Frontotemporal Lobar Degeneration Background

An individual with frontotemporal lobar degeneration, or FTLD, will present with striking changes to their ability to think, behave and/or move. FTLD can be clinically characterized by the development of inappropriate behavior and apathy. For example, a respected member of the community may start making rude comments or stop bathing. Other symptoms can include a significant change in appetite — a person who has eaten healthy his entire life may start eating excessive sweets. Other symptoms include difficulty with speech and understanding or problems with movement. A person does not have to have all of these symptoms to have a form of FTLD.  Also, many of these symptoms overlap with other conditions and a person should visit a neurologist for proper diagnosis.

A mistake (mutation) in one of a handful of genes can cause FTLD. For example, mutations in the gene called MAPT causes a form of FTLD that often presents with motor problems that resemble Parkinson’s Disease. A mutation in another gene called granulin causes another form of FTLD and typically results in the partial loss of a particular protein that may help the brain age in a healthy manner.  Mistakes in another gene, termed C9ORF72, causes the gene to expand; however, it is still not clear exactly how this causes damage.

At this time, there is no cure for FTLD, but several labs at UF are working on understanding the basis of these diseases, developing model systems, and testing therapies. FTLD shares both symptoms and nerve cell changes in the brain and spinal cord that are similar to other diseases.  For this reason, many scientists who work on FTD also work on amyotrophic lateral sclerosis (ALS), Alzheimer’s disease and Parkinson’s disease. By addressing FTLD as part of a broader spectrum of brain and spinal cord disorders, we hope to be able to find common pathways that could be therapeutic targets for multiple diseases.

More Information

The Association for Frontotemporal Dementia