Frontotemporal Dementia

Frontotemporal dementia is a type of degenerative brain disease. Unlike Alzheimer's disease, frontotemporal dementia affects people younger than 65. Learn how Lehigh Valley Health Network’s Neuroscience Center and the Fleming Memory Center at LVH–17th Street can help you and your family.

Frontotemporal dementia is a degenerative brain disease and occurs when the nerve cells in the frontal and temporal lobes of the brain are damaged, causing the lobes to shrink. Frontotemporal dementia can affect a person’s behavior, personality, language skills and movement.

About 250,000 Americans have a type of frontotemporal dementia. These forms of dementia are the most likely to strike younger people. Symptoms typically start between the ages of 40 and 65 but also can affect those who are younger or older. Frontotemporal dementia affects men and women equally.

What causes frontotemporal dementia?

The cause of frontotemporal dementia is unknown. Researchers have linked certain subtypes of frontotemporal dementia to mutations on several genes. Some people with frontotemporal dementia have tiny structures – called Pick bodies – in their brain cells. Pick bodies contain an abnormal amount or type of protein. Until other variants of this disease were identified, this form of dementia was known as Pick’s disease.

A family history of frontotemporal dementia is the only known risk factor, but most people with this brain disease have no family history of it or other types of dementia.

The most common types of frontotemporal dementia are:

  • Frontal variant, which affects a person’s behavior and personality
  • Two subtypes of primary progressive aphasia: Progressive nonfluent aphasia, which affects a person’s ability to speak, and Semantic dementia, which affects a person’s ability to use and understand language
  • A less common form of frontotemporal dementia affects a person’s movement, causing symptoms similar to Parkinson’s disease or amyotrophic lateral sclerosis (Lou Gehrig’s disease)
One out of three senior citizens will be diagnosed with dementia. Locally, the over-65 population in Lehigh and Northampton counties is projected to nearly double by 2030.

Diagnosis and next steps

Diagnosis often comes after a family member notices changes in behavior or language skills. No single test can determine a frontotemporal dementia diagnosis. Routine blood tests and physical exams may be performed to rule out other conditions.

A person can live with frontotemporal dementia for many years. Pneumonia is the most common cause of death in those who have frontotemporal dementia. They also are at increased risk for infections and fall-related injuries.

Currently, no treatments are available to cure or slow the progression of frontotemporal dementia, but doctors may prescribe medications to treat symptoms, such as antidepressants or prescription sleeping medications. Physical and occupational therapists can help a patient adjust to some of the changes caused by frontotemporal dementia.

Advance directives, power of attorney and other legal actions may make it easier to decide about the care of the person with dementia. Legal advice should be sought early before the person with dementia is unable to make such decisions.

Fleming Memory Center

Fleming Memory Center is an outpatient office at Lehigh Valley Hospital–17th Street that serves the needs of people with dementia and their caregivers.

Resources for caregivers include:

  • Monthly caregiver support groups (call 610-969-3390 for schedule)
  • Quarterly educational sessions
  • Social worker drop-in hours

For more information or an appointment, please call 888-402-LVHN (5846).