Frontotemporal Dementia
Overview
Frontotemporal dementia (FTD) is a group of degenerative diseases that gradually damage or shrink the brain's frontal and anterior temporal lobes, located in the front of the brain. These lobes are the center of many important brain functions, including language skills and the abilities to focus attention, make plans and decisions, and control impulses. FTDs include frontotemporal lobar degeneration, Pick's disease, progressive aphasia and semantic dementia.
Symptoms often involve personality or mood changes, compulsive or repetitive behaviors, and lack of emotion, inhibition or social tact.
FTD affects an estimated 1 in 5,000 to 10,000 people. Calculating its prevalence is difficult because most experts believe it is underdiagnosed.
Researchers now believe FTD is the second leading cause of dementia in middle age. In the past, it was often confused with Alzheimer's disease or other forms of dementia, but FTD appears earlier in life than Alzheimer's disease. Most patients are diagnosed during their 50s and 60s, and the disease lasts an average of eight years. Men and women are equally affected.
About 40 percent of patients have a clear family history. The remaining 60 percent of cases occur randomly. 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.
Our approach to frontotemporal dementia
UCSF is home to one of the few centers in the country that specialize in research and diagnosis of frontotemporal dementia. We offer extensive assessments to correctly diagnose FTD, which may be mistaken for other conditions, as well as medications to minimize symptoms. Because caring for a loved one with FTD can be very challenging, we hold a monthly support group to provide information and comfort for families and caregivers. We also work to connect families with outside support services.
Awards & recognition
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Among the top hospitals in the nation
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Best in the West and No. 3 in the nation for neurology & neurosurgery
Signs & symptoms
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.
As FTD progresses, it takes a toll on mental abilities, affecting memory and other functions that are more common in Alzheimer's disease and other dementias. In Alzheimer's, one of the first symptoms is memory loss. With FTD, unusual or antisocial behavior as well as loss of speech or language are usually the first symptoms.
In later stages, patients develop movement disorders such as unsteadiness, rigidity, slowness, twitches, muscle weakness or difficulty swallowing. Some patients develop Lou Gehrig's disease or amyotrophic lateral sclerosis (ALS). People in the final stages of FTD cannot care for themselves.
Behavioral symptoms
Early signs of frontotemporal dementia may involve the following symptoms:
- Apathy or an unwillingness to talk
- Change in personality and mood, such as depression
- Lack of inhibition or lack of social tact
- Obsessive or repetitive behavior, such as compulsively shaving or collecting items
- Unusual verbal, physical or sexual behavior
- Weight gain due to dramatic overeating (Read more about FTD and binge eating.)
Patients may neglect hygiene and resist encouragement to attend to themselves. They also may lack awareness or concern that their behavior has changed.
At the UCSF Memory and Aging Center, doctors have found a small group of FTD patients who develop new creative skills in music and art. The artistic talents developed when brain cell loss occurred predominantly in the left frontal lobe, which controls functions such as language. It is believed that the right side of the brain regulates more abstract reasoning.
Language symptoms
Language problems are less common but do occur in the early stages of FTD before other thought processes, such as memory, are affected. Patients may experience difficulty speaking or finding the correct word when naming objects. Difficulties reading and writing then develop. As the disease progresses, less and less language is used, until the patient becomes virtually mute. Other patients may have a severe problem recalling words and understanding word meaning, but continue to have otherwise normal speech.
Diagnosis
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.
Because memory loss and other related symptoms are often complex, the evaluation may require two to three visits to determine the cause of the symptoms and recommend treatment.
After the evaluation, the medical team meets to discuss the diagnosis and potential treatments and then discusses its findings with the patient and the family. In some cases, a diagnosis will be deferred until more information from blood tests or brain imaging is collected.
FTD leads to loss of brain tissue that is visible on imaging tests, such as magnetic resonance images (MRI), which are key in identifying the characteristic shrinking of the frontal and temporal lobes, located in the front of the brain. Other tests include positron emission tomography (PET), computed tomography (CT) and single photon emission computed tomography (SPECT).
As part of the diagnosis, a written report is sent to the patient's primary care physician or specialist who referred the patient to UCSF.
Treatments
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.
Doctors may prescribe anti-psychotics, medications that can alleviate extremely unrealistic or disorganized thinking such as hallucinations, delusions and aggression. Older anti-psychotic medications that block dopamine may be dangerous for FTD patients because some of them have Parkinson's disease, which causes a loss of dopamine, a chemical messenger that transmits signals within the brain.
Cholinesterase inhibitors – the class of drugs currently used to treat memory symptoms in Alzheimer's – do not help FTD patients. These drugs temporarily increase supplies of the messenger chemical acetylcholine to failing nerves, but FTD does not affect nerves in the acetylcholine communication system.
Some patients with FTD develop Lou Gehrig's disease, also known as amyotrophic lateral sclerosis (ALS). Doctors don't yet fully understand the connection between the two diseases but are studying the trend.
Many FTD patients remain at home and others require nursing home care. Being a caregiver of an FTD patient can be physically and emotionally exhausting. If you are a caregiver, you should seek as much help as possible to carry out your day-to-day tasks. The UCSF Memory and Aging Center sponsors a Frontotemporal Dementia Support Group that meets monthly to provide support and education to families of FTD patients and referrals for community resources.
UCSF Health medical specialists have reviewed this information. It is for educational purposes only and is not intended to replace the advice of your doctor or other health care provider. We encourage you to discuss any questions or concerns you may have with your provider.
