Frontotemporal Disorders vs. Frontotemporal Dementia (FTD): Understanding Terms
Frontotemporal disorders is an umbrella term that is also used to describe the prime of life diseases. There are different types of frontotemporal disorders:
Types of frontotemporal disorders:
- Progressive behavior/personality decline—characterized by changes in personality, behavior, emotions, and judgment (called behavioral variant frontotemporal dementia)
- Progressive language decline—marked by early changes in language ability, including speaking, understanding, reading, and writing (called primary progressive aphasia or PPA)
- Progressive motor decline—characterized by various difficulties with physical movement, including the use of one or more limbs, shaking, difficulty walking, frequent falls, and poor coordination (called corticobasal syndrome, progressive supranuclear palsy, or amyotrophic lateral sclerosis)
One of the challenges shared by patients, families, clinicians, and researchers is confusion about how to classify and label frontotemporal disorders. A diagnosis by one doctor may be called something else by a second, and the same condition or syndrome referred to by another name by a pathologist who examines the brain after death. For many years, scientists and physicians used the term frontotemporal dementia (FTD) to describe a group of illnesses. After further research, FTD is now understood to be just one of several possible variations and is more precisely called behavioral variant frontotemporal dementia, or bvFTD. To describe all diseases in this groups, we now use the term frontotemporal disorders with frontotemporal lobar degeneration (FTLD).
Frontotemporal disorders are the result of damage to neurons (nerve cells) in parts of the brain called the frontal and temporal lobes. Almost all prime of life disease are part of the frontotemporal disorder spectrum.
As neurons die in the frontal and temporal regions, these lobes atrophy, or shrink. Gradually, this damage causes difficulties in thinking and behaviors normally controlled by these parts of the brain. Many possible symptoms can result, including unusual behaviors, emotional problems, trouble communicating, difficulty with work, or difficulty with walking.
FTD = bvFTD: the definition of behavioral variant FTD
Frontotemporal dementia (FTD) is a disease process that results in progressive damage to the temporal and/or frontal lobes of the brain. It causes a group of brain disorders that share many clinical features. FTD is also commonly referred to as frontotemporal degeneration, frontotemporal lobar degeneration (FTLD) or Pick’s disease.
FTD is distinct from other forms of dementia in two important ways:
- The hallmark of FTD is a gradual, progressive decline in behavior and/or language (with memory usually relatively preserved). 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 on caregivers.
- Onset of FTD often occurs in a person’s 50’s and 60’s, but has been seen as early as 21 and as late as 80 years. Roughly 60% of cases occur in people 45-64 years old (Knopman, 2011), thus FTD can affect work and family in a way dementia in older patients does not. There are currently no treatments to slow or stop the progression of the disease.
Although specific symptoms may vary from patient to patient, FTD is marked by an inevitable deterioration in functioning. The length of progression varies, from 2 to over 20 years with a mean course of 7-13 years from the onset of symptoms (Onyike and Diehl-Schmid, 2013). FTD itself is not life-threatening. It does, however, predispose patients to serious complications such as pneumonia, infection or injury from a fall. The most common cause of death is pneumonia.
Day To Day Life With FTD
Information for Patients, Families and Caregivers
This brochure has been created by the National Institute of Neurological Disorders and Stroke (the NINDS), the nation’s medical research agency.
The brochure contains the most up to date information available on symptoms, causes, diagnosis, treatments of frontotemporal disorders.
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