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What are Frontotemporal Dementias?

Frontotemporal dementias (FTDs) are a group of closely related brain diseases that affect the frontal lobe and/or temporal lobes of the brain, leading to impairments in social behavior, self-control, emotional responsiveness and language skills. The diseases affects more areas of the brain over time. They commonly affect individuals between the ages of 40 and 65, with the onset of symptoms typically occurring between the ages of 50 and 55. Men and women are equally affected. In nearly half of all cases, there is a family history of the disease.

In the beginning stages, the primary symptoms involve abnormal social behavior, neglect of personal hygiene and habits, or problems with speech and language. Memory is usually unaffected until later in the disease.  Typically patients suffer loss of concern for others, indifference, lack of initiative, tactlessness, and impulsiveness, loss of social graces, neglect of personal hygiene, a change in eating habits, irresponsibility, poor social judgment, and irritability. Many patients also manifest mainly language problems such as difficulty finding the right words, labored speech, reduced amount of speech, inability to name objects, pronunciation and naming errors, and forgetting the meaning of words. In addition, many patients suffer motor symptoms that may include abnormal walking, eye movement problems, slowness, muscle weakness, stiffness, and tremors.

As the damage to the brain becomes more widespread, all aspects of a person’s intellectual and social functioning deteriorate. Memory is affected eventually, and patients progressively lose the ability to attend to their own basic needs. late in the disease many individuals require nursing home care.

How is FTD diagnosed?

  • We take a detailed history of the symptoms from the person affected with this illness, as well as from family members or friends. Generally, FTDs manifest with changes in personality, social behavior, and speech or language rather than with forgetfulness or disorientation. These are some of the helpful features in the distinction between FTDs and Alzheimer's disease (and other causes of dementia).
  • A neurological exam and paper and pencil tests to assess intellectual abilities are used to define specific problem areas and identify patterns of disability that fit with the diagnosis (or with another condition).
  • Brain scans help in distinguishing FTDs from strokes and Alzheimer's disease. At present, genetic testing is not routine in the diagnosis of FTDs.

What Treatments are Available?

At present, treatments for frontotemporal dementias are focused on disease management, since curative treatments are not available.  These interventions include:

  • Prescription medicines to lesson the cognitive and behavioral manifestations of the FTDs.
  • Social work services, which include practical assistance in the management of employment, retirement, and disability applications; guidance in the planning of long-term care and in the management of social and financial resources and liaison with respite and residential programs.
  • Inpatient care in the Johns Hopkins Neuropsychiatry and Memory Unit.
  • Community support services, including support groups focused on FTD and young-onset dementias (see below)
  • Guidance with issues pertaining to dependent children.

CLINICAL CARE
Refer a patient to or to make an appointment in the Frontotemporal and Young-Onset Dementia Clinic.

RESEARCH
Learn more about ongoing JHU research on Creutzfeldt-Jakob Disease?

Additional Resources

The Association for Frontotemporal Dementias (AFTD)

The Alzheimer’s Disease and Related Disorders Association

What if it’s not Alzheimer’s? A Caregiver’s Guide to Dementia, Lisa Radin and Gary Radin, editors, Prometheus Books, 2003.

The Banana Lady and Other Stories of Curious Behavior and Speech by Dr. Andrew Kertesz, Trafford Publishing, 2006. 


 
 

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