Frontotemporal Degeneration Unit 

The Frontotemporal Degeneration Unit at the Nantz National Alzheimer Center provides diagnosis and treatment for frontotemporal dementia (FTD) and related disorders including primary progressive aphasia, semantic dementia, corticobasal degeneration syndrome, and progressive supranuclear palsy. Our aim is to develop better knowledge about diagnosis and treatment of all forms of FTD and related focal dementia syndromes.

What is FTD?

Frontotemporal dementia (FTD) is 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.

People with FTD often come to the doctor’s office because of gradual and steady behavior changes, language dysfunction, or weakness/slowing of movement. Behavioral changes may include apathy, loss of sympathy or empathy, impulsivity, a disregard for social conventions, repetitive or compulsive behaviors, dietary changes, and poor judgment and planning. Patients with one of the language variants have difficulties expressing themselves, and may have problems with reading or writing. Movement changes are often described as muscle stiffness, slowness, or weakness and feeling uncoordinated.

There is no treatment or cure yet that can reverse the damage, but medications and lifestyle changes can help relieve the symptoms. FTD is not contagious.

How is Age Related to FTD?

In people under age 60, FTD is the most common cause of dementia and affects as many people as Alzheimer's disease in the 45-64 age group. Men are affected more commonly than women.

What are the Forms of Frontotemporal Dementia?

• Behavioral variant frontotemporal dementia (bvFTD): The bvFTD form affects behavior and personality. Patients have increasing trouble controlling their behavior and may say inappropriate things or ignore other people's feelings. Behavioral changes can include apathy, lack of motivation, loss of sympathy or empathy for others and overeating.
• Semantic variant primary progressive aphasia (svPPA): The svPPA form affects the ability to use and understand language. Patients often have increasing trouble understanding the meaning of words, finding words, or naming people and objects. In general, the tone, rhythm and fluctuations of pitch (prosody) sound normal.
• Non-fluent/agrammatic variant primary progressive aphasia (nfvPPA): The nfvPPA form affects the ability to speak. Patients often have increasing trouble getting words out, pronouncing words and putting sentences together.
• Corticobasal syndrome (CBS): The CBS form affects movement, language skills, or both. CBS often begins with movement problems – such as stiff muscles on one side of the body involving the arm, leg, or both. Language problems and/or changes in thinking or behavior may occur as well.
• Progressive supranuclear palsy (PSP): The PSP form affects movement, language and behavior. Difficulties with balance that lead to frequent falls, eye movement problems, slowed movement, stiffness and difficulty walking, changes in speaking, thinking or behavior are common.
• Motor neuron disease (FTD-MND): The FTD-MND form affects movement, causing progressive muscle weakness and atrophy with symptoms similar to Parkinson’s disease or amyotrophic lateral sclerosis. Changes in behavior, personality and language can also occur.
• There are several forms of the disease that lead to slightly different behavioral, language and/or motor symptoms. Due to the symptoms, FTD can be mistaken for Alzheimer's disease, Parkinson's disease or a primary psychiatric disorder like depression, manic-depression, obsessive-compulsive disease or schizophrenia.

What is the treatment for FTD?

Unfortunately, FTD has no cure, but medications and lifestyle changes can help relieve the symptoms. In some cases, physical therapy that focuses on balance and stretching also is important. A speech-language pathologist may help improve speech problems. Furthermore, researchers are actively searching for new treatments and running clinical trials to test promising new medications.

Participate in Research

Our Frontotemporal Degeneration Unit actively conducts research to improve the diagnosis and treatment of FTD, including a search for the cure. Patient participation helps us reach these goals If you have been diagnosed with FTD, talk to your doctor or contact us to see if joining a research project is right for you. Our research includes:

 

• Social Cognition and Neuroimaging in FTD
• ALLFTD: Longitudinal Frontotemporal Lobar Degeneration Study
• INFRONT-3: A trial of AL001 in Participants with a Progranulin Mutation Causative of Frontotemporal Dementia
• Veri-T: A Trial of Verdiperstat in Patients with Semantic Variant Primary Progressive Aphasia
• IL2PlusA-PPA: A Trial of Interleukin-2 plus Abatacept in Patients with Non-Fluent Variant Primary Progressive Aphasia and Semantic Variant Primary Progressive Aphasia

Resources

• The Association for Frontotemporal Degeneration
• The Frontotemporal Dementia Support Group
• Frontotemporal Dementia Caregiver Support Center
• The Bluefield Project/Consortium for Frontotemporal Research (CFR)
• Family Caregiver Alliance
• National Institutes of Health