Memory Disorders | Frontotemporal Dementia

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. Some estimate that the disease may represent 10 percent to 20 percent of dementia cases.

Early symptoms of frontotemporal dementia 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. At the UCSF Memory and Aging Center, doctors 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 and other dementias. In Alzheimer's disease, 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 Gherig's disease or amyotrophic lateral sclerosis (ALS). People in the final stages of FTD cannot care for themselves.

Early signs of frontotemporal dementia may involve the following symptoms:

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 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.

Because the first symptoms tend to affect personality and behavior, frontotemporal dementia may be mistaken as a psychiatric disorder. Correct identification requires a thorough physical examination and a careful 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. Information from the caregiver is sought in every case.

Because memory loss and other related symptoms are often complex, a comprehensive evaluation is necessary. The evaluation may require two to three visits to determine the cause of the symptoms and recommend treatment.

After the evaluation, the medical team involved with each patient meets to discuss the diagnosis and potential treatments. After this meeting, the team 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.

The disease 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.

If FTD is diagnosed early in the course of the disease, doctors can prescribe the appropriate 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 dopanmine 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 Gherig'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.