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.
No treatment has been shown to slow the progression of FTD. Behavior modification may help control unacceptable or dangerous behaviors.
Aggressive, agitated, or dangerous behaviors could require medication. Anti-depressants have been shown to improve some symptoms.
The outcome for people with FTD is poor. The disease progresses steadily and often rapidly, ranging from less than 2 years in some individuals to more than 10 years in others.
Eventually some individuals with FTD will need 24-hour care and monitoring at home or in an institutionalized care setting.
The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health (NIH) conduct and fund research on FTD.
Among several research projects, scientists hope to identify novel genes involved with FTD, perhaps leading to therapeutic approaches where delivery of normal genes would improve or restore brain function. Clinical imaging may help researchers better understand changes in the brains of people with FTD, as well as help diagnose these disorders.
Other projects are aimed a better understanding the toxic effects of protein buildup and how it is related to the development of FTD and related dementias.
