 percent of patients have a clear family history. The remaining 60 percent of cases occur sporadically. If a parent has frontotemporal dementia, one in two of the children are likely to develop the disease. In some cases where the disorder appears to be inherited, there is an apparent change or mutation in a gene on chromosome 3, 9 or 17. Other genes may be involved as well. The cause of the illness, however, is unknown.
In the past, patients with frontotemporal dementia (FTD) often were misdiagnosed with depression, schizophrenia or Alzheimer's disease. Because some FTD cases still may be misidentified, doctors at the UCSF Center for Memory and Aging say it's difficult to determine the prevalence of the disorder but they believe FTD is the most common dementia diagnosed in patients under age 60 and is as common as Alzheimer's disease among patients age 45 to 64.
Early symptoms typically involve personality or mood changes such as depression and withdrawal, sometimes obsessive behavior and language difficulties. Many patients lose their inhibitions and exhibit antisocial behavior.
Doctors at UCSF have identified a small group of patients who develop extraordinary visual or musical creativity, while experiencing language and social impairment.
Because the first symptoms tend to affect personality and behavior, frontotemporal dementia may be mistaken as a psychiatric disorder. Diagnosis requires a thorough physical examination and interview with family members.
At the UCSF Memory and Aging Center, patients undergo an extensive neurological, neurophysicial and nursing assessment, usually taking about three hours. Caregivers are also interviewed.
If FTD is diagnosed early in the course of the disease, doctors can prescribe medications and help families prepare and cope with symptoms. The condition may last from three to 17 years before death, with an average duration of eight years after diagnosis.
Therapy is designed to relieve the symptoms or behaviors caused by frontotemporal dementia, but there is no treatment to stop or reverse the underlying brain deterioration. Antidepressants called selective serotonin reuptake inhibitors (SSRIs) may offer some relief from apathy and depression and help reduce food cravings, loss of impulse control and compulsive activity.
Reviewed by health care specialists at UCSF Medical Center.