Disorder Definitions

Disease Name:

Frontotemporal Lobar Degeneration
 

What is frontotemporal lobar degeneration?
Frontotemporal lobar degeneration (FTLD) is the umbrella term for a group of progressive, degenerative brain diseases that gradually destroy the ability to behave appropriately, empathize with others, learn, reason, make judgments, communicate, and carry out daily activities. These diseases include behavioral variant frontotemporal dementia (FTD), semantic variant primary progressive aphasia (svPPA), nonfluent/agrammatic variant primary progressive aphasia (nfvPPA), corticobasal degeneration (CBD), and progressive supranuclear palsy (PSP). There are several forms of the disease that lead to slightly different behavioral, language and/or motor symptoms. Due to the symptoms, FTLD can be mistaken for Alzheimer's disease, Parkinson's disease or a primarily psychiatric disorder like depression, manic-depression, obsessive-compulsive disease or schizophrenia. There is no treatment or cure yet that can reverse the damage, but medications and lifestyle changes can help relieve the symptoms. FTLD is not contagious.

 

Who gets FTLD syndromes?
People all over the world get FTLD. In people under age 60, FTLD is the most common cause of dementia and affects as many people as Alzheimer's disease in the 45–64 age group. Both men and women can develop FTLD, but it is more common in men.

 

What causes FTLD?
In most cases, the cause of FTLD is unknown. Approximately 40% of people with FTLD have a family history of FTD or another related dementia, while 5–10% of patients have a family history that shows an autosomal dominant inheritance pattern. This means that a child of someone with a disease has a 50% chance of inheriting the same illness-causing trait.

 
Healthy FTD

How is FTLD diagnosed?
People with FTLD typically first come to the doctor's office because of gradual and steady changes in behavior, language dysfunction or weakness or slowing of movement. The diagnosis of FTLD is made by reviewing the data from a neurological exam, neuropsychological testing, laboratory tests, and neuroimaging studies.

 

What is the treatment for FTLD?
There is no treatment or cure yet that can reverse the damage, but medications and lifestyle changes can help relieve the symptoms.


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