Frontotemporal Dementia (FTD) is a degenerative condition of the front (anterior) part of the brain. It differs from other causes of dementia such as Alzheimer’s, Lewy Body and Creutzfeldt Jakob’s diseases. FTD is currently understood as a clinical syndrome that groups together Pick's disease, primary progressive aphasia and semantic dementia. The areas of the brain affected by FTD—the frontal and anterior temporal lobes—control reasoning, personality, movement, speech, social graces, language and some aspects of memory.
FTD is marked by dramatic changes in personality, behavior and some thought processes. Changes in personal and social conduct occur in early stages of the disease, including loss of inhibition, apathy, social withdrawal, hyperorality (mouthing of objects), and ritualistic compulsive behaviors. These symptoms may lead to misdiagnosis as a psychological or emotionally-based problem, or, in the elderly, be mistaken for withdrawal or eccentricity. FTD progresses to immobility and loss of speech and expression. Structural changes in the FTD patient’s brain can be seen via scans or neuroimaging.
As many as seven million Americans may be afflicted with a form of dementia. Frontotemporal Dementia may account for 2-5 percent or 140,000 – 350,000 cases of dementia and for as many as 25 percent of pre-senile dementias.
FTD occurs predominantly after age 40 and usually before age 65, with equal incidence in men and women. In nearly half of the patients, a family history of dementia exists in a first degree relative (parent or sibling), suggesting a genetic component in these cases. Additionally, a form of dementia found in persons with motor neuron disease (amyotrophic lateral sclerosis, commonly known as “Lou Gehrig’s Disease”) may be associated with FTD.
Initial symptoms of FTD are primarily changes in personality and behavior. In addition to the symptoms described below, FTD patients often present two seemingly opposite behavioral profiles in the early and middle stages of the disease. Some individuals are overactive, restless, distractible and disinhibited. Others are apathetic, inert, aspontaneous and emotionally blunted. These differences in outward activity disappear in the late stages of the disease.
Major symptoms of FTD are:
In late stage FTD symptoms include:
FTD differs markedly in several ways when compared to other dementias, especially Alzheimer’s disease:
FTD can be accurately diagnosed with brain scans or imaging. Computed tomography (CT scan) and magnetic resonance imaging (MRI) reveal cerebral atrophy in the frontotemporal regions. Degeneration of the corpus striatum, thalamus and other subcortical structures occurs. Functional brain imaging and single photon emission tomography may reveal dysfunction of the frontal lobes, decreased blood flow, and a selective reduced uptake of tracer in the anterior (front) cerebral hemispheres. Electroencephalography (EEG) remains normal, however, even in advanced stages. In autopsies, brain tissue changes include large neuronal cell loss with secondary spongiform change and astrocyctic gliosis.
Neuropsychological testing is useful to obtain a clinical assessment of the disease. Tests evaluate conduct, language, visuo-spatial abilities, memory, abstraction, planning and mental control, motor skills and intelligence. Tests might show:
The length of FTD varies, with some patients declining rapidly over two to three years and others showing only minimal changes over a decade. Studies have shown persons with FTD to live with the disease an average of eight years, with a range from three years to 17 years.
No medications are known currently to treat or prevent FTD. Serotonin-boosting medications may alleviate some behaviors.
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Reviewed by Bruce Miller, M.D., Director, Memory and Aging Center, University of California at San Francisco. Prepared by Family Caregiver Alliance in cooperation with California’s Caregiver Resource Centers, a statewide system of resource centers serving families and caregivers of brain-impaired adults. Funded by the California Department of Mental Health. ©2005 Family Caregiver Alliance. All rights reserved.