Frontotemporal Dementia: General Overview
Synopsis: Frontotemporal Dementia describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. The presence of abnormalities in the nerve cells of the brain, called Pick bodies, distinguishes frontal lobe dementia from other types of dementia. No treatment has been shown to slow the progression of FTD. Behavior modification may help control unacceptable or dangerous behaviors.
- Frontotemporal Dementia (FTD)
Frontotemporal dementia (FTD) or frontotemporal degenerations refers to a group of disorders caused by progressive nerve cell loss in the brain's frontal lobes (the areas behind your forehead) or its temporal lobes (the regions behind your ears). These brain areas are generally associated with personality, behavior, and language. Frontotemporal dementia often begins between the ages of 40 and 65 but also occurs later in life. FTD is the cause of approximately 10% to 20% of dementia cases. Signs and symptoms of frontotemporal dementia can differ from one individual to the next. Signs and symptoms get progressively worse over time, usually over the years. Some people with frontotemporal dementia have dramatic changes in their personalities and become socially inappropriate, impulsive, or emotionally indifferent, while others lose the ability to use language properly. A family history of FTD is the only known risk for these diseases. Although experts believe that some cases of FTD are inherited, most people with FTD have no family history of it or other types of dementia.
Alternate Names: Frontotemporal Lobar Degeneration, Dementia with Lobar Atrophy and Neuronal Cytoplasmic Inclusions, Diffuse Degenerative Cerebral Disease, Lobar Atrophy of the brain, Pick Disease of the brain-Type 1, Wilhelmsen-Lynch Disease.
Frontotemporal Dementia (FTD) describes a clinical syndrome associated with the shrinking of the frontal and temporal anterior lobes of the brain.
The U.S. Social Security Administration (SSA) has included Frontotemporal Dementia (FTD), Picks Disease,Type A, Adult as a Compassionate Allowance to expedite a disability claim.
Originally known as Pick's disease, the name, and classification of Frontotemporal Dementia (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.
The presence of abnormalities in the brain's nerve cells, called Pick bodies, distinguishes frontal lobe dementia from other types of dementia. As it is defined today, the symptoms of FTD fall into two clinical patterns that involve either:
- Changes in behavior
- Problems with language
The first type features behavior that can be either impulsive (dis-inhibited) or bored and listless (apathetic). It also includes inappropriate social behavior; lack of social tact; lack of empathy; distractibility; 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. The disease has a strong genetic component; FTD often runs in families.
Diagnostic tests may include:
- physical exam, clinical assessment, and blood tests,
- neurological exam that checks awareness and responsiveness, vital signs, reflexes, sensory responses, and coordination.
- neuropsychological testing assesses memory, ability to reason and judge, problem-solving and language skills.
- Brain imagining, such as MRI and CT, may demonstrate shrinkage of the frontal and temporal lobes and also help exclude other causes of dementia, such as strokes and brain tumors. PET and SPEC tomography testing may be used to evaluate brain activity.
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 and tranquilizers have been shown to improve some symptoms.
The outcome for individuals with FTD is poor.
The disease progresses steadily and often rapidly, ranging from less than two years in some individuals to more than ten years in others. Eventually, some individuals with FTD will need 24-hour care and monitoring at home or in an institutionalized care setting.
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