Frontotemporal Dementia
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Frontotemporal dementia (FTD) describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick’s disease, the name and classification of FTD has been a topic of discussion for over a century. The current designation of the syndrome groups together Pick’s disease, primary progressive aphasia, and semantic dementia as FTD. Some doctors propose adding corticobasal degeneration and progressive supranuclear palsy to FTD and calling the group Pick Complex. These designations will continue to be debated.
As it is defined today, the symptoms of FTD fall into two clinical patterns that involve either (1) changes in behavior, or (2) problems with language. The first type features behavior that can be either impulsive (disinhibited) or bored and listless (apathetic) and includes inappropriate social behavior; lack of social tact; lack of empathy; distractability; loss of insight into the behaviors of oneself and others; an increased interest in sex; changes in food preferences; agitation or, conversely, blunted emotions; neglect of personal hygiene; repetitive or compulsive behavior, and decreased energy and motivation. The second type primarily features symptoms of language disturbance, including difficulty making or understanding speech, often in conjunction with the behavioral type’s symptoms. Spatial skills and memory remain intact. There is a strong genetic component to the disease; FTD often runs in families.
Frontotemporal dementia (FTD), sometimes called frontal lobe dementia, describes a group of diseases characterized by degeneration of nerve cells - especially those in the frontal and temporal lobes of the brain. Unlike Alzheimer's Disease (AD), FTD usually does not include formation of amyloid plaques. In many people with FTD, there is an abnormal form of tau protein in the brain, which accumulates into neurofibrillary tangles. This disrupts normal cell activities and may cause the cells to die.
Experts believe FTD accounts for 2 to 10 percent of all cases of dementia. Symptoms of FTD usually appear between the ages of 40 and 65. In many cases, people with FTD have a family history of dementia, suggesting that there is a strong genetic factor in the disease. The duration of FTD varies, with some patients declining rapidly over 2 to 3 years and others showing only minimal changes for many years. People with FTD live with the disease for an average of 5 to 10 years after diagnosis.
Because structures found in the frontal and temporal lobes of the brain control judgment and social behavior, people with FTD often have problems maintaining normal interactions and following social conventions. They may steal or exhibit impolite and socially inappropriate behavior, and they may neglect their normal responsibilities. Other common symptoms include loss of speech and language, compulsive or repetitive behavior, increased appetite, and motor problems such as stiffness and balance problems. Memory loss also may occur, although it typically appears late in the disease.
In one type of FTD called Pick's disease, certain nerve cells become abnormal and swollen before they die. These swollen, or ballooned, neurons are one hallmark of the disease. The brains of people with Pick's disease also have abnormal structures called Pick bodies, composed largely of the protein tau, inside the neurons. The cause of Pick's disease is unknown, but it runs in some families and thus it is probably due at least in part to a faulty gene or genes. The disease usually begins after age 50 and causes changes in personality and behavior that gradually worsen over time. The symptoms of Pick's disease are very similar to those of AD, and may include inappropriate social behavior, loss of mental flexibility, language problems, and difficulty with thinking and concentration. There is currently no way to slow the progressive degeneration found in Pick's disease. However, medication may be helpful in reducing aggression and other behavioral problems, and in treating depression.
In some cases, familial FTD is linked to a mutation in the tau gene. This disorder, called frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17), is much like other types of FTD but often includes psychiatric symptoms such as delusions and hallucinations.
Primary progressive aphasia (PPA) is a type of FTD that may begin in people as early as their forties. "Aphasia" is a general term used to refer to deficits in language functions, such as speaking, understanding what others are saying, and naming common objects. In PPA one or more of these functions can become impaired. Symptoms often begin gradually and progress slowly over a period of years. As the disease progresses, memory and attention may also be impaired and patients may show personality and behavior changes. Many, but not all, people with PPA eventually develop symptoms of dementia.
Patients with Frontal Lobe Dementia Needed
Investigators at the National Institute of Neurological Disorders and Stroke (NINDS) conducting neurobehavioral studies seek patients with progressive dementia diagnosed as Pick's disease, frontal lobe dementia, progressive aphasia, or lobar atrophy of the frontal lobes. Patients with radiological evidence of focal atrophy of the frontal lobes are particularly needed.
Eligible patients must not have concurrent immune, respiratory, renal, hepatic, or gastrointestinal disease. Those enrolled in the study will undergo neuropsychological testing and brain imaging studies. The studies will be conducted at the National Institutes of Health (NIH) Clinical Center in Bethesda, MD. All study-related expenses will be paid by the NIH.
For more information, physicians should contact:
Dr. Jordan Grafman
Chief, Cognitive Neuroscience Section, NINDS, NIH
Building 10, Room 5C205, 10 Center Drive MSC 1440
Bethesda, MD 20892-1440;
- Telephone: 301-496-0220
- Fax: 301-480-2909
- E-mail: grafmanj@ninds.nih.gov.
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well this is my first trip to this site, but I have been dealing with this for quite a few years now, and now am trying to look for information.My FIL was diagnosed with FLD ,a few years ago and has come to a point on where we have to make a decision.. took him to the urologist today, and he basically said ,his brain and his bladder arent working on the same wavelength.. my husband (his second son) and I live an hr away from him, he lives, with his oldest son and DIL at his own home..
They do not give him the care he deserves and basically ignore him, we have fought to get him help, he does have a companion that comes during the week for a couple of hrs a day in the morning, he goes to adult day care m-f,begrudgingly~~ he says he is doing it for me,he enjoys himself when he is there,but more and more is complaining of just wanting to sleep and stay home..
The Dr suggested to speak with the social worker who set him up with all the services and think about putting him into a facility that would get him up and going around the clock.. he doesnt have people around him that would do that where he lives now, and my husband and I work full time. for the past 3 yrs we go,3 times a week,I go on my day off,take him to drs visits,make sandwiches for him, do his laundry,strip and make his bed and do his pills, all while working full time..
There is always one that gets saddled with the care and I dont mind doing it,but I have some health issues of my own and the other family members wont pitch in, they control his finances and all other aspects but wont give him the care that he deserves..
waiting for a call back from his case manager to see where we go from here..wow it is hard being in the sandwich generation