Frontotemporal degeneration (FTD) is a disease process that results in progressive damage to the anterior temporal and/or frontal lobes of the brain. It causes a group of brain disorders that share many clinical features. The hallmark of FTD is a gradual, progressive decline in behavior and/or language that often has a relatively young age at onset (mid-50s to 60s), but has been seen as early as 21 and as late as 80 years. As the disease progresses, it becomes increasingly difficult for people to plan or organize activities, behave appropriately in social or work settings, interact with others, and care for oneself, resulting in increasing dependency.
FTD represents an estimated 10%-20% of all dementia cases and is recognized as one of the most common dementias affecting a younger population. It is estimated that FTD affects approximately 50,000-60,000 Americans. FTD occurs equally in men and women. In a small percentage of cases, it is inherited.
While there are currently no treatments to slow or stop the progression of the disease, FTD research is expanding, producing greater understanding of the disorders. We anticipate that this knowledge will result in a growing number of potential therapeutics entering clinical testing within the next few years.
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 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:
- 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 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.
Information sourced from The New York Times:
Frontotemporal dementia, also called frontotemporal degeneration or Pick’s disease, refers to a group of diseases that destroy nerve centers in the frontal and temporal lobes — the home of decision-making, emotion, judgment, behavior and language. Some forms of the disease also cause movement disorders.
Most cases occur sporadically, in people with no family history of the illness — like Michael French — but a small percentage are inherited.
Patients generally receive from one to four misdiagnoses, and it may take years to finally get the right answer. Mistaken diagnoses can include Alzheimer’s disease, stroke, midlife crisis or psychiatric illnesses like depression, bipolar disorder, post-traumatic stress or anxiety. Many relatives of patients say doctors dismiss their reports of personality change. But it is real.
There are eight subtypes of frontotemporal degeneration, sorted by the symptoms they cause. Some affect behavior. Others, grouped under the heading primary progressive aphasia, affect language. Still others affect movement, leading to disorders that resemble Parkinson’s orLou Gehrig’s disease (also called amyotrophic lateral sclerosis or A.L.S.). But patients may match more than one category, and the subtype may change as the disease progresses.
According to Dr. Edward Huey, an assistant professor of psychiatry and neurology at Columbia University Medical Center:
“The frontal lobes are sort of the last frontier in the brain,” Dr. Huey said, adding that the losses these patients suffer are helping researchers understand more about what the frontal lobes do. As the brain atrophy progresses, Dr. Huey said, patients “have pieces of psychiatric syndromes, but not the whole syndrome.” For instance, they have compulsions, but not the usual accompaniment, obsessions. So they may wash their hands over and over again, but not in a worried or anxious way. Some lose their inhibitions and moral judgment. Shoplifting is not uncommon. Many have the apathy and social disconnection that usually go with depression, but they do not feel depressed. “They’re not down, but they just don’t enjoy things as much as they used to,” Dr. Huey said. “There appears to be a dysfunction in the reward circuit, where activities that were rewarding and pleasurable no longer seem to be. These patients lose themselves.”